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Primary PNET of Maxilla: An Unusual Presentation

Mohindra, Pranshu MD*; Zade, Bhooshan MBBS*; Basu, Ayan MD*; Patil, Nikhilesh MD*; Viswanathan, Seethalakshmi MD; Bakshi, Ashish DM; Muckaden, Mary Ann MD*; Laskar, Siddhartha MD*

Journal of Pediatric Hematology/Oncology: June 2008 - Volume 30 - Issue 6 - pp 474-477
doi: 10.1097/MPH.0b013e31816e2309
Clinical and Laboratory Observations

Primitive neuroectodermal tumors (PNETs) are a type of small round cell tumors developing from migrating embryonal cells of the neural crest. Peripheral primitive neuroectodermal tumors (pPNETs) are less common with varying incidence of occurrence in head and neck region. Only 8 reported cases of primary PNET of maxilla are available in English literature. We report a case of 8-year-old boy diagnosed as pPNET of maxilla after detailed radiologic, histopathologic, including immuno-histochemical examination and molecular diagnosis using reverse transcription-polymerase chain reaction showing EWS-FLI1 translocation. The boy was treated with multiagent combination chemotherapy to be followed by definitive radiation therapy. A brief literature review of diagnosis and management of the previous 8 reported cases is done. In view of no definitive guideline for management of such cases, treatment on the lines of other pPNET is suggested.

Departments of *Radiation Oncology

Pathology

Medical Oncology, Tata Memorial Hospital, Mumbai, India

Reprints: Dr Siddhartha Laskar, MD, Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai 400012, India (e-mail: laskars2000@yahoo.com).

Received for publication September 17, 2007; Accepted February 12, 2008

© 2008 Lippincott Williams & Wilkins, Inc.