Biomaterial Database

Atypical teratoid/rhabdoid tumor: an unusual presentation. 2004

Chirag D Gandhi, and Mark D Krieger, and J Gordon McComb

Department of Neurosurgery, The Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1136, Annenberg 8-06, New York, NY 10029-6574, USA. chirag.gandhi@mssm.edu

Atypical teratoid/ rhabdoid tumor (AT/RT) of the central nervous system is a rare, highly aggressive malignancy of infancy. Although it is reported infrequently in the literature, it has often been histologically confused with a primitive neuroectodermal tumor (PNET)/medulloblastoma (MB) but has a much worse prognosis. We present an infant with two AT/RT tumors, one suprasellar in location and the other within the vermis without evidence of tumor elsewhere. What makes this case unusual is that there were two separate lesions in different cranial compartments, with no evidence of subarachnoid seeding. In addition, the lesions had different magnetic resonance imaging (MRI) characteristics even though they were histologically the same.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D005260	Female		Females
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
D015173	Supratentorial Neoplasms	Primary and metastatic (secondary) tumors of the brain located above the tentorium cerebelli, a fold of dura mater separating the CEREBELLUM and BRAIN STEM from the cerebral hemispheres and DIENCEPHALON (i.e., THALAMUS and HYPOTHALAMUS and related structures). In adults, primary neoplasms tend to arise in the supratentorial compartment, whereas in children they occur more frequently in the infratentorial space. Clinical manifestations vary with the location of the lesion, but SEIZURES; APHASIA; HEMIANOPSIA; hemiparesis; and sensory deficits are relatively common features. Metastatic supratentorial neoplasms are frequently multiple at the time of presentation.	Cancer, Supratentorial,Benign Supratentorial Neoplasms,Malignant Supratentorial Neoplasms,Neoplasms, Supratentorial,Primary Supratentorial Neoplasms,Supratentorial Neoplasms, Benign,Supratentorial Neoplasms, Malignant,Supratentorial Neoplasms, Primary,Supratentorial Tumors,Benign Supratentorial Neoplasm,Cancers, Supratentorial,Malignant Supratentorial Neoplasm,Neoplasm, Benign Supratentorial,Neoplasm, Malignant Supratentorial,Neoplasm, Primary Supratentorial,Neoplasm, Supratentorial,Neoplasms, Benign Supratentorial,Neoplasms, Malignant Supratentorial,Neoplasms, Primary Supratentorial,Primary Supratentorial Neoplasm,Supratentorial Cancer,Supratentorial Cancers,Supratentorial Neoplasm,Supratentorial Neoplasm, Benign,Supratentorial Neoplasm, Malignant,Supratentorial Neoplasm, Primary,Supratentorial Tumor,Tumor, Supratentorial,Tumors, Supratentorial
D015192	Infratentorial Neoplasms	Intracranial tumors originating in the region of the brain inferior to the TENTORIUM CEREBELLI, which contains the cerebellum, FOURTH VENTRICLE; CEREBELLOPONTINE ANGLE; BRAIN STEM, and related structures. Primary tumors of this region are more frequent in children, and may present with ATAXIA; CRANIAL NERVE DISEASES; vomiting; HEADACHE; HYDROCEPHALUS; or other signs of neurologic dysfunction. Relatively frequent histologic subtypes include TERATOMA; MEDULLOBLASTOMA; GLIOBLASTOMA; ASTROCYTOMA; EPENDYMOMA; CRANIOPHARYNGIOMA; and choroid plexus papilloma (PAPILLOMA, CHOROID PLEXUS).	Infratentorial Tumors,Posterior Fossa Neoplasms,Benign Infratentorial Neoplasms,Infratentorial Cancer,Infratentorial Neoplasms, Benign,Infratentorial Neoplasms, Malignant,Malignant Infratentorial Neoplasms,Neoplasms, Infratentorial,Neoplasms, Posterior Fossa,Posterior Fossa Tumors,Benign Infratentorial Neoplasm,Cancer, Infratentorial,Infratentorial Cancers,Infratentorial Neoplasm,Infratentorial Neoplasm, Benign,Infratentorial Neoplasm, Malignant,Infratentorial Tumor,Malignant Infratentorial Neoplasm,Neoplasm, Benign Infratentorial,Neoplasm, Infratentorial,Neoplasm, Malignant Infratentorial,Neoplasm, Posterior Fossa,Neoplasms, Malignant Infratentorial,Posterior Fossa Neoplasm,Posterior Fossa Tumor,Tumor, Infratentorial,Tumor, Posterior Fossa
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