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.
"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. "
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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
[Show abstract][Hide abstract] ABSTRACT: Orbital masses in children are uncommon but extremely challenging problems for clinicians and pathologists due to their critical location and availability of limited diagnostic material. We analyzed 47 specimens comprising biopsies, excision specimens, and FNAC of extraconal pediatric orbital masses (excluding retinoblastoma) accessioned in the pathology department over 5 years in a tertiary referral cancer center. Immunohistochemistry (IHC-74%) and molecular methods (one case) were done where necessary. The chief presenting symptom was proptosis in 55.3% patients and radiologically 53.8% malignant tumors showed extraorbital extension. A diagnostic algorithm was formulated to assess which cases needed pathology evaluation. Malignant round cell tumors (76.6%), chiefly embryonal rhabdomyosarcoma (51%), benign spindle cell neoplasms, and infectious lesions (tuberculosis, fungal infections), were seen. Of the malignant tumors, those confined to the orbit achieved good treatment response and had an event-free follow-up while those with extraorbital spread had poor outcome. Pediatric orbital masses range from completely treatable infectious lesions, surgically resectable benign neoplasms to aggressive malignancies requiring chemotherapy and radiotherapy. Pathologists play a key role in distinguishing these on small biopsy material and expediating accurate treatment thus saving the vision or life of a patient.
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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