Biomaterial Database

Embryonal Tumors of the Central Nervous System: An Update. 2020

Melissa M Blessing, and Sanda Alexandrescu

Department of Pathology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address: Melissa.Blessing@Childrens.Harvard.Edu.

Embryonal tumors of the central nervous system (CNS) are rare, high-grade neoplasms predominantly affecting the pediatric population. Well-defined embryonal tumors include medulloblastoma, atypical teratoid/rhabdoid tumor, embryonal tumor with multilayered rosettes, C19MC-altered and embryonal tumor with multilayered rosettes, not otherwise specified, pineoblastoma, pituitary blastoma, CNS neuroblastoma, and ganglioneuroblastoma. Although their prognosis is nearly uniformly poor, the rapidly evolving understanding of their molecular biology contributes to diagnosis, prognosis, treatment, and clinical trial participation. Knowledge of current tumor stratification and diagnostic techniques will help pathologists guide care and preserve tissue for necessary or desired additional testing.

UI	MeSH Term	Description	Entries
D008527	Medulloblastoma	A malignant neoplasm that may be classified either as a glioma or as a primitive neuroectodermal tumor of childhood (see NEUROECTODERMAL TUMOR, PRIMITIVE). The tumor occurs most frequently in the first decade of life with the most typical location being the cerebellar vermis. Histologic features include a high degree of cellularity, frequent mitotic figures, and a tendency for the cells to organize into sheets or form rosettes. Medulloblastoma have a high propensity to spread throughout the craniospinal intradural axis. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2060-1)	Arachnoidal Cerebellar Sarcoma, Circumscribed,Medulloblastoma, Desmoplastic,Medullomyoblastoma,Sarcoma, Cerebellar, Circumscribed Arachnoidal,Medulloblastoma, Adult,Medulloblastoma, Childhood,Melanocytic Medulloblastoma,Adult Medulloblastoma,Adult Medulloblastomas,Childhood Medulloblastoma,Childhood Medulloblastomas,Desmoplastic Medulloblastoma,Desmoplastic Medulloblastomas,Medulloblastoma, Melanocytic,Medulloblastomas,Medulloblastomas, Adult,Medulloblastomas, Childhood,Medulloblastomas, Desmoplastic,Medulloblastomas, Melanocytic,Medullomyoblastomas,Melanocytic Medulloblastomas
D009373	Neoplasms, Germ Cell and Embryonal	Neoplasms composed of primordial GERM CELLS of embryonic GONADS or of elements of the germ layers of the EMBRYO, MAMMALIAN. The concept does not refer to neoplasms located in the gonads or present in an embryo or FETUS.	Germ Cell Cancer,Germ Cell Tumor,Neoplasms, Embryonal and Mixed,Cancer, Embryonal,Cancer, Embryonal and Mixed,Embryonal Neoplasms,Germ Cell Neoplasms,Germ Cell and Embryonal Neoplasms,Germ Cell and Embryonic Neoplasms,Neoplasms, Embryonal,Neoplasms, Germ Cell,Neoplasms, Germ Cell and Embryonic,Cancer, Germ Cell,Cancers, Embryonal,Cancers, Germ Cell,Embryonal Cancer,Embryonal Cancers,Embryonal Neoplasm,Germ Cell Cancers,Germ Cell Tumors,Neoplasm, Embryonal,Tumor, Germ Cell,Tumors, Germ Cell
D011379	Prognosis	A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.	Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D002528	Cerebellar Neoplasms	Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141)	Benign Cerebellar Neoplasms,Cerebellar Cancer,Malignant Cerebellar Neoplasms,Cerebellar Neoplasms, Benign,Cerebellar Neoplasms, Malignant,Cerebellar Neoplasms, Primary,Cerebellar Tumors,Neoplasms, Cerebellar,Neoplasms, Cerebellar, Benign,Neoplasms, Cerebellar, Malignant,Neoplasms, Cerebellar, Primary,Primary Neoplasms, Cerebellum,Benign Cerebellar Neoplasm,Cancer, Cerebellar,Cerebellar Cancers,Cerebellar Neoplasm,Cerebellar Neoplasm, Benign,Cerebellar Neoplasm, Malignant,Cerebellar Neoplasm, Primary,Cerebellar Tumor,Cerebellum Primary Neoplasm,Cerebellum Primary Neoplasms,Malignant Cerebellar Neoplasm,Neoplasm, Benign Cerebellar,Neoplasm, Cerebellar,Neoplasm, Cerebellum Primary,Neoplasm, Malignant Cerebellar,Primary Cerebellar Neoplasm,Primary Cerebellar Neoplasms,Primary Neoplasm, Cerebellum,Tumor, Cerebellar
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013724	Teratoma	A true neoplasm composed of a number of different types of tissue, none of which is native to the area in which it occurs. It is composed of tissues that are derived from three germinal layers, the endoderm, mesoderm, and ectoderm. They are classified histologically as mature (benign) or immature (malignant). (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1642)	Dysembryoma,Teratoid Tumor,Teratoma, Cystic,Teratoma, Mature,Teratoma, Benign,Teratoma, Immature,Teratoma, Malignant,Benign Teratoma,Benign Teratomas,Dysembryomas,Immature Teratoma,Immature Teratomas,Malignant Teratoma,Malignant Teratomas,Teratoid Tumors,Teratomas,Teratomas, Benign,Teratomas, Immature,Teratomas, Malignant,Tumor, Teratoid,Tumors, Teratoid
D016543	Central Nervous System Neoplasms	Benign and malignant neoplastic processes that arise from or secondarily involve the brain, spinal cord, or meninges.	CNS Neoplasm,CNS Neoplasms,Central Nervous System Neoplasm,Central Nervous System Tumor,Neoplasms, Central Nervous System,Primary Central Nervous System Neoplasm,Central Nervous System Neoplasms, Primary,Central Nervous System Tumors,Primary Central Nervous System Neoplasms,Tumors, Central Nervous System,Neoplasm, CNS,Neoplasms, CNS
D018335	Rhabdoid Tumor	A rare but highly lethal childhood tumor found almost exclusively in infants. Histopathologically, it resembles RHABDOMYOSARCOMA but the tumor cells are not of myogenic origin. Although it arises primarily in the kidney, it may be found in other parts of the body. The rhabdoid cytomorphology is believed to be the expression of a very primitive malignant cell. (From Holland et al., Cancer Medicine, 3d ed, p2210)	Rhabdoid Tumors,Tumor, Rhabdoid,Tumors, Rhabdoid