We report a case of peripheral primitive neuroectodermal tumor (pPNET), which belongs to the pPNET/Ewing's sarcoma family, arising in the chest wall of a 69-year-old man. He had high levels of serum neuron-specific enolase and pro-gastrin-releasing peptide, which are believed to be useful diagnostic blood markers for small cell lung carcinoma (SCLC). Microscopically, the tumor was composed of s...

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (pPNET/EWS) is an aggressive type of sarcoma that is rarely observed in the kidney. pPNET of the kidney principally occurs in young patients (<50 years old) and is very rare in older patients (≥50 years old). Additionally, only six cases of pPNET of the kidney have been reported in the literature in older patients (≥50 years old), and p...

Peripheral primitive neuroectodermal tumor (pPNET) is a rare, very aggressive neoplasm that belongs to a small round cell tumor, and most often arises from the chest wall. Here, we report a female case with proven pPNET who was treated in our institution. She presented with a history of left side chest pain, cough, and significant weight loss. Contrast enhanced CT imaging of the chest showed m...

The peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant soft tissue neoplasm in children and young adults. Fewer than 10 reported cases of pPNET of maxilla are available in the English literature. A 28-year-old woman was presented with the pPNET of the maxilla and metastasis. Two years after diagnosis, she experienced diplopia, and then magnetic resonance imaging w...

A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate proces...

BACKGROUND The peripheral primitive neuroectodermal tumor (pPNET) is a rare malignant tumor originating from neuroectoderm. The accurate diagnosis is essential for the treatment of pPNET. METHODS we performed the largest cases of retrospective analysis thus far to review the unique computed tomography (CT), magnetic resonance imaging (MRI), and clinicopathological features of pPNET. The tumor...

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) arising in the kidney is very rare, and only a few cases have been reported. Here we present two cases of renal ES/pPNET with different prognosis. Both patients underwent radical nephrectomy. One patient had complex EWSR1 gene chromosome translocation, high Ki-67 proliferative index and died of metastatic disease 2 months after...

Sunitinib malate (Sutent(TM); Pfizer Inc., New York, N.Y., USA) is a small molecule kinase inhibitor with activity against a number of tyrosine kinase receptors, including vascular endothelial growth factor receptors, stem-cell factor receptor, and platelet-derived growth factor receptors alpha and beta. Sunitinib, registered for the treatment of renal cell carcinoma and gastrointestinal stroma...

Extraskeletal Ewing’s Sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from the more common Ewing’s sarcoma of the bone. Primitive Ewing’s sarcoma of the breast is exceptionally reported. We present the case of a 26-year-old woman with Ewing sarcoma/PNET diagnosed by molecular biology showing the specific t ranscr ipt of Ewing/ per ipheral pr imi t ive neuroectoderm...

Running title: anti-apoptotic and neuroectodermal pathways in Ewing family of tumors Supported by grant 01/0673 fom FIS (Madrid) and PROTHETS (EC Contract number 503036). ABSTRACT Aims An important criteria for EFT is that it originates from a primitive committed cell with neuroectodermal capacity. The degree of neural differentiation within EFT, as measured with morphological features and expr...