Peripheral primitive neuroectodermal tumors (pPNETs)

Ba L, et al. Radiologic and clinicopathologic findings of peripheral primitive neuroectodermal tumors. Acta Radiol. 2015 Jul;56(7):820-8.

Primitive neuroectodermal tumors (PNETs) constitute a rare type of malignant neuroectodermal tumors that have chromosomal translocations identical to Ewing’s sarcoma (ES), and the characteristics of this disease remain unclear. pPNETs can involve any part of the body, and a large, ill-defined, aggressive soft tissue mass and heterogeneous enhancement with or without osteolytic bone destruction on CT or MR images could suggest the diagnosis.

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Tan Y, et al. Peripheral primitive neuroectodermal tumor: dynamic CT, MRI and clinicopathological characteristics–analysis of 36 cases and review of the literature. Oncotarget. 2014 Dec 30;5(24):12968-77.

“The further diagnosis of pPNET should be suggested in young men when the following criteria was matched: images show a single large ill-defined solid mass with small area of necrosis; scarcely had classification or hemorrhage; locally invasive to adjacent structures, especially for those show iso-intense on T1WI and T2WI, which have significant enhancement.”

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Qian X, Kai X, Shaodong L, Gaohong C, Hong M, Jingjing L. Radiological and
clinicopathological features of pPNET. Eur J Radiol. 2013 Dec;82(12):e888-93.

The diagnosis of pPNET should be suggested in young patient when imaging depicts a large, ill-defined, soft-tissue mass containing area of cystic degeneration with internal septations and extending along the neural route and nearby the middle of the trunk. The key for correct diagnosis is to inosculate imaging to clinical data.

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Tsokos M, Alaggio RD, Dehner LP, Dickman PS. Ewing sarcoma/peripheral
primitive neuroectodermal tumor and related tumors. Pediatr Dev Pathol. 2012;15(1
Suppl):108-26.

Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.

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More PubMed results on pPNETs.

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