Primary PNET of maxilla: an unusual presentation.
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.
Department of Radiation Oncology, Tata Memorial Hospital, Mumbai, India.
Pranshu
P
Mohindra
Bhooshan
B
Zade
Ayan
A
Basu
Nikhilesh
N
Patil
Seethalakshmi
S
Viswanathan
Ashish
A
Bakshi
Mary Ann
MA
Muckaden
Siddhartha
S
Laskar
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002675:Child, Preschool; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008441:Maxillary Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D018242:Neuroectodermal Tumors, Primitive / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D011878:Radiotherapy; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014057:Tomography, X-Ray Computed; D014157:Transcription Factors
C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors