Primitive neuroectodermal tumor (PNET) is presumably of neural crest origin, and cases of supratentorial PNET in adult are extremely rare. We reported a case of PNET presenting as a dumbbell-shaped tumor at the middle cranial fossa of skull base mimicking trigeminal schwannoma both clinically and radiologically. The patient underwent surgery on July 13, 2010, using a combination of subtemporal,...

Mechanisms of neuroendocrine tumor (NET) proliferation are poorly understood, and therapies that effectively control NET progression and metastatic disease are limited. We found amplification of a putative oncogene, RABL6A, in primary human pancreatic NETs (PNET) that correlated with high-level RABL6A protein expression. Consistent with those results, stable silencing of RABL6A in cultured BON-...

Primitive neuroectodermal tumors (PNET) are an aggressive group of small round cell tumors usually arising in the nervous system and affecting children. They have a tendency for local invasion, distant spread and formation of tumor thrombi. The kidney is a rare primary location for these tumors. Outcomes are frequently poor due to late diagnosis (Wilms tumor is a more common tumor in this popul...

RATIONALE Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first c...

Deletions in the short arm of chromosome 17 (17p) are the most common genetic abnormality in primitive neuroectodermal tumors of the posterior fossa/medulloblastoma (PNET/Mb). The biological consequences of these deletions are not known for children with PNET/Mb; however, the presence of a tumor suppressor gene located in 17p, distinct from p53, has been implicated in tumorigenesis. Two recent ...

A case of primary primitive neuroectodermal tumor (PNET) of the chest wall in an adolescent is presented with imaging findings including 18F-fluorodeoxyglucose (18F-FDG) positron-emission computed tomography (PET-CT). To date, radiological description of the PNET of the chest wall has mainly been carried out by radiographic analysis, computed tomography (CT), and magnetic resonance imaging (MRI...

Primitive neuroectodermal tumor (PNET) is a rare disease and mostly diagnosed in children and young adults. This tumor is presumed to be of neuroectodermal origin, probably developing from migrating embryonic cells of the neural crest. Carcinoembryonic antigen is a glycoprotein and frequently elevated in patients with a variety of epithelial malignancies. We report hereby a 59-year old male pat...

Primitive neuroectodermal tumor (PNET) is a rare "small round blue cell tumor" that is diagnosed by open biopsy or percutaneous biopsy of the lesion under radiologic guidance. In this case report, we present a novel approach to the diagnosis of a retroperitoneal PNET by endoscopic ultrasound- (EUS-) guided fine needle aspiration (FNA). A 35-year-old man presented with the history of left-sided ...

Lungs are one of most metastatic areas for primitive neuroectodermal tumor (PNET), however primary pulmonary PNET is extremely rare. Here we present a case of a 58-year-old male patient with a tumor in the right lung that originated from the lung but not from chest wall. Patient was diagnosed with PNET following histological and immunohistochemical examination of CT-guided percutaneous tru-cut ...

The retinoblastoma (RB) tumor suppressor pathway is likely important in primitive neuroectodermal tumors (PNET) of the brain. In fact, 10% to 15% of children born with RB mutations develop brain PNETs, commonly in the pineal gland. Cyclin D1, which in association with cyclin-dependent kinase (Cdk) 4 and Cdk6 phosphorylates and inactivates the RB protein, is expressed in 40% of sporadic medullob...