C C177536 GDC Property Terminology C129439 Medulloblastoma, Molecularly Defined A term that refers to the classification of medulloblastomas according to their molecular characteristics. medulloblastoma_molecular_classification medulloblastoma molecular classification C C177536 GDC Property Terminology C158874 Primary Site of Disease The anatomic location where a disease of interest originated. primary_site primary site C C177536 GDC Property Terminology C172676 Age-Days Age of subject in days. days_to_birth days to birth C C177536 GDC Property Terminology C177585 Ann Arbor B Symptoms Indicator An indication of whether a record includes B-symptom data based on the Ann Arbor lymphoma classification system guidelines. ann_arbor_b_symptoms ann arbor b symptom C C177536 GDC Property Terminology C177586 Ann Arbor Extranodal Involvement Indicator An indication of whether a record includes data regarding extranodal involvement based on the Ann Arbor lymphoma classification system guidelines. ann_arbor_extranodal_involvement ann arbor extranodal involvement C C177536 GDC Property Terminology C185261 Extraocular Nodule Size The dimensions of a nodule found near but outside the eye. size_extraocular_nodule size extraocular nodule A C177537 GDC Value Terminology C169918 Disitamab Vedotin An antibody-drug conjugate (ADC) composed of a monoclonal antibody targeting human epidermal growth factor receptor 2 (EGFR2; HER2) and conjugated to an as of yet undisclosed cytotoxic agent, with potential antineoplastic activity. Upon administration of anti-HER2 ADC RC48, the antibody moiety targets and binds to HER2 on tumor cells. Upon antibody/antigen binding and internalization, the cytotoxic agent induces tumor cell apoptosis, through an as of yet not publicly known mechanism. HER2, a tyrosine kinase receptor, is overexpressed by many cancer cell types. Anti-HER2 Antibody-drug Conjugate RC48 | therapeutic_agents C1909 therapeutic_agents A C177537 GDC Value Terminology C175823 Mipasetamab Uzoptirine An antibody-drug conjugate (ADC), consisting of mipasetamab, a humanized immunoglobulin (Ig) G1 monoclonal antibody directed against AXL receptor tyrosine kinase (AXL; UFO) that is site-specifically conjugated to uzoptitine (PL1601), which contains a valine-alanine cleavable linker and SG3199, a cytotoxic pyrrolobenzodiazepine (PBD) dimer, with potential antineoplastic activity. Upon administration of mipasetamab uzoptirine, mipasetamab binds to AXL, which is expressed on the surfaces of a variety of cancer cell types. Upon endocytosis and enzymatic cleavage, SG3199 is released and forms highly cytotoxic DNA interstrand cross-links, thereby blocking cell division and killing AXL-expressing cancer cells. AXL, a member of the TAM (TYRO3, AXL and MER) family of receptor tyrosine kinases, is overexpressed by many tumor cell types, and plays a key role in tumor cell proliferation, survival, invasion and metastasis; its expression is associated with drug resistance and poor prognosis. Anti-AXL/PBD Antibody-drug Conjugate ADCT-601 | therapeutic_agents C1909 therapeutic_agents A C177537 GDC Value Terminology C175858 Tarlatamab A bispecific T-cell engager (BiTE) antibody composed of two single-chain variable fragments (scFv), one directed against the tumor-associated antigen (TAA) delta-like protein 3 (DLL3) fused to one that is directed against the CD3 antigen found on T-lymphocytes, with potential immunostimulating and antineoplastic activities. Upon administration, tarlatamab binds to both the CD3 antigen on cytotoxic T-lymphocytes (CTLs) and the DLL3 antigen found on DLL3-expressing tumor cells. This activates and redirects CTLs to DLL3-expressing tumor cells, which results in the CTL-mediated cell death of DLL3-expressing tumor cells. DLL3, a Notch pathway protein, is overexpressed on a variety of cancer cell types. It plays a key role in embryonic development and in tumor initiation and proliferation. Anti-DLL3/CD3 BiTE Antibody AMG 757 | therapeutic_agents C1909 therapeutic_agents A C177537 GDC Value Terminology C187992 Thyroid Gland Mixed Medullary and Follicular Carcinoma A primary carcinoma of the thyroid gland containing a medullary carcinoma component that is immunohistochemically positive for calcitonin, and follicular carcinoma component that is immunohistochemically positive for thyroglobulin. 8346/3 | morphology || Mixed medullary-follicular carcinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis A C177537 GDC Value Terminology C187993 Thyroid Gland Mixed Medullary and Papillary Carcinoma A primary carcinoma of the thyroid gland containing a medullary carcinoma component that is immunohistochemically positive for calcitonin, and papillary carcinoma component that is immunohistochemically positive for thyroglobulin. 8347/3 | morphology || Mixed medullary-papillary carcinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis A C177537 GDC Value Terminology C27350 Myeloproliferative Neoplasm, Unclassifiable This subgroup of myeloproliferative neoplasms includes cases which do not meet the morphologic criteria of any of the defined myeloproliferative neoplasms, or which have characteristics that overlap at least two of the myeloproliferative neoplasms. 9975/3 | morphology || Myeloproliferative neoplasm, unclassifiable | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis D C177537 GDC Value Terminology C130011 Anti-HER2 Antibody-drug Conjugate RC48 An antibody-drug conjugate (ADC) composed of a monoclonal antibody targeting human epidermal growth factor receptor 2 (EGFR2; HER2) and conjugated to an as of yet undisclosed cytotoxic agent, with potential antineoplastic activity. Upon administration of anti-HER2 ADC RC48, the antibody moiety targets and binds to HER2 on tumor cells. Upon antibody/antigen binding and internalization, the cytotoxic agent induces tumor cell apoptosis, through an as of yet not publicly known mechanism. HER2, a tyrosine kinase receptor, is overexpressed by many cancer cell types. Anti-HER2 Antibody-drug Conjugate RC48 | therapeutic_agents C1909 therapeutic_agents D C177537 GDC Value Terminology C147027 Anti-DLL3/CD3 BiTE Antibody AMG 757 A bispecific T-cell engager (BiTE) antibody composed of two single-chain variable fragments (scFv), one directed against the tumor-associated antigen (TAA) delta-like protein 3 (DLL3) fused to one that is directed against the CD3 antigen found on T-lymphocytes, with potential immunostimulating and antineoplastic activities. Upon administration of anti-DLL3/CD3 BiTE antibody AMG 757, this bispecific antibody binds to both the CD3 antigen on cytotoxic T-lymphocytes (CTLs) and the DLL3 antigen found on DLL3-expressing tumor cells. This activates and redirects CTLs to DLL3-expressing tumor cells, which results in the CTL-mediated cell death of DLL3-expressing tumor cells. DLL3, a Notch pathway protein, is overexpressed on a variety of cancer cell types. It plays a key role in embryonic development and in tumor initiation and proliferation. Anti-DLL3/CD3 BiTE Antibody AMG 757 | therapeutic_agents C1909 therapeutic_agents D C177537 GDC Value Terminology C160890 Anti-AXL/PBD Antibody-drug Conjugate ADCT-601 An antibody-drug conjugate (ADC), consisting of a humanized immunoglobulin (Ig) G1 monoclonal antibody directed against AXL receptor tyrosine kinase (AXL; UFO) that is site-specifically conjugated to PL1601, which contains a valine-alanine cleavable linker and SG3199, a cytotoxic pyrrolobenzodiazepine (PBD) dimer, with potential antineoplastic activity. Upon administration, the monoclonal antibody moiety of anti-AXL/PBD antibody-drug conjugate ADCT-60 binds to AXL, which is expressed on the surfaces of a variety of cancer cell types. Upon endocytosis and enzymatic cleavage, free PBD is released and forms highly cytotoxic DNA interstrand cross-links, thereby blocking cell division and killing AXL-expressing cancer cells. AXL, a member of the TAM (TYRO3, AXL and MER) family of receptor tyrosine kinases, is overexpressed by many tumor cell types, and plays a key role in tumor cell proliferation, survival, invasion and metastasis; its expression is associated with drug resistance and poor prognosis. Anti-AXL/PBD Antibody-drug Conjugate ADCT-601 | therapeutic_agents C1909 therapeutic_agents D C177537 GDC Value Terminology C46104 Thyroid Gland Mixed Medullary and Follicular Cell Carcinoma A primary carcinoma of the thyroid gland containing a medullary carcinoma component that is immunohistochemically positive for calcitonin, and follicular cell carcinoma structures that are immunohistochemically positive for thyroglobulin. 8346/3 | morphology || 8347/3 | morphology || Mixed medullary-follicular carcinoma | primary_diagnosis || Mixed medullary-papillary carcinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C102330 Perihilar Lymph Node A lymph node located in the area around the hilum of an organ. Hilar | lymph_node_involved_site || Lymph Node(s) Hilar | biospecimen_anatomic_site || Lymph Node(s) Hilar | treatment_anatomic_site C171435 || C33027 || C70729 biospecimen_anatomic_site || lymph_node_involved_site || treatment_anatomic_site C C177537 GDC Value Terminology C120023 Histone H3 Acetyl Lys28 A post-translationally modified form of histone H3 where the lysine residue at position 28 is acetylated. This modification may be a marker for transcriptional enhancement. H3K27ac | chipseq_target C177614 chipseq_target C C177537 GDC Value Terminology C174110 Histone H3 Trimethyl Lys28 A post-translationally modified form of histone H3 where the lysine residue at position 28 is trimethylated. This modification is associated with formation of heterochromatin and polycomb repressive complex 1 (PRC1). H3K27me3 | chipseq_target C177614 chipseq_target C C177537 GDC Value Terminology C2851 Acquired Immunodeficiency Syndrome A syndrome resulting from the acquired deficiency of cellular immunity caused by the human immunodeficiency virus (HIV). It is characterized by the reduction of the Helper T-lymphocytes in the peripheral blood and the lymph nodes. Symptoms include generalized lymphadenopathy, fever, weight loss, and chronic diarrhea. Patients with AIDS are especially susceptible to opportunistic infections, tuberculosis, candida infections, and cryptococcosis), and the development of malignant neoplasms (usually non-Hodgkin lymphoma and Kaposi sarcoma). The human immunodeficiency virus is transmitted through sexual contact, sharing of contaminated needles, or transfusion of contaminated blood. HIV / AIDS | comorbidity C16457 comorbidity C C177537 GDC Value Terminology C3390 Stroke A sudden loss of neurological function secondary to hemorrhage or ischemia in the brain parenchyma due to a vascular event. Stroke | comorbidity C16457 comorbidity C C177537 GDC Value Terminology C34415 Beckwith-Wiedemann Syndrome A genetic syndrome caused by abnormalities in chromosome 11. It is characterized by large birth weight, macroglossia, umbilical hernia, ear abnormalities, and hypoglycemia. Patients with this syndrome have an increased risk of developing embryonal tumors (gonadoblastoma, hepatoblastoma, Wilms tumor, rhabdomyosarcoma) and adrenal cortex carcinomas. Beckwith-Wiedemann | comorbidity || Beckwith-Wiedemann | risk_factor C16457 || C17103 comorbidity || risk_factor C C177537 GDC Value Terminology C3476 Li-Fraumeni Syndrome An autosomal dominant cancer predisposition syndrome caused by germline mutations of the TP53 gene. It is associated with breast carcinoma, choroid plexus carcinoma, adrenal cortex carcinoma, astrocytic tumors, medulloblastoma, soft tissue sarcoma, osteosarcoma, and leukemia. Li-Fraumeni Syndrome | comorbidity || Li-Fraumeni Syndrome | risk_factor C16457 || C17103 comorbidity || risk_factor C C177537 GDC Value Terminology C3422 Trophoblastic Tumor A gestational or non-gestational neoplasm characterized by excessive proliferation of trophoblastic cells. Trophoblastic neoplasms | disease_type C2991 disease_type C C177537 GDC Value Terminology C14338 Human Papillomavirus-16 A virus comprised of a protein coat (capsid) surrounding a circular, double-stranded DNA organized into coding and non-coding regions, which can induce skin and mucosal epithelial lesions. Human papillomavirus-16 (HPV16) has been directly linked to cervical cancer and is significantly associated with invasiveness. Progression from low- to high-grade neoplasia is often associated with the integration of the HPV16 genome into the host chromosome. 16 | hpv_positive_type C157110 hpv_positive_type C C177537 GDC Value Terminology C14377 Human Papillomavirus-18 A virus comprised of a protein coat (capsid) surrounding a circular, double-stranded DNA organized into coding and non-coding regions, which can induce skin and mucosal epithelial lesions. Human papillomavirus-18 (HPV18) has been directly linked to cervical cancer and plays a role in the pathogenesis of the disease. The virus integrates its DNA at specific chromosomal locations, such as 8q24 and 12q15. The use of molecular markers for HPV18 infection may allow the identification of patients with early stage cervical cancer and those at high risk for disease recurrence. 18 | hpv_positive_type C157110 hpv_positive_type C C177537 GDC Value Terminology C15361 Fine-Needle Aspiration The removal of tissue or fluid with a thin needle for examination under a microscope. Fine Needle Aspiration | method_of_diagnosis || Fine Needle Aspiration | method_of_sample_procurement C177576 || C70700 method_of_diagnosis || method_of_sample_procurement C C177537 GDC Value Terminology C148647 Regulatory Region Deletion A deletion mutation where the deleted sequence includes a regulatory region for a gene. Regulatory Region Ablation | molecular_consequence C97926 molecular_consequence C C177537 GDC Value Terminology C35911 Papillary Growth Pattern A morphologic finding indicating the presence of a cellular infiltrate with papillary growth in a tissue sample. Papillary, NOS | morphologic_architectural_pattern C35886 morphologic_architectural_pattern C C177537 GDC Value Terminology C36181 Micropapillary Growth Pattern A morphologic finding indicating the presence of a growth pattern dominated by the presence of small papillary structures. Micropapillary | morphologic_architectural_pattern C35886 morphologic_architectural_pattern C C177537 GDC Value Terminology C129351 Supratentorial Ependymoma, ZFTA-RELA Fusion-Positive A supratentorial ependymoma characterized by a gene fusion involving ZFTA and RELA genes. It accounts for the majority of supratentorial ependymomas in children. It has an unfavorable outcome when compared to other ependymoma subtypes. 9396/3 | morphology || Ependymoma, RELA fusion-positive | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C129499 Embryonal Tumor with Multilayered Rosettes without C19MC Alteration An embryonal tumor with multilayered rosettes characterized by the absence of C19MC amplification. Approximately half of embryonal tumors with multilayered rosettes that lack a C19MC alteration carry DICER1 mutations. 9478/3 | morphology || Embryonal tumor with multilayered rosettes, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C27729 Thyroid Gland Well-Differentiated Tumor of Uncertain Malignant Potential An encapsulated or well-circumscribed thyroid gland tumor composed of well-differentiated follicular cells with well-developed or partially developed nuclear features of papillary thyroid carcinoma and with questionable capsular or vascular invasion. This is a tumor indeterminate between follicular adenoma and follicular carcinoma. Tumors in which vascular invasion has been excluded by all means are called non-invasive follicular thyroid neoplasms with papillary-like nuclear features. (WHO) 8330/1 | morphology || Atypical follicular adenoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C2932 Carotid Body Paraganglioma A parasympathetic paraganglioma arising from paraganglia adjacent to or in the bifurcation of the common carotid artery. Most patients present with a slow growing, painless mass in the neck. 8693/1 | morphology || Carotid body paraganglioma | primary_diagnosis || Carotid body tumor | primary_diagnosis || Chemodectoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3061 Jugulotympanic Paraganglioma An extra-adrenal parasympathetic paraganglioma arising from paraganglia in the base of the skull and middle ear. 8690/1 | morphology || Glomus jugulare tumor, NOS | primary_diagnosis || Jugular paraganglioma | primary_diagnosis || Jugulotympanic paraganglioma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3254 Angiomyxoma A benign soft tissue neoplasm characterized by the presence of neoplastic spindle and stellate cells, and vascular proliferation in a myxoid stroma. 8841/1 | morphology || Angiomyxoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3308 Paraganglioma A neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases. 8680/1 | morphology || Paraganglioma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3309 Extra-Adrenal Paraganglioma A paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland. 8693/1 | morphology || Extra-adrenal paraganglioma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3326 Adrenal Gland Pheochromocytoma A neuroendocrine neoplasm of the sympathetic nervous system that arises from the chromaffin cells of the adrenal medulla and secretes catecholamines. Clinical presentation includes headaches, palpitations, chest and abdominal pain, hypertension, fever, and tremor. Microscopically, a characteristic nesting (zellballen) growth pattern is usually seen. Other growth patterns including trabecular pattern may also be present. 8700/0 | morphology || Adrenal medullary paraganglioma | primary_diagnosis || Chromaffin paraganglioma | primary_diagnosis || Pheochromocytoma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3329 Pituitary Neuroendocrine Tumor A well-differentiated neuroendocrine tumor that arises from the adenohypophysial cells of the anterior lobe of the pituitary gland. The tumor can be hormonally functioning or not. It has a low frequency of metastatic spread. When metastatic, the term metastatic pituitary neuroendocrine tumor is endorsed instead of pituitary carcinoma. (WHO) 8272/0 | morphology || Pituitary adenoma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3342 Lactotroph Pituitary Neuroendocrine Tumor A pituitary neuroendocrine tumor that produces prolactin. 8271/0 | morphology || Prolactinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3502 Thyroid Gland Follicular Adenoma A benign, encapsulated neoplasm arising from the follicular cells of the thyroid gland. It may be associated with thyroid hormone secretion but it does not have malignant characteristics. 8330/0 | morphology || Follicular adenoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C3706 Medullomyoblastoma A rare malignant embryonal neoplasm arising from the cerebellum. It is characterized by the morphologic features of a medulloblastoma and the presence of a striated muscle component. Its clinical behavior is similar to medulloblastoma. 9472/3 | morphology || Medullomyoblastoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4127 Diffuse Type Adenocarcinoma An adenocarcinoma characterized by the presence of a diffuse cellular infiltrate which is composed of poorly cohesive cells with minimal or no glandular formations. Representative example is the gastric diffuse adenocarcinoma. 8145/3 | morphology || Adenocarcinoma, diffuse type | primary_diagnosis || Carcinoma, diffuse type | primary_diagnosis || Poorly cohesive carcinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4216 Sympathetic Paraganglioma A paraganglioma arising from the chromaffin cells of the paraganglia that are located along the sympathetic nerves. It includes extra-adrenal paragangliomas and paragangliomas that arise from the adrenal medulla. The latter are commonly referred to as pheochromocytomas. Representative examples of extra-adrenal sympathetic paragangliomas include the bladder, and superior and inferior paraaortic paragangliomas. Clinical signs are related to the secretion of catecholamines resulting in hypertension. 8700/0 | morphology || Chromaffin tumor | primary_diagnosis || Chromaffinoma | primary_diagnosis || Sympathetic paraganglioma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4217 Parasympathetic Paraganglioma An extra-adrenal paraganglioma that arises from paraganglia located along the parasympathetic nerves. Representative examples include aorticopulmonary, carotid body, jugulotympanic, and mediastinal paragangliomas. 8682/1 | morphology || Parasympathetic paraganglioma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4218 Aorticopulmonary Paraganglioma An extra-adrenal parasympathetic paraganglioma that arises from paraganglia adjacent to the base of the heart and great vessels. 8691/1 | morphology || Aortic body paraganglioma | primary_diagnosis || Aortic body tumor | primary_diagnosis || Aorticopulmonary paraganglioma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4219 Metastatic Extra-Adrenal Paraganglioma An extra-adrenal paraganglioma that metastasizes to regional or distant anatomic sites. Common sites of metastasis include the lymph nodes, lungs, bones, and liver. 8693/3 | morphology || Extra-adrenal paraganglioma, malignant | primary_diagnosis || Nonchromaffin paraganglioma, malignant | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4220 Metastatic Adrenal Gland Pheochromocytoma A pheochromocytoma that metastasizes to other anatomic sites. Common sites of metastasis include lymph nodes, bones, liver, and lung. Morphologic features associated with malignant pheochromocytomas include: atypical mitotic figures, capsular and vascular invasion, tumor cell necrosis, and high mitotic activity. 8700/3 | morphology || Adrenal medullary paraganglioma, malignant | primary_diagnosis || Pheochromoblastoma | primary_diagnosis || Pheochromocytoma, malignant | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4291 Thyroid Carcinoma Arising in Struma Ovarii A carcinoma with histological features identical to thyroid carcinoma, arising in ovarian mature teratoma with aberrant thyroid tissue (struma ovarii). 9090/3 | morphology || Struma ovarii, malignant | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4324 Astroblastoma, MN1-Altered A rare glial neoplasm characterized by structural rearrangements of the MN1 gene at chromosome band 22q12.1. It is usually found in the cerebral hemispheres of young adults and children and predominantly affects females. Morphologically, it consists of elongated glial cells with abundant eosinophilic cytoplasm and GFAP-positive processes, arranged perivascularly. 9430/3 | morphology || Astroblastoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4536 Metastatic Pituitary Neuroendocrine Tumor Pituitary neuroendocrine tumor that has spread from its original site of growth to another anatomic site. 8272/3 | morphology || Pituitary carcinoma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C46105 Thyroid Gland Spindle Epithelial Tumor with Thymus-Like Elements A rare, slow growing malignant tumor of the thyroid gland arising from intrathyroidal thymic tissue. It is characterized by a lobulated architectural pattern and the presence of a biphasic cellular population composed of spindle epithelial cells and glandular cells. A small number of cases are composed exclusively of spindle epithelial cells or glandular cells. 8588/3 | morphology || SETTLE | primary_diagnosis || Spindle epithelial tumor with thymus-like differentiation | primary_diagnosis || Spindle epithelial tumor with thymus-like element | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C48314 Non-Metastatic Paraganglioma A paraganglioma that is confined to the site of origin. 8680/0 | morphology || Paraganglioma, benign | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C4915 Embryonal Tumor with Multilayered Rosettes, C19MC-Altered An aggressive embryonal tumor with multilayered rosettes characterized by the presence of amplification of the C19MC region on chromosome 19 (19q13.42). 9392/3 | morphology || 9478/3 | morphology || Embryonal tumor with multilayered rosettes C19MC-altered | primary_diagnosis || Ependymoblastoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C5398 Central Nervous System Embryonal Tumor, Not Otherwise Specified A term that refers to central nervous system embryonal tumors which are not fully characterized. 9473/3 | morphology || CPNET | primary_diagnosis || Central primitive neuroectodermal tumor, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C5592 Chordoid Glioma A rare, slow-growing neuroepithelial neoplasm of uncertain origin affecting adults. It is located in the third ventricle. It is characterized by the presence of epithelioid cells which express GFAP, and mucinous stroma which contains lymphoplasmacytic infiltrates. 9444/1 | morphology || Chordoid glioma | primary_diagnosis || Chordoid glioma of third ventricle | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C6040 Poorly Differentiated Thyroid Gland Carcinoma A follicular-derived thyroid gland carcinoma that is histologically poorly differentiated and has high-grade features. 8337/3 | morphology || Insular carcinoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C6042 Thyroid Gland Oncocytic Adenoma A thyroid gland adenoma composed of large cells with abundant granular eosinophilic cytoplasm and large nuclei with prominent nucleoli. 8290/0 | morphology || Hurthle cell adenoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C65200 Thyroid Gland Follicular Carcinoma, Minimally Invasive A follicular carcinoma of the thyroid gland showing capsular invasion only. 8335/3 | morphology || Follicular carcinoma, minimally invasive | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C66850 Invasive Encapsulated Follicular Variant Thyroid Gland Papillary Carcinoma A papillary carcinoma of the thyroid gland which is encapsulated and resembles an encapsulated follicular neoplasm. Capsular invasion is present. The malignant follicular cells display the nuclear features that characterize the papillary adenocarcinomas of the thyroid gland. 8509/3 | morphology || Encapsulated papillary carcinoma with invasion | primary_diagnosis || Invasive encapsulated follicular variant of papillary thyroid carcinoma (invasive EFVPTC) | primary_diagnosis || Solid papillary carcinoma with invasion | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C6967 Pineal Parenchymal Tumor of Intermediate Differentiation A WHO grade 2 or 3 pineal parenchymal neoplasm of intermediate-grade malignancy, affecting all ages. It is composed of diffuse sheets or large lobules of uniform cells with mild to moderate nuclear atypia and low to moderate level mitotic activity. (Adapted from WHO) 9362/3 | morphology || Pineal parenchymal tumor of intermediate differentiation | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C7468 Struma Ovarii An ovarian mature teratoma characterized by the presence of aberrant thyroid tissue. 9090/0 | morphology || Struma ovarii, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C8559 Metastatic Paraganglioma A paraganglioma that metastasizes to regional or distant anatomic sites. Extraadrenal paragangliomas have a higher tendency to metastasize, as compared to pheochromocytomas. Common sites of metastasis include the lymph nodes, lungs, bones, and liver. 8680/3 | morphology || Paraganglioma, malignant | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C8997 Blastoma A rare embryonal neoplasm affecting children. It is associated with DICER1 gene mutation. This category includes pituitary gland blastoma and pleuropulmonary blastoma. 8000/3 | morphology || Blastoma, NOS | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C9497 Melanocytic Medulloblastoma A rare malignant embryonal neoplasm characterized by the presence of small cells which resemble the cells of classic medulloblastoma and a minor population of melanin-forming neuroepithelial cells. It usually has an unfavorable clinical course. 9470/3 | morphology || Melanotic medulloblastoma | primary_diagnosis C176985 || C177621 morphology || primary_diagnosis C C177537 GDC Value Terminology C126598 Thyroid Gland Noninvasive Follicular Neoplasm with Papillary-Like Nuclear Features A non-invasive neoplasm of thyroid follicular cells with a follicular growth pattern and nuclear features of papillary thyroid carcinoma that has an extremely low malignant potential. These tumors were formerly classified as non-invasive encapsulated follicular variant of papillary thyroid carcinoma or well-differentiated tumor of uncertain malignant potential. (WHO) Non-invasive EFVPTC | primary_diagnosis || Non-invasive FTP | primary_diagnosis || Non-invasive encapsulated follicular variant of papillary thyroid carcinoma (non-invasive EFVPTC) | primary_diagnosis || Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C129527 Central Nervous System Solitary Fibrous Tumor, Grade 3 A solitary fibrous tumor that arises from the central nervous system. It most often corresponds to the tumor previously diagnosed as anaplastic hemangiopericytoma. Solitary fibrous tumor/hemangiopericytoma Grade 3 (CNS) | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C129528 Central Nervous System Solitary Fibrous Tumor, Grade 2 A solitary fibrous tumor that arises from the central nervous system. It corresponds to the more cellular, less collagenous tumor with plump cells and staghorn vasculature which was diagnosed as central nervous system hemangiopericytoma in the past. Solitary fibrous tumor/hemangiopericytoma Grade 2 (CNS) | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C129530 Central Nervous System Solitary Fibrous Tumor, Grade 1 A solitary fibrous tumor that arises from the central nervous system. It corresponds most often to the collagenous and low cellularity spindle cell tumor which was diagnosed as central nervous system solitary fibrous tumor in the past. Solitary fibrous tumor/hemangiopericytoma Grade 1 (CNS) | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C27281 L2 Acute Lymphoblastic Leukemia An antiquated term that refers to acute lymphoblastic leukemia with large and irregular lymphoblasts. Acute lymphoblastic leukemia, L2 type, NOS | primary_diagnosis || FAB L2 | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C27780 Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable This entity includes cases that have clinical, laboratory, and morphologic features that support the diagnosis of both a myelodysplastic syndrome and a myeloproliferative neoplasm, but do not meet the criteria for any of the other entities included in the myelodysplastic/myeloproliferative neoplasm category. (WHO, 2001) Myelodysplastic/myeloproliferative neoplasm, unclassifiable | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C46068 Thyroid Gland Oncocytic Neoplasm An adenoma or carcinoma arising from the follicular cells of the thyroid gland. It is composed of large oncocytic cells with abundant granular eosinophilic cytoplasm. Hurthle cell tumor | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C94537 Spindle Cell Oncocytoma A very rare, WHO grade 1 neoplasm of the posterior pituitary. It is characterized by the presence of spindle cells with eosinophilic, granular cytoplasm forming fascicles. Electron microscopic studies demonstrate the accumulation of intracytoplasmic mitochondria and lack of secretory granules. Immunohistochemical studies are negative for pituitary hormones. Patients may present with pituitary hypofunction, visual disturbances, headache, nausea and vomiting. The clinical course is usually benign. Spindle cell oncocytoma | primary_diagnosis C177621 primary_diagnosis C C177537 GDC Value Terminology C4815 Thyroid Gland Carcinoma A carcinoma arising from the thyroid gland. It includes the following main subtypes: follicular, papillary, medullary, poorly differentiated, and anaplastic carcinoma. Thyroid Cancer | relationship_primary_diagnosis C178243 relationship_primary_diagnosis C C177537 GDC Value Terminology C9382 Rectal Carcinoma A malignant epithelial neoplasm that arises from the rectum. The vast majority are adenocarcinomas. Rectal Cancer | relationship_primary_diagnosis C178243 relationship_primary_diagnosis C C177537 GDC Value Terminology C5541 High Grade Prostatic Intraepithelial Neoplasia Prostatic intraepithelial neoplasia characterized by the presence of morphologically malignant cells lining the acini and ducts. The malignant cells are enlarged, contain prominent nucleoli, and have increased nuclear/cytoplasmic ratio. The malignant cells may grow within the ducts and acini in four architectural patterns: flat, tufting, micropapillary, and cribriform. The presence of high grade prostatic intraepithelial neoplasia in needle biopsy is a risk factor for the subsequent detection of carcinoma on repeat biopsy. High-grade Prostatic Intraepithelial Neoplasia (PIN) | risk_factor C17103 risk_factor C C177537 GDC Value Terminology C98096 PIERCE2 Gene This gene may play a role in microtubule structure and ciliary motility. C15orf65 | gene_symbol || C15orf65 | second_gene_symbol C171253 || C173595 second_gene_symbol || gene_symbol C C177537 GDC Value Terminology C12366 Bone The structural organ comprised of specialized connective tissue that forms the skeletal components of the body. Bone | biospecimen_anatomic_site || Bone | metastasis_at_diagnosis_site || Bone | treatment_anatomic_site || Bone, NOS | progression_or_recurrence_anatomic_site || Bone, NOS | site_of_resection_or_biopsy || Bone, NOS | tissue_or_organ_of_origin C156421 || C156422 || C171435 || C177570 || C177625 || C70729 site_of_resection_or_biopsy || tissue_or_organ_of_origin || biospecimen_anatomic_site || progression_or_recurrence_anatomic_site || metastasis_at_diagnosis_site || treatment_anatomic_site C C177537 GDC Value Terminology C12664 Abdomen The cavity located between the lungs and pelvis. It contains the lower esophagus, stomach, pancreas, intestines, liver, gallbladder and spleen. Abdomen | biospecimen_anatomic_site || Abdomen | metastasis_at_diagnosis_site || Abdomen, NOS | progression_or_recurrence_anatomic_site || Abdomen, NOS | site_of_resection_or_biopsy || Abdomen, NOS | tissue_or_organ_of_origin || Abdomen, total | treatment_anatomic_site C156421 || C156422 || C171435 || C177570 || C177625 || C70729 site_of_resection_or_biopsy || tissue_or_organ_of_origin || biospecimen_anatomic_site || progression_or_recurrence_anatomic_site || metastasis_at_diagnosis_site || treatment_anatomic_site C C177537 GDC Value Terminology C54187 Floor of Mouth The horseshoe-shaped part of the oral cavity bounded superiorly by the ventral surface of the tongue, inferiorly by the mylohyoid muscle, anterolaterally by the gingival surfaces of the mandible, and posteriorly by the anterior tonsillar pillar. Floor Of Mouth | biospecimen_anatomic_site || Floor of Mouth | treatment_anatomic_site || Floor of mouth | primary_site || Floor of mouth, NOS | progression_or_recurrence_anatomic_site || Floor of mouth, NOS | site_of_resection_or_biopsy || Floor of mouth, NOS | tissue_or_organ_of_origin C156421 || C156422 || C158874 || C171435 || C177570 || C70729 site_of_resection_or_biopsy || tissue_or_organ_of_origin || primary_site || biospecimen_anatomic_site || progression_or_recurrence_anatomic_site || treatment_anatomic_site C C177537 GDC Value Terminology C106250 Atezolizumab A humanized, Fc optimized, monoclonal antibody directed against the protein ligand PD-L1 (programmed cell death-1 ligand 1; CD274), with potential immune checkpoint inhibitory and antineoplastic activities. Atezolizumab binds to PD-L1, blocking its binding to and activation of its receptor programmed death 1 (PD-1; PDCD1) expressed on activated T-cells, which may enhance the T-cell-mediated immune response to neoplasms and reverse T-cell inactivation. In addition, by binding to PD-L1, atezolizumab also prevents binding of this ligand to B7.1 (CD80) expressed on activated T cells, which further enhances the T-cell-mediated immune response. PD-L1 is overexpressed on many human cancer cell types and on various tumor-infiltrating immune cells. PD-L1 binding to PD-1 on T-cells suppresses the immune system and results in increased immune evasion. PD-1, a transmembrane protein, is a negative regulator of the immune system that limits the expansion and survival of CD8+ T cells. The Fc region of atezolizumab is modified in such a way that it does not induce either antibody-dependent cytotoxicity (ADCC) or complement-dependent cytotoxicity (CDC). Atezolizumab | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C106254 Tovorafenib An orally available inhibitor of wild-type and certain mutant forms of A-Raf, B-Raf and C-Raf protein kinases, with potential antineoplastic activity. Upon administration, tovorafenib inhibits Raf-mediated signal transduction pathways, which may lead to an inhibition of tumor cell growth. Raf protein kinases play a key role in the RAF/MEK/ERK signaling pathway, which is often deregulated in human cancers and plays a key role in tumor cell proliferation and survival. pan-RAF Kinase Inhibitor TAK-580 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C116068 Aurora A Kinase Inhibitor VIC-1911 An orally bioavailable inhibitor of the serine/threonine protein kinase aurora A, with potential antimitotic and antineoplastic activities. Upon intravenous administration, aurora A kinase inhibitor VIC-1911 binds to and inhibits aurora A kinase, which may result in disruption of the assembly of the mitotic spindle apparatus, disruption of chromosome segregation, inhibition of cell division and the induction of apoptosis in cells overexpressing aurora A kinase. Aurora A kinase localizes to the spindle poles and to spindle microtubules during mitosis; it plays an essential role in the regulation of spindle assembly. Aurora kinase A is overexpressed in a wide variety of cancers. Aurora A Kinase Inhibitor TAS-119 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C118450 Utidelone A genetically engineered epothilone analog with potential antineoplastic activity. Upon administration, utidelone binds to tubulin, induces microtubule polymerization and stabilizes microtubules against depolymerization, which may result in the inhibition of cell division, the induction of G2/M arrest, and apoptosis. Compared to first-generation epothilones, this agent exhibits greater safety and enhanced activity against certain multidrug-resistant (MDR) tumors. Epothilone Analog UTD1 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C120210 Topical Tirbanibulin An ointment containing an inhibitor for both Src tyrosine kinase and tubulin polymerization, with potential antineoplastic activity. Unlike other Src kinase inhibitors which bind to the ATP-binding site, tirbanibulin binds to the peptide substrate binding site of Src kinase, upon topical application. This inhibits both downstream signaling and the proliferation of tumor cells overexpressing Src. Src tyrosine kinase, a non-receptor tyrosine kinase upregulated in many tumor cell types, plays an important role in tumor cell proliferation, motility, invasiveness and survival. Tirbanibulin also binds to tubulin heterodimers and inhibits microtubule polymerization, which disrupts microtubule formation and mitosis, leading to further inhibition of cell proliferation. In addition, Tirbanibulin inhibits T-cell migration. Src Kinase Inhibitor KX2-391 Ointment | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C121850 Cetuximab Sarotalocan Sodium The sodium salt form of cetuximab sarotalocan, which consists of a chemical conjugate composed of the dye IR700 linked to cetuximab, a monoclonal antibody directed against the epidermal growth factor receptor (EGFR), with potential antineoplastic activity. Upon injection of cetuximab sarotalocan sodium, the cetuximab moiety targets and binds to EGFR-expressing tumor cells, resulting in the internalization of the conjugate. Upon localized application of near-infrared (NIR) light, the IR700 dye becomes activated, disrupts the cell membrane and selectively kills the EGFR-expressing tumor cells. EGFR, a tyrosine kinase receptor, is overexpressed in a variety of cancers. Cetuximab Sarotalocan | therapeutic_agents || Cetuximab-IR700 Conjugate RM-1929 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C126646 Padnarsertib An orally bioavailable inhibitor of both the serine/threonine kinase P21-activated kinase 4 (PAK4) and the nicotinamide adenine dinucleotide (NAD)-synthesizing enzyme nicotinamide phosphoribosyltransferase (NAMPT; NAMPRTase), with potential antineoplastic activity. Upon administration, padnarsertib allosterically binds to, destabilizes and causes degradation of PAK4. This inhibits PAK4-mediated signaling, induces cell death in, and inhibits the proliferation of PAK4-overexpressing tumor cells. In addition, padnarsertib binds to and inhibits the activity of NAMPT. This depletes cellular NAD and inhibits NAD-dependent enzymes, both of which are needed for rapid cell proliferation; this results in tumor cell death in NAMPT-overexpressing cancer cells. PAK4, a serine/threonine kinase and member of the PAK family of proteins upregulated in various cancer cell types, regulates cell motility, proliferation and survival. NAMPT, an enzyme that is responsible for maintaining the intracellular NAD pool, plays a key role in the regulation of cellular metabolism and has cytokine-like activities. NAMPT is often overexpressed in a variety of cancers and metabolic disorders and tumor cells rely on NAMPT activity for their NAD supply. PAK4/NAMPT Inhibitor KPT-9274 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C148134 Safusidenib An orally available inhibitor of isocitrate dehydrogenase type 1 (IDH1; IDH-1; IDH1 [NADP+] soluble) mutant forms, including substitution mutations at the arginine in position 132, IDH1(R132) (IDH1-R132), with potential antineoplastic activity. Upon oral administration, safusidenib specifically binds to and inhibits certain mutant forms of IDH1, thereby inhibiting the formation of the oncometabolite 2-hydroxyglutarate (2HG) from alpha-ketoglutarate (a-KG). This prevents 2HG-mediated signaling and leads to both an induction of cellular differentiation and an inhibition of cellular proliferation in tumor cells expressing IDH1 mutations. IDH1(R132) mutations are highly expressed in certain malignancies, including gliomas; they initiate and drive cancer growth by both blocking cell differentiation and catalyzing the formation of 2HG. Safusidenib minimally targets and affects wild-type IDH1, which is expressed in normal, healthy cells. Mutant IDH1 Inhibitor DS-1001 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C153137 Orludodstat An orally available inhibitor of dihydroorotate dehydrogenase (DHODH), with potential antineoplastic activity. Upon administration, orludodstat specifically targets, binds to and prevents the activation of DHODH, thereby preventing the fourth enzymatic step in de novo pyrimidine synthesis. This prevents uridine monophosphate (UMP) formation, DNA synthesis, cell division and cellular proliferation, causes reactive oxygen species (ROS) production, enables differentiation and induces apoptosis in susceptible tumor cells. DHODH, a mitochondrial enzyme, catalyzes the conversion of dihydroorotate (DHO) to orotate in the endogenous synthesis of UMP. Dihydroorotate Dehydrogenase Inhibitor BAY2402234 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C156399 Emirodatamab A bispecific T-cell engager (BiTE) antibody composed of two single-chain variable fragments (scFv), one directed against the tumor-associated antigen (TAA) FLT3 tyrosine kinase receptor (Fms-like tyrosine kinase 3; FLT3; FLT-3; CD135; fetal liver kinase-2; FLK2), fused to one that is directed against the CD3 antigen found on T-lymphocytes, with potential immunostimulating and antineoplastic activities. Upon administration, emirodatamab binds to both CD3 on cytotoxic T-lymphocytes (CTLs) and FLT3 found on FLT3-expressing tumor cells. This activates and redirects CTLs to FLT3-expressing tumor cells, which results in the CTL-mediated cell death of FLT3-expressing tumor cells. FLT3, a cytokine receptor belonging to the class III tyrosine kinase receptors, is overexpressed or mutated in most B-lineage and acute myeloid leukemias (AMLs). Anti-FLT3/CD3 BiTE Antibody AMG 427 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C158096 Deutenzalutamide A deuterated form of enzalutamide, an orally bioavailable, organic, non-steroidal small molecule targeting the androgen receptor (AR) with potential antineoplastic activity. Upon administration, deutenzalutamide competitively binds to and inhibits the activity of ARs expressed on prostate cancer cells, which impairs nuclear translocation and DNA binding, resulting in apoptosis of prostate cancer cells. This results in a reduction in prostate cancer cell growth. AR overexpression in prostate cancer represents a key mechanism associated with prostate cancer hormone resistance. Deuterium incorporation, by replacing the hydrogen atoms of the N-CH3 moiety with deuterium atoms, decreases enzalutamide's metabolism and allows for an increased pharmacokinetic profile, thereby enhancing its anti-tumor efficacy compared to non-deuterated enzalutamide. As the deuterated form can't cross the blood-brain barrier (BBB), the deutenzalutamide form also reduces the unwanted brain-related side effects of enzalutamide and improves its safety profile. Deuterated Enzalutamide | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C158603 Izorlisib An orally bioavailable inhibitor of the class I phosphatidylinositol-4,5-bisphosphate 3-kinase (PI3K) catalytic subunit alpha (PIK3CA), with potential antineoplastic activity. Upon administration, izorlisib selectively binds to and inhibits PIK3CA and its mutated forms in the PI3K/Akt (protein kinase B)/mammalian target of rapamycin (mTOR) pathway. This results in both apoptosis and growth inhibition in PIK3CA-expressing tumor cells. By specifically targeting PIK3CA, izorlisib may be more efficacious and less toxic than pan-PI3K inhibitors. In addition, izorlisib also targets mutated forms of PI3K gamma (PI3Kg). It may also stimulate the immune system to restore CD8+ T-cell activation and cytotoxicity. Dysregulation of the PI3K/Akt/mTOR pathway is often found in solid tumors and results in the promotion of tumor cell growth, survival, and resistance to chemo- and radio-therapy. PIK3CA, one of the most frequently mutated oncogenes, encodes the p110-alpha catalytic subunit of the class I PI3K. In most solid tumors, the activation of the PI3K pathway is induced by mutations of PIK3CA. PI3K-alpha Inhibitor MEN1611 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C159495 Vobramitamab Duocarmazine An antibody-drug conjugate (ADC) comprised of vobamitamab, an anti-B7-homolog 3 (B7-H3, CD276) humanized immunoglobulin G1 (IgG1)/kappa monoclonal antibody, conjugated to the cleavable linker-duocarmycin payload duocarmazine (valine-citrulline-seco duocarmycin hydroxybenzamide azaindole; vc-seco-DUBA), with potential antineoplastic activity. Upon administration of vobramitamab duocarmazine, vobramitamab specifically targets and binds to the cell surface antigen B7-H3, leading to internalization of the ADC by B7-H3-expressing tumor cells. The vc linker is cleaved inside the tumor cell by proteases, thereby releasing the duocarmycin payload. Duocarmycin binds to the minor groove of DNA, alkylates adenine at the N3 position, and induces cell death. B7-H3, a type I transmembrane protein and a member of the B7 co-stimulatory protein superfamily, is overexpressed on certain tumor cell types and on various immune cells but is minimally expressed by normal human tissues. B7-H3 is a negative regulator of T-cell activation and its overexpression plays a key role in immuno-evasion, tumor cell invasion and metastasis, and its expression is correlated with poor prognosis. Anti-B7H3 Antibody-drug Conjugate MGC018 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C162532 Tinengotinib An orally available small molecule inhibitor of Aurora kinases (AKs) A and B, Janus kinases (JAKs), fibroblast growth factor receptors (FGFRs) and vascular endothelial growth factor receptors (VEGFRs), with potential antineoplastic and immunomodulatory activities. Upon oral administration, tinengotinib selectively binds to and inhibits AKs A and B, which inhibit cell division in tumor cells that overexpress AKs. Tinengotinib also targets JAKs that are involved in cytokine signaling and inflammation, and FGFRs and VEGFRs, which are overexpressed in the microenvironment (TME) and contribute to neovascularization, tumor growth and metastasis. These kinases are overexpressed by a wide variety of cancer cell types and drive tumor cell proliferation. Aurora kinase A/B inhibitor TT-00420 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C162645 Pegenzileukin A pegylated recombinant, engineered variant of cytokine interleukin-2 (IL-2; IL2) where novel amino acid is encoded in the IL-2 gene that is leveraged for use in site-specific pegylation, with potential immunostimulating activity. Upon administration of pegenzileukin, the IL-2 variant moiety binds to dimers containing the IL-2 receptor beta and gamma chains (IL2Rbg; IL2Rbetagamma) on immune cells, such as cytotoxic T-lymphocytes (CTLs) and natural killer (NK) cells, thereby activating these cells and inducing their expansion. It also induces the expression of certain cytotoxic cytokines, such as interferon-gamma (IFNg) and transforming growth factor-beta (TGFb). The specific induction of T-cell-mediated cytotoxic immune responses against tumor cells causes tumor cell destruction. The addition of the novel amino acid and the concurrent pegylation prevents the binding of the IL-2 moiety to the IL-2 receptor alpha chain (IL2Ra), thereby blocking the IL2Ra-mediated activation of CD4-positive regulatory and immunosuppressive T-cells (Tregs). Since it cannot bind to IL2Ra expressed on innate lymphoid cells in the vascular endothelium, pegenzileukin prevents IL-2-mediated recruitment and activation of eosinophils, and inhibits the induction of eosinophil-mediated vascular leak syndrome (VLS). Pegylation also extends the half-life of pegenzileukin. Compared to recombinant IL-2, pegenzileukin allows for increased IL-2Rbg binding and proliferation of CTLs without stimulating the toxicity caused by binding to IL-2Ra. Pegylated Recombinant Interleukin-2 THOR-707 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C165166 Imvotamab An engineered immunoglobulin M (IgM) bispecific antibody, with potential antineoplastic activity. Imvotamab contains ten high affinity binding domains for the tumor-associated antigen (TAA) CD20, and one binding domain for CD3, a T-cell surface antigen. Upon administration, imvotamab binds to both T-cells and CD20-expressing B-lineage tumor cells. The resulting cross-linkage may trigger a potent cytotoxic T-lymphocyte (CTL) response against the CD20-expressing tumor B-cells. Additionally, imvotamab induces complement-dependent cytotoxicity (CDC) to a greater extent than anti-CD20/anti-CD3 IgG bispecific antibodies, thereby further enhancing the killing CD20-expressing tumor cells. The extra binding units of imvotamab may bind cancer cells that express relatively low amounts of CD20. Also, compared to IgG format bispecific T-cell engaging antibodies, imvotamab appears to induce less cytokine release, which may reduce the risk of cytokine release syndrome (CRS). CD20 is exclusively expressed on B-cells during most stages of B-cell development and is often overexpressed in B-cell malignancies. Anti-CD20/Anti-CD3 Bispecific IgM Antibody IGM2323 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C167205 Sunvozertinib An orally available, irreversible, dual kinase inhibitor of epidermal growth factor receptor (EGFR) and human epidermal growth factor receptor 2 (HER2) that shows similar activity against certain activating mutations, including exon 20 insertions (exon20ins), with potential antineoplastic activity. Upon oral administration,sunvozertinib binds to and inhibits EGFR and HER2, which may result in the inhibition of tumor growth and angiogenesis, and tumor regression in EGFR/HER2-expressing tumors. EGFR and HER2 are receptor tyrosine kinases that play major roles in tumor cell proliferation and tumor vascularization. In contrast to other agents active against exon20ins mutations, sunvozertinib appears to be more selective against mutated EGFR than wild-type (wt) EGFR. This may lessen wtEGFR-related dose-limiting toxicity and may allow for the administration of the desired therapeutic dose of sunvozertinib. EGFR/HER2 Inhibitor DZD9008 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C170516 Interleukin-15/Interleukin-15 Receptor Alpha Sushi+ Domain Fusion Protein SOT101 A human fusion protein consisting of the cytokine interleukin (IL)-15 and the high-affinity binding sushi+ domain of IL-15 receptor alpha (IL-15Ra), with potential antineoplastic activities. Upon administration, SOT101 activates and increases the levels of natural killer (NK) cells and memory CD8+ T-cells. The memory T-cells enhance the secretion of the cytokine interferon-gamma (IFN-g), which further potentiates the immune response against tumor cells. This may increase tumor cell killing and decrease tumor cell proliferation. IL-15 regulates CD8+ T and NK cell development, activation and proliferation. SOT101 is more potent than unmodified IL-15 and does not require endogenous IL-15Ra for its action. Interleukin-15/Interleukin-15 Receptor Alpha Sushi+ Domain Fusion Protein SO-C101 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C171059 Tuxobertinib An orally bioavailable, irreversible, selective, small-molecule inhibitor of certain oncogenic driver, allosteric mutations of the ErbB receptor tyrosine kinases epidermal growth factor receptor (EGFR/ErbB1) and human epidermal growth factor receptor 2 (HER2/neu or ErbB2), including extracellular domain allosteric mutations of HER2, and EGFR and HER2 exon 20 insertion mutations, with potential antineoplastic activity. Upon oral administration, tuxobertinib selectively binds to and inhibits these allosteric ErbB mutants while sparing wild-type EGFR, which may result in the selective inhibition of cellular proliferation and angiogenesis in tumor cells and tumors expressing these allosteric ErbB mutations. EGFR and HER2, ErbB receptor tyrosine kinases mutated or overexpressed in many tumor cell types, play a key role in tumor cell proliferation and tumor vascularization. Allosteric ErbB Inhibitor BDTX-189 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C172056 Boserolimab A humanized agonistic monoclonal antibody targeting the cell surface antigen CD27, with potential immunostimulatory and antineoplastic activities. Upon administration, boserolimab targets and binds to CD27 on a variety of immune cell types, including most T-lymphocytes. This induces CD27-dependent signaling pathways and enhances T-cell-mediated responses, including the expansion of antigen-activated T-cells and the cytotoxic T-lymphocyte (CTL)-mediated immune response against tumor cells. CD27, a co-stimulatory molecule and member of the tumor necrosis factor receptor superfamily (TNFRSF), is expressed on T-lymphocytes, memory B-cells and natural killer (NK) cells. It plays an important role in NK cell-mediated cytolytic activity and T- and B-lymphocyte proliferation and activation. It is overexpressed in certain tumor cell types. Anti-CD27 Agonistic Monoclonal Antibody MK-5890 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C172819 Danburstotug A human monoclonal antibody directed against the immunosuppressive ligand programmed cell death-1 ligand 1 (PD-L1; cluster of differentiation 274; CD274), with potential immune checkpoint inhibitory and antineoplastic activities. Upon administration, danburstotug specifically targets and binds to PD-L1, blocking its binding to and activation of its receptor programmed cell death 1 (PD-1; cluster of differentiation 279; CD279). This reverses T-cell inactivation caused by PD-1/PD-L1 signaling and enhances the cytotoxic T-lymphocyte (CTL)-mediated anti-tumor immune response against PD-L1-expressing tumor cells. In addition, anti-PD-L1 monoclonal antibody IMC-001 also induces antibody-dependent cell-mediated cytotoxicity (ADCC). PD-L1 is overexpressed by many human cancer cell types. PD-L1 binding to PD-1 on T-cells suppresses the immune system and results in immune evasion. PD-1, a transmembrane protein belonging to the immunoglobulin superfamily (IgSF) expressed on activated T-cells, is a negative regulator of the immune system that limits the expansion and survival of CD8-positive T-cells. Anti-PD-L1 Monoclonal Antibody IMC-001 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C61493 Gebasaxturev A preparation of naturally occurring, oncolytic enterovirus, with potential antineoplastic activity. Upon administration, gebasaxturev targets and binds to intracellular adhesion molecule 1 (ICAM-1) and decay acceleration factor (DAF), both cell surface molecules that are overexpressed on certain malignant cells. After entering the cells, gebasaxturev replicates in these cancer cells, thereby causing cancer cell lysis. This results in a reduction of tumor cell growth. Coxsackievirus A21 | therapeutic_agents || Coxsackievirus V937 | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C77370 Alestramustine The l-alanine ester form of estramustine, a combination of the nitrogen mustard normustine coupled via a carbamate to estradiol, with antineoplastic activity. Upon conversion of alestramustine to estramustine, estramustine binds to microtubule-associated proteins (MAPs) and beta tubulin, thereby interfering with microtubule dynamics and leading to microtubule disassembly and cell cycle arrest. Due to the estrogen moiety, this agent is able to selectively bind to and be taken up by estrogen receptor-positive cells. Alestramustine | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C78082 Clanfenur A substituted benzoylphenylurea and an analogue of the pesticide diflubenzuron with potential antineoplastic activity. Upon administration, clanfenur binds to the colchicine-binding site on beta-tubulin and inhibits the polymerization of microtubules, thereby preventing tumor cell replication. Clanfenur | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C78837 Pacritinib An orally bioavailable inhibitor of Janus kinase 2 (JAK2), the JAK2 mutant JAK2V617F and FMS-like tyrosine kinase 3 (FLT3; CD135; STK1; FLK2), with potential antineoplastic activity. Upon oral administration, pacritinib competes with JAK2 and the JAK2 mutant JAK2V617F for ATP binding, which may result in inhibition of JAK2 activation, inhibition of the JAK-signal transducer and activator of transcription (STAT) signaling pathway, and the induction of apoptosis. In addition, pacritinib targets, binds to and inhibits the activity of FLT3. This inhibits FLT3-mediated signaling and the proliferation of FLT3-expressing cancer cells. JAK2, often upregulated or mutated in a variety of cancer cells, plays a key role in tumor cell proliferation and survival. The JAK2V617F gain-of-function mutation involves a valine-to-phenylalanine modification at position 617. The JAK-STAT signaling pathway is a major mediator of cytokine activity. FLT3, a class III receptor tyrosine kinase (RTK), is overexpressed or mutated in most B-lineage neoplasms and in acute myeloid leukemias. In addition, JAK2 and FLT3 play a key role in the regulation of the inflammatory response and dendritic cell (DC) proliferation, differentiation and function. Inhibition of JAK2- and FLT3-mediated signaling may suppress the generation and differentiation of DCs, and may regulate inflammatory and immune responses. Pacritinib | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C95202 Pixatimod A synthetic sugar modified heparan sulfate mimetic and agonist of toll-like receptor 9 (TLR9), with potential immunostimulating, antineoplastic and anti-viral activities. Upon administration, pixatimod binds to and activates TLR9 expressed by dendritic cells (DCs) and B-cells. This initiates cytokine release from DCs and activates innate immune signaling pathways, and leads to the activation of natural killer (NK) cells to destroy tumor cells. The combination of pixatimod with certain checkpoint inhibitors may enhance the activation of cytotoxic T-lymphocytes to further kill tumor cells. In addition, pixatimod inhibits the cleavage of heparan sulfate from cell surface proteoglycan by heparanase (HPSE) and inhibits the infiltration of tumor-associated macrophages (TAMs). TLR9, a member of the TLR family, plays a fundamental role in pathogen recognition and activation of innate immunity. Angiogenesis/Heparanase Inhibitor PG545 | therapeutic_agents || Pixatimod | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C97951 Maveropepimut-S A lipid depot-based therapeutic cancer vaccine composed of survivin epitopes, a universal T Helper peptide and a polynucleotide adjuvant encapsulated in liposomes and then formulated in the hydrophobic carrier Montanide ISA51 VG, with potential immunopotentiating and antineoplastic activities. Upon injection of Maveropepimut-S, a depot is created at the injection site from which the antigens and adjuvant are released. This vaccine may elicit a long lasting cellular response against survivin-expressing cancers, resulting in a decrease in tumor cell proliferation and an induction of tumor cell death. Survivin, a member of the inhibitor of apoptosis (IAP) family expressed during embryonic development, is upregulated in a variety of human cancers while absent in most normal adult cells; its expression in tumors is associated with a more aggressive phenotype, decreased survival, and increased resistance to chemotherapy. Survivin Antigen Vaccine DPX-Survivac | therapeutic_agents C1909 therapeutic_agents C C177537 GDC Value Terminology C18213 Stable Disease A disease that is neither decreasing nor increasing in extent or severity. SD-Stable Disease | best_overall_response || SD-Stable Disease | disease_response || Stable Disease | treatment_outcome C18919 || C50995 || C94536 treatment_outcome || disease_response || best_overall_response C C177537 GDC Value Terminology C16039 Autologous Hematopoietic Stem Cell Transplantation Stem cell transfer or transplantation in which the patient is their own donor. Stem Cell Transplantation, Autologous | treatment_type C25218 treatment_type