Abdominal cystic lymphangiomatosis: report of a case and review of the literature

Abstract

Abdominal cystic lymphangiomas are uncommon. We report the radiological and nuclear magnetic resonance (NMR) features of a patient presenting in adult life with gross involvement of the liver, retroperitoneum and pelvis. A 42-year-old woman presented in 1974 with an abdominal mass. Laparotomy and biopsy revealed numerous abdominal cysts and a histological diagnosis of cystic lymphangioma was made. No treatment was undertaken and she remained fairly well. She was referred to Hammersmith Hospital in 1982 following the development of thrombocytopenia and abdominal pain. Examination revealed bleeding from the gums, and a large right-sided abdominal mass was palpable with lobulated areas anterior to it. The haemoglobin was 13.6 g/dl, white cell count 7.4×l09/1 and platelet count 5 × 109/1. A bone marrow aspirate showed an active marrow with plentiful megakaryocytes indicating peripheral destruction of platelets. A chest radiograph was normal and there was no evidence of any osseous lesions. An intravenous urogram performed at this time (Fig. 1) showed a large abdominal mass with multiple areas of punctate calcification with deviation of both ureters, displacement of the kidneys and bilateral hydronephrosis. A 99Tcm sulphur colloid liver scan showed a grossly enlarged liver with numerous filling defects, the liver being displaced anteriorly. A chromium-labelled damaged red cell study showed a normal spleen being displaced upwards by a mass which was pooling red cells. Computed tomography (CT) (Fig. 2) showed extensive cystic changes involving the retroperitoneum.