Angiomyolipoma is a benign tumoral lesion of the kidney which microscopically contains fat, smooth muscle and blood vessels, and may result in retroperitoneal hemorrhage.
Lymphangiomyomatosis is a lesion that may lead to diffuse involvement of lungs and cause respiratory failure, spontaneous pneumothorax or pleural effusion. It is characterized by proliferation of a mixture of lymphatic and smooth muscle. Both lesions may be components of tuberous sclerosis, an uncommon syndrome.
Our case is 41-year-old female presented with chest pain and dyspnea. Thin walled air cysts were determined in lung parenchyma by thorax tomography and images of enlarged kidneys and mass lesions including areas of adipose density were supplied by abdominal tomography. Right nephrectomy was performed because of intraabdominal hemorrhage.
Infiltrative tumor microscopically composed of mature lipomatose, myomatose components and congested blood vessels, and reactive for HMB-45 [Melanoma (gp100) Ab-3 (clone HMB45+HMB50), Neomarkers] was diagnosed as angiomyolipoma. Microscopic evaluation of the lung lesions revealed cystic lymphatic containing smooth muscle cells. The tumor with presence of smooth muscle in the walls of lymphatic shown with trichrome and was diagnosed as lymphangiomyomatosis.
The patient died because of irreversible cardiopulmonary arrest on the postoperative 16th day.
Key Words: Angiomyolipoma, Lymphangiomyomatosis, Tuberosclerosis
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