Unicentric Castleman Disease of the Mesentery: A Systematic Review

单中心性肠系膜卡斯尔曼病:系统性综述

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Abstract

Castleman disease (CD) is a rare lymphoproliferative disorder categorized into unicentric and multicentric forms. The unicentric form usually manifests as a localized lymph node enlargement. Mesenteric involvement is rare and can mimic other intra-abdominal neoplasms such as gastrointestinal stromal tumors, lymphoma, or metastatic disease, making preoperative diagnosis difficult. The study aimed to systematically analyze published cases of mesenteric unicentric CD (UCD) and evaluate demographic trends, anatomical distribution, histopathological variants, tumor characteristics, treatment strategies, and clinical outcomes. A systematic review of individual case reports published between 1996 and 2026 was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) framework. The literature search was conducted using PubMed and Scopus as the primary databases. Reports describing mesenteric UCD were screened, and relevant details, including age, sex, lesion size, location, histopathology, and management, were extracted. Data were summarized using descriptive statistics and synthesized narratively. The mean age at diagnosis was 35.3 ± 17.9 years, and the mean lesion size was 5.51 ± 2.25 cm. The mesentery (unspecified) was the most frequently reported site (75.68%), followed by the small bowel mesentery (18.92%) and large bowel mesentery (5.41%). The prevalence of mesenteric UCD with a hyaline vascular variant was 64.86%, followed by plasma cell (21.62%) and mixed types (12.16%). In 1.35% of cases, histology was not specified. Complete surgical excision represented the primary therapeutic approach (60.81%) and was associated with favorable outcomes in most patients. For mesenteric UCD, surgical excision remains the cornerstone of treatment, offering an excellent prognosis. Early recognition and accurate histopathological diagnosis are essential to avoid misclassification and guide optimal management.

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