Kantelhardt T, Hallfeldt K, Müller-Höcker J, Schweiberer L, Löhrs U
Pathologisches Institut, Ludwig-Maximilians-Universität München.
Chirurg. 1996 Jun;67(6):653-7.
Lymphangioleiomyomatosis is a rare disease with proliferation of smooth muscle cells within the lymphatics, mediastinal and retroperitoneal lymph nodes and in the lungs. The clinical symptoms are increasing dyspnea, chylous effusion, intestinal obstruction and thoracic or abdominal pain. The authors report the case of a 42-year-old woman who primarily suffered from thoracic pain, dyspnea and chylous effusion. In further examinations we discovered a leftsided retroperitoneal tumor and a tumor in the mesentery. The diagnostic difficulties experienced are described and the necessity of explorative laparotomy for definite diagnosis is demonstrated. Furthermore, the article provides a review of the latest developments in pathology, diagnostics and therapy.
淋巴管平滑肌瘤病是一种罕见疾病，其淋巴管、纵隔和腹膜后淋巴结以及肺部存在平滑肌细胞增殖。临床症状包括进行性呼吸困难、乳糜胸、肠梗阻以及胸腹痛。作者报告了一例42岁女性患者，其主要症状为胸痛、呼吸困难和乳糜胸。在进一步检查中，我们发现了左侧腹膜后肿瘤和肠系膜肿瘤。描述了所遇到的诊断困难，并证明了进行剖腹探查以明确诊断的必要性。此外，本文还综述了病理学、诊断学和治疗学的最新进展。
