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Primary Ewing sarcoma/primitive neuroectodermal tumor in the adrenal gland
Lian Zhang1, Min Yao1, Masanori Hisaoka2
1Department of Pathology, The Second Hospital of Jilin University, Changchun, China.
Summary
Adrenal gland tumors are rare, with Ewing sarcoma or primitive neuroectodermal tumor (PNET) being exceptionally uncommon. This case highlights a 48-year-old woman with adrenal PNET, presenting a diagnostic and management challenge.
Area of Science:
- Oncology
- Pathology
Background:
- Primary adrenal gland tumors are rare.
- Ewing sarcoma or primitive neuroectodermal tumor (PNET) of the adrenal gland is an extremely rare entity.
Observation:
- A 48-year-old Chinese woman presented with left upper quadrant abdominal pain and swelling.
- CT revealed a large (12 cm) circumscribed mass in the left adrenal region.
- Surgical resection showed a 13 cm encapsulated mass with cystic degeneration, necrosis, and hemorrhage.
Findings:
- Histology revealed compact short spindle and oval tumor cells arranged in sheets.
- Immunohistochemistry was positive for vimentin, CD99, Bcl-2, NKX2.2, EMA, and CD117, with weak FLI-1 positivity.
- Fluorescence in situ hybridization confirmed EWSR1 gene rearrangement, characteristic of Ewing sarcoma/PNET.
Implications:
- This case underscores the rarity and diagnostic considerations for adrenal Ewing sarcoma/PNET.
- The patient experienced a localized recurrence 4.5 years post-adrenalectomy, highlighting the importance of long-term surveillance.
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