Tubulopapillary adrenocortical adenoma in a patient with familial adenomatous polyposis: a morphologic, ultrastructural, and molecular study
Patients with familial adenomatous polyposis have a higher incidence for developing adrenal neoplasms, most of which are nonfunctioning with conventional histologic appearance. We report a patient with a history of multiple colon polyps who developed an adrenocortical adenoma with unusual morphology...
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Published in | Human pathology Vol. 87; pp. 51 - 56 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
United States
Elsevier Inc
01.05.2019
Elsevier Limited |
Subjects | |
Online Access | Get full text |
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Summary: | Patients with familial adenomatous polyposis have a higher incidence for developing adrenal neoplasms, most of which are nonfunctioning with conventional histologic appearance. We report a patient with a history of multiple colon polyps who developed an adrenocortical adenoma with unusual morphology. The tumor showed a tubulopapillary architecture and plasmacytoid cytomorphology that were distinct from conventional adrenocortical adenomas. β-Catenin stain showed aberrant nuclear positivity in the tumor, suggesting an altered β-catenin–related pathway. The unusual morphology prompted molecular characterization, and sequencing demonstrated the patient to be germline heterozygous for a 5-base-pair APC deletion at codon 1309 with loss of heterozygosity in the tumor. Our study provides further evidence of genetic predisposition to extraintestinal tumors in the familial adenomatous polyposis population.
•This case describes the highly unusual finding of an adrenocortical adenoma with distinct histology in a familial adenomatous polyposis (FAP) patient, which has been seldom reported in the literature.•The tumor demonstrates a unique tubulopapillary and plasmacytoid histologic appearance with β-catenin nuclear translocation.•The case also demonstrates the importance of careful histologic assessment for prompting a molecular workup for hereditary changes in the APC gene.•This is the first report of APC codon 1309 variant associated with the development of adrenal cortical adenoma found in an FAP patient.•Our report provides further evidence that germline APC mutations contribute to higher incidence of extraintestinal tumors in FAP patients. |
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Bibliography: | ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0046-8177 1532-8392 |
DOI: | 10.1016/j.humpath.2018.08.022 |