Primary PNET of maxilla: an unusual presentation.

Department of Radiation Oncology, Tata Memorial Hospital, Mumbai, India.
Journal of Pediatric Hematology/Oncology (Impact Factor: 0.97). 06/2008; 30(6):474-7. DOI: 10.1097/MPH.0b013e31816e2309
Source: PubMed

ABSTRACT 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.

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    ABSTRACT: Peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant undifferentiated tumor, which presents in infants and young adults. pPNETs in the head and neck region are uncommon and have a varying incidence of occurrence. Peripheral PNETs of the maxilla and mandible are particularly rare. At present, only 16 cases of pPNET of the maxilla and 13 cases of pPNET of the mandible have been reported. The present study describes a case of pPNET of the maxilla in a 16-year-old male and a case of pPNET of the mandible in another 16-year-old male. The present study reports the radiological findings and the clinical courses of the two patients.
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    ABSTRACT: 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 was done, which showed a mass in the optic chiasma and parasellar region. The typical appearance resembled large noncalcified soft tissue masses in the magnetic resonance image and computed tomographic scan of the maxilla. Diagnosis was established by immunohistochemical features. She was treated with surgery, chemotherapy, radiation therapy, and gamma knife. She was under close observation since then (approximately 8 mo), and there has been no recurrence of tumor up to now.
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