Retroperitoneal Lymphangioma in an Adult: A Case Report of a Rare Clinical Entity

Abstract

Background. Retroperitoneal lymphangioma is a rare, benign mesodermal tumor arising from the retroperitoneal lymphatics which usually presents in infancy and it is worthy to report a case when it has presented in an adult. After a thorough literature search in English we concluded that less than 200 cases of adult retroperitoneal lymphangioma have been reported so far. Apart from being a rare entity it also presents as a diagnostic dilemma and final diagnosis is often made on surgical exploration. Case Presentation. We report a case of retroperitoneal lymphangioma in a 55-year-old male who presented with abdominal distension and dull aching abdominal pain. Conclusion. Retroperitoneal lymphangiomas are rare tumors of infancy but may also present in adults where they are a diagnostic challenge. Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone are often inconclusive and surgery is frequently required for definitive diagnosis and to ameliorate the symptoms.

A Huge Cystic Retroperitoneal Lymphangioma Presenting with Back Pain

“…Among all patients, pancreatic lymphangiomas were shown to be encountered more commonly in female patients with the female-to-male ratio of 29 : 16 and with the mean age of 40 years [4]. 75% of lymphangiomas are encountered in the neck and 20% in the axillary region, and only 5% are intra-abdominal including spleen, liver, pancreas, and very rarely in the retroperitoneum [5]. Retroperitoneal location being one of the rarest locations accounts for about 1% of all lymphangiomas [6].…”

Retroperitoneal lymphatic malformation in a dog

“…Although lymphatic malformations can be subclassified as microcystic (diameter ≤ 5 mm), macrocystic (> 5 mm) or combined; retroperitoneal malformations are mostly macrocystic [2,[30][31][32], as seen in our patient. The most common presenting signs in humans with retroperitoneal cystic lymphatic malformations are abdominal pain and abdominal distension, but patients can also be presented with a palpable mass, back pain, anorexia, fever, nausea and diarrhoea [33][34][35][36][37][38][39]. Haemorrhage within the cystic space is common, indicating recent trauma or spontaneous intralesional bleeding [27,32]; therefore symptoms can occur when patients become older due to increased size of the cystic space.…”

Retroperitoneal lymphatic malformation: a case report

“…The tissue assessment post surgical excision is virtually diagnostic of lymphangioma and shows cystic spaces lined with a single thin layer of endothelium, lymphoid tissue and smooth muscles. [4][5][6][7][8] However excision was deferred in this case due to involvement of mesenteric vessels and multiple compartments. Interventional radiology was opted to help in reducing the size of the lesions by attempting multiple Sclerotherapy sessions.…”

“…5 Sometimes it is difficult to differentiate lymphatic malformation from other retroperitoneal cystic lesions, hence surgical excision and histopathology assessment are recommended. 7,9 Also it is needed to reduce the complication like progressive increased in size, infection, intra-lesional bleed. 9…”