Authors
Dyah Listyaningtyas, Hari Nugroho, Bagus Ngurah Brahmantara, Heriyawati, Ayu Farikha Nandiaty
Published in
Gynecologic oncology reports. Volume 66. Pages 102172. Epub Jul 19, 2026.
Abstract
Primary ovarian synovial sarcoma is an exceedingly rare malignancy that may mimic more common ovarian tumors, particularly in adolescents, posing significant diagnostic challenges.
A 14-year-old girl presented with progressive abdominal distension and respiratory compromise. Imaging revealed a large mixed solid-cystic adnexal mass with ascites and pleural effusion. Serum CA-125 was elevated, while AFP and CEA were normal. The patient underwent right salpingo-oophorectomy and omentectomy. Histopathology showed a monophasic spindle cell neoplasm with focal cartilaginous differentiation. Immunohistochemistry demonstrated diffuse nuclear positivity for SS18 and TLE1, with co-expression of BCL-2, CD99, and EMA, while inhibin, calretinin, and WT-1 were negative. The positive SS18 immunohistochemical staining provided additional diagnostic confirmation of synovial sarcoma, supporting the diagnosis in conjunction with the characteristic histopathologic findings. The patient deteriorated rapidly and died before initiation of chemotherapy.
Primary ovarian synovial sarcoma is a rare and aggressive tumor that can mimic germ cell neoplasms. Accurate diagnosis requires careful integration of histopathology and immunohistochemistry. Although molecular confirmation remains the diagnostic gold standard, SS18 fusion-specific immunohistochemistry provides a reliable diagnostic surrogate when molecular testing is unavailable.
PMID:
42555456
Bibliographic data and abstract were imported from PubMed on 06 Aug 2026.
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