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Multiple Lipoma Syndromes

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Syndromes with multiple lipomatous tumors.

Legius Syndrome: Multiple familial lipomas and multiple cafe-au-lait spots without neurofibromas.

Bannayan-Zonana Syndrome: Multiple congenital lipomas, hemangiomas, and macrocephaly. Mutations in PTEN gene.

Cowden Syndrome: Multiple lipomas, hemangiomas, goiter, skin/mucosal lesions (lichenoid, papular, papillomatous). Mutations in PTEN gene.

Frohlich Syndrome (aka Prune-Belly Syndrome): Multiple lipomas, obesity, sexual infantilism.

Proteus Syndrome: Hamartomatous disorder due to mosaic variants in AKT1 gene. Multiple lipomas, pelvic lipomatosis, fibroplasia of hands/feet, skeletal hypertrophy, exostoses and scoliosis, and pigmented skin lesions.

The image shows multiple lipomas removed from the arm of a patient with familial multiple lipomatosis. Image Source: Wikimedia Commons; By JR3336 - Own work; image cropped from the original, background changed and used under Creative Commons Attribution-Share Alike 4.0 License (CC BY-SA 4.0).

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