Definition
Epithelioid angiomyolipoma is a rare variant which consists of at least 80% epithelioid cells.
Clinical Features
As for classic, epithelioid angiomyolipoma has been reported in patients with or without tuberous sclerosis, as well as in the TSC2/PKD1 contiguous gene syndrome.
Incidence
It represents 4.6% of all resected angiomyolipomas.
Age
The mean age at of diagnosis is 50 years.
Sex
There is no gender predominance.
Site
There is no site predominance.
Treatment
For local tumors, partial or radical nephrectomy is the treatment of choice. For aggressive cases (metastatic or local recurred), it has been reported the use of mTOR inhibitors.
Outcome
Epithelioid angiomyolipoma can behave in a malignant fashion. The frequency of aggressive biological behavior (including both local recurrence and metastasis) is extremely variable. Prognostic factors includes the presence of TSC, tumor size, carcinoma-like morphologic pattern,...
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References and Further Reading
Caliò, A., Brunelli, M., Segala, D., Pedron, S., Tardanico, R., Remo, A., Gobbo, S., Meneghelli, E., Doglioni, C., Hes, O., Zampini, C., Argani, P., & Martignoni, G. (2018). t(6;11) renal cell carcinoma: A study of seven cases including two with aggressive behavior, and utility of CD68 (PG-M1) in the differential diagnosis with pure epithelioid PEComa/epithelioid angiomyolipoma. Modern Pathology, 31(3), 474–487.
Martignoni, G., Pea, M., Bonetti, F., Zamboni, G., Carbonara, C., Longa, L., Zancanaro, C., Maran, M., Brisigotti, M., & Mariuzzi, G. M. (1998). Carcinomalike monotypic epithelioid angiomyolipoma in patients without evidence of tuberous sclerosis: A clinicopathologic and genetic study. The American Journal of Surgical Pathology, 22(6), 663–672.
Nese, N., Martignoni, G., Fletcher, C. D., Gupta, R., Pan, C. C., Kim, H., Ro, J. Y., Hwang, I. S., Sato, K., Bonetti, F., Pea, M., Amin, M. B., Hes, O., Svec, A., Kida, M., Vankalakunti, M., Berel, D., Rogatko, A., Gown, A. M., & Amin, M. B. (2011). Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: Detailed assessment of morphology and risk stratification. The American Journal of Surgical Pathology, 35(2), 161–176.
Pea, M., Bonetti, F., Martignoni, G., Henske, E. P., Manfrin, E., Colato, C., & Bernstein, J. (1998). Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: The identification of malignant epithelioid angiomyolipoma. The American Journal of Surgical Pathology, 22(2), 180–187.
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Caliò, A., Segala, D., Martignoni, G. (2019). Epithelioid Angiomyolipoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3737-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3737-1
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