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Single lymphangiomyoma, rarely encountered, is a form of lymphangiomyomatosis with characteristic proliferation of smooth muscle cells in lymph nodes and vessels. We observed a case in a 47-year-old woman with no past medical history. An echography performed for menorrhage revealed a single iliac localization in the right pelvis. The precise pre-operative diagnosis was difficult, but the pathology report formally identified the tumor as an isolated form without thoracic involvement. Unlike lymphangiomatosis itself which involves pulmonary lesions and which may require complementary treatment, total exeresis of a single tumour is the only treatment necessary, as was confirmed in this case.

We report a case of lymphangiomyomatosis (LAM) which occurred in a twenty six year old woman, presenting with protein losing enteropathy and pancreatic fibrosis due to lymphostasis. Despite having no pulmonary lesion, the patient died quickly. The proliferation of smooth muscle involved the mesenteric, peripancreatic lymphatic channels but also the submucosal lymphatic vessels of the duodenum. Only one other case of LAM presenting with protein losing enteropathy has been reported, in which there were no abnormalities in the intestinal tractus or in the pancreas.