A Unique Case of Intra-Abdominal Diffuse Lymphangiomatosis Mimicking a Pseudomyxoma Peritonei.

IF 0.7 Q4 SURGERY
Surgical Case Reports Pub Date : 2025-01-01 Epub Date: 2025-02-21 DOI:10.70352/scrj.cr.24-0037
Andreas R R Weiss, Georg F Weber, Maximilian Brunner, Robert Grützmann, Abbas Agaimy, Christian Krautz
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Abstract

Introduction: Localized cystic lymphangiomas (CL) are rare benign tumors deriving from the lymphatic system. CL is diagnosed more frequently in children than in the adult population and, although commonly affecting the cervical and axillary regions, can develop in various parts of the body. Abdominal cystic lymphangioma (ACL) comprises less than 5% of all CL cases.

Case presentation: A 35-year-old female patient with a history of benign appendectomy in childhood was transferred to our tertiary center for the operative management of a suspected extensive pseudomyxoma peritonei (PMP). In accordance with the multidisciplinary team discussion, cytoreductive surgery with hyperthermic intraperitoneal chemotherapy was planned. Intraoperatively, a typical "jelly belly" with high disease burden throughout the abdominal cavity and the small pelvis was found. A multi-visceral resection with complete cytoreduction (CCR 0) was performed. The postoperative histopathological findings revealed a diffuse, partially cystic lymphangiomatosis involving the peritoneum extensively without evidence of PMP or malignancy.

Conclusions: ACLs are uncommon in the adult population, and diffuse peritoneal involvement is even rarer. Surgical management with complete resection is the preferred treatment option. Other benign cysts, as well as infectious diseases and malignancy, should be considered during the preoperative workup.

腹内弥漫性淋巴管瘤病模拟腹膜假性粘液瘤的独特病例。
简介:局限性囊性淋巴管瘤是一种罕见的起源于淋巴系统的良性肿瘤。CL在儿童中的诊断频率高于成人,虽然通常影响颈部和腋窝区域,但可以在身体的各个部位发展。腹腔囊性淋巴管瘤(ACL)占所有CL病例的不到5%。病例介绍:一名35岁女性患者,童年时期有良性阑尾切除术史,因怀疑腹膜广泛假性黏液瘤(PMP)被转移到我们的三级中心进行手术治疗。根据多学科小组讨论,计划进行细胞减少手术并腹腔内高温化疗。术中发现典型的“果冻肚”,整个腹腔疾病负担高,骨盆小。多脏器切除并完全细胞减少(CCR 0)。术后组织病理学结果显示弥漫性,部分囊性淋巴管瘤病广泛累及腹膜,无PMP或恶性肿瘤的证据。结论:ACLs在成人中并不常见,弥漫性累及腹膜更是罕见。手术治疗和完全切除是首选的治疗方案。其他良性囊肿,以及感染性疾病和恶性肿瘤,应在术前检查时考虑。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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13 weeks
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