Department of Musculoskeletal Pathology, The Royal Orthopaedic Hospital NHS Foundation Trust, University of Birmingham, UK.
Int J Gynecol Pathol. 2011 Jan;30(1):84-91. doi: 10.1097/PGP.0b013e3181f0c510.
Synovial sarcoma is a morphologically and genotypically distinctive soft tissue sarcoma with a strong predilection for young and middle aged adults and the deep soft tissues of the extremities. Rare cases of synovial sarcoma have been reported in a large variety of unusual sites, one of the least common being the female genital tract. We report 4 young women with synovial sarcoma involving the vulva (3 cases) and vagina (1 case). Two of the tumors were of the biphasic and 2 of the monophasic type; 3 of the tumors were poorly differentiated. The diagnosis in all the cases was supported by immunohistochemical findings and reverse transcription polymerase chain reaction and sequencing demonstration of SS18/SSX fusion transcripts. Two of the patients developed recurrent disease (1 dies of disease after 8 years) and 2 are currently disease-free.This study shows the importance of pathologists being aware of the rare occurrence of synovial sarcoma in the female genital tract, discusses the differential diagnosis with particular reference to this location, emphasizes the need for molecular genetic support in such cases, and reviews the sparse, earlier literature. Synovial sarcoma should be considered in the differential diagnosis of a vulvovaginal mesenchymal lesion, especially in a young female.
滑膜肉瘤是一种形态学和基因型上都具有特征性的软组织肉瘤，好发于年轻和中年成年人，以及四肢深部软组织。滑膜肉瘤在许多不常见的部位也有罕见报道，其中最不常见的部位之一是女性生殖道。我们报道了 4 例发生于外阴（3 例）和阴道（1 例）的滑膜肉瘤年轻女性患者。其中 2 例为双相型，2 例为单相型；3 例肿瘤分化差。所有病例的诊断均得到免疫组织化学、逆转录聚合酶链反应和 SS18/SSX 融合转录本测序的支持。其中 2 例患者出现复发性疾病（1 例患者在 8 年后死于疾病），2 例患者目前无疾病。本研究表明病理学家意识到滑膜肉瘤在女性生殖道中罕见的重要性，讨论了特别针对该部位的鉴别诊断，强调在这种情况下需要分子遗传学支持，并回顾了早期文献。滑膜肉瘤应作为外阴阴道间质性病变的鉴别诊断之一，尤其是在年轻女性中。
