Title

Unusual adrenal cortical tumor of unknown biologic potential: A nodule in a nodule in a nodule

Document Type

Journal Article

Publication Date

8-1-2005

Journal

Pediatric and Developmental Pathology

Volume

8

Issue

4

DOI

10.1007/s10024-005-1123-3

Keywords

Adrenal adenoma; Loss of heterozygosity; Oncocytic

Abstract

Adrenocortical tumors are uncommon neoplasms in childhood. Most pediatric adrenal tumors are virilizing and carcinomas are more common than adenomas. Recent molecular data suggest an adenoma-to-carcinoma progression sequence in adrenal cortical neoplasms. We report a case of a 5-year-old boy who presented with virilizing symptoms secondary to an adrenal tumor that was resected laparoscopically. The bulk of the tumor was a large, yellow mass with typical features of an adrenal cortical adenoma. In the center was a well-circumscribed tan-brown nodule that was distinct from the adenoma and had oncocytic features. A third minute focus (3.0 mm) was noted that was not circumscribed or encapsulated, but showed marked pleomorphism and abundant mitoses, including atypical forms and increased Ki67 compared with the outer 2 nodules. Molecular analysis to assess the clonality and mutation rates of the 3 distinct areas showed only 2 genetic loci with allelic imbalances. © 2005 Society for Pediatric Pathology.

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