[Retroperitoneal lymphangiomyomatosis. Immunohistochemical and ultrastructural study of a case]. / Linfangiomiomatosi ad esordio retroperitoneale. Studio immunoistochimico ed ultrastrutturale di un caso.

A case of retroperitoneal lymphangiomyomatosis occurring in a 34-year-old woman is reported. Alpha-smooth muscle actin and desmin were localized in the proliferating cells. Electron microscopy showed the presence of many thin perinuclear myofilaments with dense bodies. The differential diagnosis with leiomyosarcoma has been taken into consideration.

Lymphangioleiomyomatosis. A case report with an immunohistochemical study.

A case of pulmonary lymphangioleiomyomatosis is presented, which occurred in a 50-year-old woman suffering from severe dyspnea of 7 year's duration and with a previous history of diseases (polycystic ovaries and endometrial adenocarcinoma) usually related to hyperestrogenism. The patient showed no signs or symptoms of tuberous sclerosis, a condition often associated with pulmonary lymphangioleiomyomatosis. Both the radiological pattern and histological findings had previously been interpreted at other hospitals as consistent with either nodular interstitial fibrosis with emphysematous changes or pulmonary siderosis. Correct diagnosis was suspected on critical review of histological slides and definitely achieved by immunohistochemistry in routine sections, which showed the presence of desmin in the elements constituting the nodular lesions scattered throughout the parenchyma. The criteria for the differential diagnosis are discussed, as well as the role of immunohistochemistry in assessing the muscular nature of proliferating cells.

Pulmonary lymphangioleiomyomatosis: a case of progesterone receptor positive lymphangioleiomyomatosis treated with medroxyprogesterone, oophorectomy and tamoxifen.

A 28-year-old woman with lymphangioleiomyomatosis had severe changes in the chest radiograph and recurrent pneumothoraces. Pathological tissue from the lung was progesterone receptor positive. After treatment with medroxyprogesterone her condition improved, but did not become clinically stable until after bilateral oophorectomy. Even after oophorectomy her condition deteriorated, when the dose of medroxyprogesterone was decreased. For one year after oophorectomy she has had no episodes of pneumothorax or breathlessness. In order to depress oestrogen activity further the anti-oestrogen drug tamoxifen was added to her usual treatment with medroxyprogesterone. During 2 weeks of treatment with tamoxifen and medroxyprogesterone marked increase of the changes in the chest radiograph were seen and a pneumothorax occurred. After discontinuation of tamoxifen the condition improved and has since been stable on medroxyprogesterone treatment.

Pulmonary lymphangiomyomatosis: with particular reference to steroid-receptor assay studies and pathologic correlation.

The symptoms of progressive dyspnea, hemoptysis, spontaneous pneumothorax, chylous effusions, and cough in conjunction with ventilatory obstruction and abnormal gas exchange in a young woman should prompt the diagnosis of pulmonary lymphangiomyomatosis. Cytosol steroid-receptor assays and postmortem studies were conducted in an extensive investigation of a case of this disease. A biopsy specimen of the lung disclosed evidence of nuclear translocation of [3H]progesterone and the presence of a cytosolic receptor for progesterone, an indication that this disease could be treated effectively with progestin.