Intra-Abdominal Multi Cystic Lymphangiomas: A Case Series with Adult and Pediatric Literature Review

Avani Saxena, Pramath Kakodkar*, Dan Zhang, Alysa Poulin, Nooshin Shekari, Selliah C Kanthan and Rani Kanthan

Background: Multi-cystic Lymphangioma (MCL) is a rare intra-abdominal entity in adults. The diagnosis of a core biopsy specimen is challenging due to its rarity in general surgical pathology service. Invasive surgical management can be avoided with accurate core biopsy diagnosis. 

Methods: We present six cases of adult intra-abdominal MCL and highlight their clinical, radiological, and pathological characteristics. Demographic, clinical, radiological, and histopathological parameters were collated for analysis.

Results: Six patients (out of 958,574 cases) were identified (prevalence: 0.6/100,000 cases). Previous abdominal surgery (67%, n = 4) and abdominal pain with palpable abdominal mass (67%, n = 4) at presentation were notable in this cohort. Abdominal CT showed cystic mass with septations, punctate calcification, and fatty halos raising the differential diagnosis of mesenteric cyst (n = 2), duplication cyst (n = 2), or disseminated metastases (n = 2). MCL was distributed across the jejunal mesentery (n = 3) and was managed with exploratory laparotomy and resection (n = 4). Characteristic histopathological features include multiple variably sized cystic spaces lined by attenuated flattened epithelium, cysts filled with proteinaceous fluid, and interspersed by stroma with several dense lymphoid aggregates. The lymphatic endothelium showed positivity for D2-40 and CD31. Post-operative follow-up at 50.4 ± 49.2 months did not show any clinical or radiological recurrence.

Conclusion: Adult intra-abdominal MCL are rare and radiologically indistinguishable from other intra-abdominal lesions. Diagnostic uncertainty on core biopsy evaluation in our series required invasive surgical exploration. The recognition of the histological triad of lymphoendothelial cysts, smooth-muscle or fibrous stroma, and associated lymphoid aggregates are diagnostic for MCL. Cystectomy alone is curative (144 months without recurrent).

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Published on: Nov 29, 2024 Pages: 27-39

Full Text PDF Full Text HTML DOI: 10.17352/2455-2283.000125
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