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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 ]
Patients with TSC can develop progressive replacement of the lung parenchyma with multiple cysts, known as lymphangioleiomyomatosis (LAM). Recent genetic analysis has shown that the proliferative bronchiolar smooth muscle in TSC-related lymphangioleiomyomatosis is monoclonal metastasis from a coexisting renal angiomyolipoma.
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.
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.
lymphangioleiomyomatosis, and, clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament. abdominopelvic sarcoma of perivascular epitheloid cells [2] primary extrapulmonary "sugar" tumour [2] Thus, it has been advocated that the above could be classified PEComas. [1]
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]
Sirolimus, also known as rapamycin and sold under the brand name Rapamune among others, is a macrolide compound that is used to coat coronary stents, prevent organ transplant rejection, treat a rare lung disease called lymphangioleiomyomatosis, and treat perivascular epithelioid cell tumour (PEComa).
Lymphangioleiomyomatosis (LAM) occurs in the lung and may result in pneumothorax, cystic lung destruction and pleural effusions. Symptoms which occur as a result may include fatigue, chest pain and shortness of breath.