Angiomyolipoma is a mesenchymal neoplasm containing adipose tissue, blood vessels, and smooth muscle fibers. Arising most frequently in the kidney, the tumor may exceptionally be at the head and neck region. The literature has 1 single published report of laryngeal angiomyolipoma, which was resected through laryngofissure. In this study, a 58-year-old man presented with laryngeal angiomyolipoma with swallowing discomfort. The tumor was removed by an endolaryngeal approach.

A rare benign laryngeal tumor: angiomyolipoma.

Benign Renal Tumors

Angiomiolipoma renal epitelioide: reporte de un caso y revisión de la literatura

Angiomyolipomas are rare, benign tumours which are composed of an intimate admixture of blood vessels, smooth muscle cells and fat and hence the name. They occur at many sites, more commonly in the kidney. Usually they present as an incidental finding or with retroperitoneal haemorrhage in adults. They are seen in 25-50% of the patients with tuberous sclerosis. A 29 year old female presented with a mass in the left loin with a dragging pain, which was incidentally found during a medical check up. She had no evidence of tuberous sclerosis. A nephrectomy was done. Microscopy revealed the classical features of angiomyolipoma, which comprised of mature adipose tissue, thick walled blood vessels and bundles of smooth muscle cells. No epithelioid component was seen.

Renal Angiomyolipoma - A Case Report

The perivascular epithelioid cell (PEC) has been proposed to be the proliferating cell type in a group of tumors known as PEComas. The histogenesis of PEComas is one of the most mysterious aspects of pathology. Hypothesis on its precursor are many, including a cell from blood vessel walls or the myoblast. In the current report, we review many morphologic, clinical, ultrastructural, molecular and genetic aspects that support the hypothesis of an origin of PEComas from the neural crest.

Evidence on the neural crest origin of PEComas.

Renal angiomyolipoma is a benign tumour histologically characterized by a mixture of adipose tissue, smooth muscle cells and thick walled blood vessels. Long-believed to be a benign hamartoma, angiomyolipoma is now considered to arise from perivascular epithelioid cells. Epithelioid angiomyolipoma is a rare type of angiomyolipoma, composed partially or completely of epithelioid cells, with a potentially aggressive behaviour. Histologically it can mimic renal cell carcinoma. Positivity for HMB45, Melan A, CD68 and CD117 are useful for diagnosis. Herein, we report the clinicopathologic and immunohistochemical features of a renal tumour composed of large epithelioid mononucleated or multinucleated cells with abundant acidophilic cytoplasm and prominent nucleoli. Despite the morphologic resemblance of this tumour to renal cell carcinoma, its phenotype (HMB45, Melan A and CD68 positivity and keratin negativity) parallels the phenotypic profile of angiomyolipoma. Therefore, immunohistochemistry should be considered when diagnosing this variant of angiomyolipoma.

Epithelioid angiomyolipoma of the kidney: case report.

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Epithelioid angiomyolipoma of the kidney: case report.