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Lymphangioleiomyomatosis (LAM) Other names: lymphangiomyomatosis, LAM: Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM. Specialty ...
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.
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.
There are three distinct types of lymphangioma, each with their own symptoms. They are distinguished by the depth and the size of abnormal lymph vessels, but all involve a malformation of the lymphic system. Lymphangioma circumscriptum can be found on the skin's surface, and the other two types of lymphangiomas occur deeper under the skin.
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]
Given the phenotypic variability, there are no established diagnostic criteria. Instead, the diagnosis is made based on clinical evidence and supporting histopathology that demonstrates the presence of positive lymphatic markers, such as podoplanin (D2-40) or lymphatic vessel endothelial hyaluronan receptor 1 (), as well as negative glucose transporter 1 ().
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. It occurs in approximately 30-40% of women with TSC, up to 80% by the age of 40, and it is much less common in men. [36]
BACM symptoms that follow are most frequently calf pain, gait complaints, and inability to walk. [2] The condition is self-limited and full restitution can be expected. In very rare cases, however, rhabdomyolysis may develop. [1] Affected are preschool and school-age children with a male predominance. [2]