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Chap 19 - ANGIOMYOLIPOMA AND RELATED TUMORS (PERIVASCULAR EPITHELIOID CELL TUMORS)

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Angiomyolipoma (AML) occurs primarily in the kidney but is rare in the liver or retroperitoneum (Fig. 19.1). This tumor contains mature lipomatous, vascular, and a peculiar, variably HMB45-positive smooth muscle–like cellular component and can be associated with tuberous sclerosis.

AML belongs to a group of tumors named perivascular epithelioid cell tumors (PEComas). They are characterized by the presence of an HMB45-positive smooth muscle–like element similar to that found in angiomyolipoma. Some of these tumors are also associated with tuberous sclerosis complex.

PEComas include retroperitoneal lymphangiomyoma, pulmonary lymphangiomyomatosis, and clear cell “sugar” tumor. Other PEComas (PEComa, not otherwise specified) usually occur in the abdomen, especially in the uterus and elsewhere in the abdominal cavity, and rarely, in peripheral soft tissues. Cardiac rhabdomyoma is discussed in this chapter, although it is not a PEComa. It is associated with the tuberous sclerosis complex (TSC), however.

ANGIOMYOLIPOMA

Clinical Features

AML of the kidney usually presents in adults and rarely in children, with an overall median age ~50 years. There is a significant (3:1 or higher) female predominance (Fig. 19.2). In the author's experience, retroperitoneal AML has similar demographics, and in fact some of such tumors are extensions of a renal primary tumor. Approximately 20% of renal AMLs occur in patients with TSC. In this context, these AMLs occur in patients 20 years younger than the sporadic cases; these tumors are often multiple and sometimes bilateral. Most childhood AMLs are seen in TSC patients.

Type
Chapter
Information
Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 531 - 545
Publisher: Cambridge University Press
Print publication year: 2010

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References

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Eble, JN, Amin, MB, Young, RH. Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component. Am J Surg Pathol 1997;21:1123–1130.CrossRefGoogle ScholarPubMed
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Chan, JK, Tsang, WY, Pau, MY, Tang, MC, Pang, SW, Fletcher, CD. Lymphangiomyomatosis and angiomyolipoma: closely related entities characterized by hamartomatous proliferation of HMB-45-positive smooth muscle cells. Histopathology 1993;22:445–455.CrossRefGoogle Scholar
Fetsch, PA, Fetsch, JF, Marincola, FM, Travis, W, Batts, KP, Abati, A. Comparison of melanoma antigen recognized by T-cell (MART-1) to HMB-45: additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis and clear cell sugar tumor. Mod Pathol 1998;11:699–703.Google ScholarPubMed
Bernard, M, Lajoie, G. Angiomyolipoma: immunohistochemical and ultrastructural study of 14 cases. Ultrastruct Pathol 2001;25:21–29.Google Scholar
Stone, CH, Lee, MW, Amin, MB, Yaziji, H, Gown, AM, Ro, JY. Renal angiomyolipoma: Further immunophenotypic characterization of an expanding morphologic spectrum. Arch Lab Pathol Med 2001;125:751–758.Google ScholarPubMed
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Boorijan, SA, Sheinin, Y, Crispen, PL, Lohse, CM, Kwon, ED, Leibovich, BC. Hormone receptor expression in renal angiomyolipoma: clinicopathologic correlation. Urology Mar 26 [Epub ahead of print].
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