一种罕见的子宫肿瘤:播散性腹膜黏液瘤病。临床观察。

V Svintsitskyi, M Krotevych, M Iegorov, S Trokhymych, S Sendetskyi
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摘要

背景:播散性腹膜黏液瘤病(DPL)是一种极为罕见的良性疾病,以腹腔、盆腔和腹膜后间隙广泛病变为特征,伴有大小不等、数目不等的肿瘤结节,是一种组织学结构由平滑肌纤维构成的良性肿瘤。目的:分析DPL的临床病例,并简要回顾DPL的诊断和治疗现状:我们分析了5例DPL临床病例,这些患者均为女性,年龄在39-50岁之间(平均年龄为46.2岁),于2010年至2021年期间在美国国立癌症研究所接受了手术治疗。根据病理(使用常规苏木精/伊红染色)和免疫组化(IHC)研究,所有 5 例患者均确诊为 DPL(8898/1):所有患者均接受了开腹手术治疗,手术范围和根治程度取决于肿瘤病灶的位置和数量。随访时,5 名患者均存活,且未接受任何特殊的肿瘤治疗:DPL的临床表现多种多样,从多发性肌炎到急腹症,取决于肿瘤主灶的位置和大小。IHC分析是最终诊断的标准,根治性切除所有肿瘤病灶可获得最佳治疗预后。治疗应在高度专业化的癌症中心进行,这些中心的外科医生在实施囊肿切除手术方面已积累了足够的经验。
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A RARE UTERINE TUMOR: DISSEMINATED PERITONEAL LEIOMYOMATOSIS. CLINICAL OBSERVATIONS.

Background: Disseminated peritoneal leiomyomatosis (DPL) is an extremely rare benign disease characterized by widespread lesions of the abdominal cavity, pelvis, and retroperitoneal space with tumor nodules of varying size and number, which are benign neoplasms consisting of smooth muscle fibers in their histological structure.

Aim: To analyze clinical cases of DPL with a concise review of the current state of the DPL diagnosis and treatment.

Materials and methods: We analyzed 5 clinical cases of DPL of female patients aged 39-50 years (mean age 46.2 years) who underwent surgical treatment at the National Cancer Institute from 2010 to 2021. In all 5 patients, the diagnosis of DPL (8898/1) was verified according to pathological (using routine hematoxylin/eosin staining) and immunohistochemical (IHC) studies.

Results: All patients underwent surgical treatment with a laparotomy approach, the extent and radicality of which depended on the location and number of tumor lesions. At the time of follow-up, all 5 patients were alive and did not receive any special oncological treatment.

Conclusions: DPL is characterized by a variety of clinical manifestations from polyserositis to acute abdomen, depending on the location and size of the main tumor focus. IHC analysis is the criterion for the final diagnosis, and radical removal of all tumor foci provides the best therapeutic prognosis. The treatment should be carried out in highly specialized cancer centers where surgeons have gained sufficient experience in performing cytoreductive surgery.

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