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Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years. [ 1 ]
It is separate and distinct from lymphangiectasis, lymphangioleiomyomatosis (LAM), pulmonary capillary hemangiomatosis, Kaposi’s sarcoma, and kaposiform hemangioendothelioma. [4] Its unusual nature makes lymphangiomatosis (and Gorham’s disease) a diagnostic and therapeutic challenge.
These tumours may also arise in the tunica dartos of the scrotum and the mammillary muscle of the nipple (genital leiomyoma), the smooth muscle of blood vessels (angioleiomyoma) and the lung (pulmonary lymphangioleiomyomatosis). [5] A pseudo-Darier sign may be present.
These multifocal lesions are observed in tuberous sclerosis, [6] [7] and can be associated with lymphangioleiomyomatosis [8] and perivascular epithelioid cell tumour (PEComa or clear cell "sugar tumor")). [9] It can be diagnosed through lung biopsy using thoracoscopy. [10]
Mutations in TSC2 can cause Lymphangioleiomyomatosis, a disease caused by the enlargement of tissue in the lungs, creating cysts and tumours and causing difficulty breathing. Because tuberin regulates cell size, along with the protein hamartin, mutations to TSC1 and TSC2 genes may prevent the control of cell growth in the lungs of individuals.
They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis. Angiomyolipomas are less commonly found in the liver and rarely in other organs. Whether associated with these diseases or sporadic, angiomyolipomas are caused by mutations in either the TSC1 or TSC2 genes, which govern cell growth and proliferation. They ...
Lymphatic malformations are benign slow-flow type of vascular malformation of the lymphatic system characterized by lymphatic vessels which do not connect to the normal lymphatic circulation.
Lymphohemangioma is a disease characterized by swelling of the lymph nodes and blood vessels.It is variously described as a "mixture of clear fluid and blood-filled cysts", [citation needed] a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels.