Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET) are a group of small round cell sarcomas that show varying degrees of neuroectodermal differentiation characterized by translocation involving the EWS gene. Uterine ES/pPNET is a rare entity. A 29-year-old Chinese female who presented with abdominal swelling and pain was diagnosed with a primary uterine ES/pPNET on the basis of clinicopathologic, immunohistochemical and fluorescence in situ hybridization (FISH) data. She was given a multimodal treatment, including neoadjuvant, 95% cytoreductive, chemotherapy and radiotherapy. The patient is currently alive with persistent disease after 18 months of follow-up. We emphasized the crucial role of molecular techniques in the differential diagnosis of small round cell tumors in this unusual location. Multimodal therapy may improve the outcomes of patients.
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View Article and Find Full Text PDFAxillary lesion in a young adult ends up to a peculiar diagnosis: primitive neuro-ectodermal tumor (PNET) of ulnar nerve: a rare case report.
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Department of Pathology, Golsar Hospital, Rasht, Guilan, Iran.
Article Synopsis
- Primitive neuro-ectodermal tumor (PNET) is a rare and aggressive tumor mainly affecting children and young adults, with very few documented cases found in peripheral nerves.
- A 30-year-old male presented with symptoms leading to the discovery of a PNET in the axillary region, diagnosed through imaging and confirmed via histopathological analysis after surgical removal.
- Accurate diagnosis is essential, as treatment typically includes surgical resection combined with chemotherapy and radiotherapy, highlighting the need to consider PNET in differential diagnoses of masses in the axillary area.
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