Leponce Sandrine, Buxant Frédéric, Noël Jean-Christophe
Department of obstetrics and gynaecology, IRIS Hospitals South, 63 rue Jean Paquot, 1050 Brussels, Belgium.
Department of pathology, Hôpital Universitaire de Bruxelles, Free University of Brussels, 808 route de Lennik, 1070 Brussels, Belgium.
Case Rep Womens Health. 2025 Jan 8;45:e00681. doi: 10.1016/j.crwh.2025.e00681. eCollection 2025 Mar.
Primary retroperitoneal mucinous carcinoma is an extremely rare malignancy, posing diagnostic and therapeutic challenges due to its nonspecific clinical presentation and lack of established management guidelines. The present article reports the case of a 39-year-old woman with progressive abdominal bloating and ascites, initially evaluated for a suspected ovarian mass. Imaging studies revealed a large mass with cystic and solid components mimicking an ovarian origin. However, surgical exploration revealed a retroperitoneal mass. Subsequent pathological analysis confirmed the diagnosis of mucinous Mullerian carcinoma. Molecular analysis revealed several mutations, including BRAF (V600E). Surgical resection was successful in treating the mass and the patient was in full remission at two-year follow-up. Despite its rarity, mucinous carcinoma should always be considered in the differential diagnosis of retroperitoneal masses. This case report discusses the anatomopathological features of primary retroperitoneal mucinous carcinoma and highlights the need for further research to elucidate the optimal management strategies and prognostic factors for this rare malignancy.
原发性腹膜后黏液性癌是一种极其罕见的恶性肿瘤，由于其临床表现不具有特异性且缺乏既定的管理指南，因此在诊断和治疗方面都面临挑战。本文报告了一名39岁女性的病例，该患者出现进行性腹胀和腹水，最初因疑似卵巢肿块而接受评估。影像学检查显示一个具有囊性和实性成分的大肿块，疑似起源于卵巢。然而，手术探查发现是一个腹膜后肿块。随后的病理分析确诊为黏液性苗勒管癌。分子分析显示了几种突变，包括BRAF（V600E）。手术切除成功治疗了该肿块，患者在两年随访时完全缓解。尽管其罕见，但在腹膜后肿块的鉴别诊断中应始终考虑黏液性癌。本病例报告讨论了原发性腹膜后黏液性癌的解剖病理学特征，并强调需要进一步研究以阐明这种罕见恶性肿瘤的最佳管理策略和预后因素。
