Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study

Abstract

A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with osseous component is reported. A 23-year-old man was admitted to our hospital because of acute severe abdominal pain. Abdominal computed tomography revealed a large solid and cystic, oval shaped mass, measuring 11.0×6.0 cm in the pelvic cavity. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming Homer-Wright rosettes and perivascular pseudorosettes, and showed areas of osteoid and bone formation. Immunohistochemical studies revealed that tumor cells expressed positivity against CD99 (MIC2), CD57, neuron-specific enolase, and vimentin. Fluorescence in situ hybridization study revealed Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement on chromosome 22q12. To the authors' knowledge this is the first documentation of a peripheral neuroectodermal tumor with osteoid and bone formation of the small bowel mesentery.

Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature

“…The solid component of the tumors was enhanced on contrast-enhanced CT, but the cystic component was not[ 17 ]. According to the published medical literature, there have been only five cases of E-EWS/pPNET of the small bowel including the patient presented in this report[ 1 - 4 ]. pPNET is a rare malignant tumor that usually occurs in children[ 18 - 20 ].…”

“…Extraskeletal Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (E-EWS/pPNET) of the small bowel is an extremely rare soft tissue neoplasm that tends to strike children and young adults[ 1 - 4 ]. It is a highly malignant small round cell tumor that has been thought to be of neural crest origin.…”

Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of the literature

“…Reports of cases arising in the intestines are extremely rare. To the best of our knowledge, there are 18 cases in the literature ( 4 – 21 ). The present study reports three unusual cases of PNET arising from the mesentery and ileocecum and describes the presenting symptoms, imaging findings, pathological features and molecular genetics of the tumors.…”