Angiomyolipomas (AMLs) are mesenchymal neoplasms, named so becauseof the complex tissue composition represented by variable proportions ofmature adipose tissue, smooth muscle cells, and dysmorphic blood vessels.Although AMLs may rise in different sites of the body, they are mostly observedin the kidney and liver. In the case of renal AMLs, they are described in twotypes: isolated AMLs and AMLs associated with tuberous sclerosis (TS). Whilemost cases of AMLs are found incidentally during imaging examinations andare asymptomatic, others may reach huge proportions causing symptoms.Pulmonary lymphangioleiomyomatosis (LAM) is a rare benign diseasecharacterized by cystic changes in the pulmonary parenchyma and smoothmuscle proliferation, leading to a mixed picture of interstitial and obstructivedisease. AML and LAM constitute major features of tuberous sclerosiscomplex (TSC), a multisystem autosomal dominant tumor-suppressor genecomplex diagnosis. The authors report the case of a young female patientwho presented a huge abdominal tumor, which at computed tomography (CT)show a fat predominance. The tumor displaced the right kidney and remainingabdominal viscera to the left. Chest CT also disclosed pulmonary lesionscompatible with lymphangioleiomyomatosis. Because of sudden abdominalpain accompanied by a fall in the hemoglobin level, the patient underwent anurgent laparotomy. The excised tumor was shown to be a giant renal AML withsigns of bleeding in its interior. The authors call attention to the diagnosis ofAML and the huge proportions that the tumor can reach, as well as for rulingout the TSC diagnosis, once it may impose genetic counseling implications.
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