Biomaterial Database

Multiple primitive neuroectodermal tumors. 1994

O P Sangüeza, and P Sangüeza, and L R Valda, and C K Meshul, and L Requena

Department of Dermatology L468, Oregon Health Sciences University, Portland 97201.

Primitive neuroectodermal tumors are rare, highly aggressive neoplasms that affect both sexes and occur in all age groups. They are a large group of neoplasms with neuroepithelial differentiation, including cutaneous neuroblastomas. Histopathologically they are characterized by a rather uniform population of small, dark cells, with or without Homer Wright rosettes. Immunohistochemically they stain for one or more of the reputed neural markers. Ultrastructurally they show interdigitating processes containing neurosecretory granules, intermediate filaments, and microtubules. We describe a patient with unusual multiple peripheral neuroectodermal tumors localized to the skin, with benign biologic behavior. The patient related the appearance of new lesions for several years without evidence of visceral dissemination or systemic complications. The histopathologic, immunohistochemical, and ultrastructural findings support the diagnosis of a peripheral neuroectodermal tumor.

UI	MeSH Term	Description	Entries
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D012878	Skin Neoplasms	Tumors or cancer of the SKIN.	Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D018241	Neuroectodermal Tumors, Primitive, Peripheral	A group of highly cellular primitive round cell neoplasms which occur extracranially in soft tissue and bone and are derived from embryonal neural crest cells. These tumors occur primarily in children and adolescents and share a number of characteristics with EWING SARCOMA.	Neuroectodermal Tumor, Peripheral,Peripheral Primitive Neuroectodermal Tumor,Peripheral Primitive Neuroectodermal Tumors,(pPNET) Peripheral Primitive Neuroectodermal Tumors,Extracranial Primitive Neuroectodermal Tumor,Neuroectodermal Neoplasm, Peripheral Primitive,Neuroectodermal Tumor, Peripheral Primitive,Neuroepithelioma, Peripheral,Peripheral Primitive Neuroectodermal Neoplasm,Primitive Neuroectodermal Tumor, Extracranial,Neuroectodermal Tumors, Peripheral,Neuroepitheliomas, Peripheral,Peripheral Neuroectodermal Tumor,Peripheral Neuroectodermal Tumors,Peripheral Neuroepithelioma,Peripheral Neuroepitheliomas,Tumor, Peripheral Neuroectodermal,Tumors, Peripheral Neuroectodermal
D018242	Neuroectodermal Tumors, Primitive	A group of malignant tumors of the nervous system that feature primitive cells with elements of neuronal and/or glial differentiation. Use of this term is limited by some authors to central nervous system tumors and others include neoplasms of similar origin which arise extracranially (i.e., NEUROECTODERMAL TUMORS, PRIMITIVE, PERIPHERAL). This term is also occasionally used as a synonym for MEDULLOBLASTOMA. In general, these tumors arise in the first decade of life and tend to be highly malignant. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2059)	Ependymoblastoma,Medulloepithelioma,Neuroepithelial Tumors, Primitive,PNET,Spongioblastoma,Cerebral Primitive Neuroectodermal Tumor,Neoplasms, Primitive Neuroepithelial,Neuroectodermal Tumor, Primitive,Neuroepithelial Neoplasms, Primitive,Primitive Neuroepithelial Neoplasms,Ependymoblastomas,Medulloepitheliomas,Neoplasm, Primitive Neuroepithelial,Neuroepithelial Neoplasm, Primitive,Neuroepithelial Tumor, Primitive,PNETs,Primitive Neuroectodermal Tumor,Primitive Neuroectodermal Tumors,Primitive Neuroepithelial Neoplasm,Primitive Neuroepithelial Tumor,Primitive Neuroepithelial Tumors,Spongioblastomas,Tumor, Primitive Neuroectodermal,Tumor, Primitive Neuroepithelial,Tumors, Primitive Neuroectodermal,Tumors, Primitive Neuroepithelial