What question did this study set out to answer?

This research aims to describe the FNAC findings and histopathological features of a rare BCOR::CCNB3 sarcoma in a pediatric patient.

February 27, 2026

Primary Adrenal BCOR::CCNB3 Sarcoma: Fine Needle Aspiration Cytology Findings.

Key Result

BCOR::CCNB3 sarcoma can be diagnosed via fine needle aspiration cytology with characteristic features and ancillary testing, guiding chemotherapy treatment.

Key Points

This research aims to describe the FNAC findings and histopathological features of a rare BCOR::CCNB3 sarcoma in a pediatric patient.
Performed fine needle aspiration cytology on a suspected adrenal mass.
Conducted histopathological analysis post-adrenalectomy.
Used fluorescence in situ hybridization to confirm BCOR::CCNB3 fusion.
FNAC showed medium-sized polygonal to spindled tumor cells and slender fibrovascular cores.
Histopathology revealed sheets of tumor cells with foci of necrosis.
BCOR::CCNB3 fusion was confirmed, characterizing the sarcoma.

Structured PICO

Population

13-year-old boy presenting with severe abdominal pain and a mass in the right adrenal gland

Intervention

Fine needle aspiration cytology (FNAC) and subsequent adrenalectomy with histopathology and fluorescence in situ hybridization (FISH)

Outcome

Diagnosis of BCOR-rearranged sarcoma (BCOR::CCNB3 fusion)

This report describes the first case of BCOR-altered sarcoma occurring in the adrenal gland and highlights the cytological features on FNAC to aid in accurate diagnosis.

Main Result

Absolute Event Rate: 0% vs 0%

Abstract

Pediatric adrenal tumors are uncommon, with the most common being peripheral neuroblastic tumors. Other neoplasms like adrenal cortical tumors, pheochromocytoma, and myelolipoma may also be encountered in this age group. Rare instances of mesenchymal tumors such as Ewing sarcoma and rhabdomyosarcoma have been described. We report the fine needle aspiration cytology (FNAC) findings of the first case of BCOR-altered sarcoma occurring in the adrenal gland. A 13-year-old boy presented with severe abdominal pain. Imaging revealed a mass in the right adrenal gland. FNAC showed loosely cohesive clusters and singly dispersed medium-sized polygonal to spindled tumor cells with scant to moderate delicate cytoplasm and normochromic, mildly pleomorphic nuclei with fine granular chromatin. Slender fibrovascular cores traversed tumor fragments. Histopathology of the adrenalectomy showed sheets of round to spindled tumor cells interrupted by thin fibrovascular septae, with foci of necrosis. BCOR, SATB2, and cyclin D1 were positive, while CD99, NKX2.2, WT1, desmin, and myogenin were negative. Fluorescence in situ hybridization revealed BCOR::CCNB3 fusion, consistent with BCOR-rearranged sarcoma. BCOR-rearranged sarcomas are rare malignant neoplasms. As primary treatment is chemotherapy, accurate diagnosis on limited material such as FNAC is critical. The recognition of cytological features and follow up with appropriate ancillary testing has the potential to improve management of these patients.

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