OBJECTIVE: In adults, supratentorial primitive @entity4969 (sPNET) is a very rare undifferentiated @entity5 . Prognosis is worse in @entity2946 . Older age appears to be prognostically favorable. At present, 5-year survival rates remain below 50% in all age groups. Survival longer than 15 years in an adult has only been reported once so far. CASE REPORT: In 1987, a 33-year-old-male @entity1 presented with @entity410 following a six-month's history of dizziness. CT- and MRI-scans revealed a right occipital @entity5 with moderate contrast enhancement. The @entity5 was completely removed. The original histological diagnosis was that of an @entity3491 , @entity5 , grade III. The @entity1 was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy). Admission for another reason in 2003 led to a re-evaluation of the original diagnosis. Microscopy revealed a malignant, highly cellular, poorly differentiated @entity5 with a desmoplastic component. Up to 20% of @entity5 nuclei were labeled for Ki-67. Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent. According to these findings, the diagnosis was changed to a sPNET (WHO IVdegrees ). Other @entity5 entities were excluded by immunohistochemistry. CONCLUSIONS: Although the prognosis of sPNET is reported to be poor, a small fraction with a rather benign biological and clinical behavior exists. Parameters determining long-term-survival in sPNET are not yet known. Whenever possible, complete surgical resection should be attempted followed by postoperative radiotherapy. The value of chemotherapy is an issue of continuous investigation.

Long-term relapse-free survival with supratentorial primitive XXXX in an adult: a case report.

@entity1 :: ('9606', 'Species') :: ['patient'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizures'] @entity4969 :: ('MESH:D017599', 'Disease') :: ['neuroectodermal tumor'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'malignant hemangioendothelioma', 'embryoblastic neoplasm'] @entity3491 :: ('MESH:C580334', 'Disease') :: ['undifferentiated sarcoma'] @entity2946 :: ('MESH:D008527', 'Disease') :: ['comparison to infratentorial medulloblastoma']
@entity4969 :: (MESH:D017599,Disease) :: ['neuroectodermal tumor']
