LYMPHANGIOLEIOMYOMATOSIS - A RARE DISEASE OF THE LYMPHATIC-SYSTEM

Lymphangioleiomyomatosis is a rare disease with proliferation of smoot h muscle cells within the lymphatics, mediastinal and retroperitoneal lymph nodes and in the lungs. The clinical symptoms are increasing dys pnea, chylous effusion, intestinal obstruction and thoracic or abdomin al pain. The authors report the case of a 42-year-old woman who primar ily suffered from thoracic pain, dyspnea and chylous effusion. In furt her examinations we discovered a leftsided retroperitoneal tumor and a tumor in the mesentery. The diagnostic difficulties experienced are d escribed and the necessity of explorative laparotomy for definite diag nosis is demonstrated. Furthermore, the article provides a review of t he latest developments in pathology, diagnostics and therapy.