Background: Lymphangioleiomyomatosis (LAM) is a rare, slow progressing, low-grade neoplasm that primarily effects premenopausal women. The disease is of systemic nature and well known for its pulmonary involvement with cystic destruction, but extra-pulmonary disease may occur and present as renal angiomyolipoma or lymphangioleiomyomtous lesions in different organs. LAM-associated lesions may present mutations in the tuberous sclerosis complex gens and may develop sporadically or in the context of tuberous sclerosis as hereditary disease. Incident LAM may represent the sentinel finding of the disease.
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Müller et al. (2024) studied this question.
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