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Angiomyolipoma With Epithelial Cysts.

Michael A LeRoy1, Priya Rao

  • 1From the Department of Pathology, MD Anderson Cancer Center, Houston, Texas.

Archives of Pathology & Laboratory Medicine
|May 28, 2016
PubMed
Summary

Angiomyolipoma with epithelial cysts is a rare kidney tumor. This variant has distinct cystic and solid features, specific cell markers, and an indolent clinical course.

Area of Science:

  • Nephrology
  • Oncology
  • Pathology

Background:

  • Angiomyolipoma with epithelial cysts is a rare mesenchymal kidney tumor.
  • It is part of the differential diagnosis for adult cystic renal neoplasms.
  • Clinical presentation includes incidental findings or symptoms like flank pain and hematuria.

Purpose of the Study:

  • To describe the distinct pathological and immunohistochemical features of angiomyolipoma with epithelial cysts.
  • To highlight its differential diagnosis from conventional angiomyolipoma and other cystic renal neoplasms.
  • To report on the clinical course of this rare tumor variant.

Main Methods:

  • Gross pathological examination revealing solid and cystic areas.
  • Histological analysis of cyst lining, subepithelial layer, and tumor wall.

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  • Immunohistochemical profiling for melanocytic markers (HMB-45, Melan-A) and hormone receptors (ER, PR).

    • Main Results:

    • Grossly characterized by solid and cystic areas.
    • Histologically features cysts lined by epithelial cells, a 'cambium-like' stromal layer, and a thick wall with smooth muscle and dysplastic vessels.
    • Immunohistochemistry shows reactivity for melanocytic markers and hormone receptors.
    • Tumors exhibit an indolent clinical course with no reported progression or metastasis.

    Conclusions:

    • Angiomyolipoma with epithelial cysts is a distinct entity with unique pathological features.
    • Its characteristic immunohistochemical profile aids in diagnosis.
    • The tumor appears to have a benign, indolent clinical behavior.