Primary PNET of maxilla: An unusual presentation

Department of Radiation Oncology, Tata Memorial Hospital, Mumbai, India.
Journal of Pediatric Hematology/Oncology (Impact Factor: 0.9). 06/2008; 30(6):474-7. DOI: 10.1097/MPH.0b013e31816e2309
Source: PubMed


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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    • "Due to its similarity to Ewing sarcoma, surgical resection followed by adjuvant radiotherapy at a dose of 45–70 Gy, as well as multiagent chemotherapy if possible, is necessary to improve patient survival (3–7). However, in accordance with the studies by Yeh et al (8) and Mohindra et al (12), in the present study, the two patients were treated with concurrent radiotherapy and chemotherapy without surgical resection based on the unfavorable prognosis associated with the disease even after en bloc resection, and the unacceptable extensive cosmetic and functional destruction that would be caused following the resection of the involved portion of the maxilla or mandible. Close cooperation between surgeons, oncologists, radiotherapists and radiologists is required for the treatment of pPNET. "
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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.
    Oncology letters 08/2014; 8(2):615-619. DOI:10.3892/ol.2014.2219 · 1.55 Impact Factor
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    • "Askin's tumor, melanotic neuroectodermal tumor, ectomesenchymoma , and peripheral medulloepithelioma [7]. "
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    ABSTRACT: We report the case of a 12-year-old girl, who consulted us with one-year history of an 8 mm nose lesion that was painless and firm upon palpation. The lesion was resected conservatively. Immunohistochemistry was in favor of a primitive neuroectodermal tumor (PNET)/Ewing's sarcoma lesion, excluding epithelial, lymphoid, and other tumors. After a second resection, our patient was referred to chemotherapy and has already undergone 9 cycles out of 14. The patient is to date with no evidence of persistent or recurrent disease. To our knowledge, this is the first description of a PNET arising in the nose.
    Case Reports in Medicine 05/2013; 2013:512416. DOI:10.1155/2013/512416
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    Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 06/2009; 454(6):703-13. DOI:10.1007/s00428-009-0775-1 · 2.65 Impact Factor
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