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WHO Classification of Tumours
Angiomyolipoma
Kidney


Definition

Angiomyolipoma is a benign neoplasm often associated with tuberous sclerosis . It is histologically characterized by a mixture of perivascular epithelioid cells, smooth muscle, vessels, and mature adipose tissue. The kidney is the most common site of involvement
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Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described.

> Epithelioid angiomyolipoma (EAML)
This potentially malignant mesenchymal variant is is occasionally observed in the kidney

Click for details
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



. Histological features include proliferation of predominantly epithelioid cells, further a high degree of cytological atypia, histiocytoid appearance, presence of melanocytic pigments, solid architecture with the absence of frequent areas of alveolar pattern, tubulo-papillary formation and scarring
Click to access Pubmed
Faraji H, Nguyen BN, Mai KT (2009)
Renal epithelioid angiomyolipoma: a study of six cases and a meta-analytic study. Development of criteria for screening the entity with prognostic significance.
Histopathology 55: 525-34



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