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Pigmented adrenocortical carcinoma: Case report and review
Authors:Jordan L. Geller MD  Paul C. Azer MD  Lawrence M. Weiss MD  Dr. Richard B. Mertens MD   PhD
Affiliation:(1) Department of Internal Medicine, Division of Endocrinology, Diabetes and Metabolism, Cedars-Sinai Medical Center, 90048 Los Angeles, CA;(2) Division of Pathology, City of Hope National Medical Center, 91010 Duarte, CA;(3) Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Room 8746, 8700 Beverly Boulevard, 90048 Los Angeles, CA
Abstract:Darkly pigmented adrenocortical neoplasms are rare tumors that are often referred to as “black adenomas,” indicative of both their pigmented nature and their invariably benign clinical behavior in previously reported cases. We herein describe an exceptional case of a malignant pigmented adrenocortical neoplasm, with late recurrence and metastasis. At age 53, this female patient was diagnosed with Cushing's syndrome and underwent a laparoscopic right adrenalectomy, revealing a 3 cm well-circumscribed, darkly pigmented adrenocortical tumor. The tumor exhibited several atypical histologic features and was diagnosed as an atypical pigmented adrenal cortical neoplasm of uncertain malignant potential. Eight years later, the patient developed clinical and biochemical evidence of recurrent Cushing's syndrome, and imaging studies revealed the presence of several masses in the right retroperitoneum. At subsequent exploratory laparotomy, three separate tumor nodules exhibiting varying degrees of pigmentation and ranging from 2.2 to 3.3 cm maximum dimension were excised. Histologically, the tumor nodules were consistent with local recurrence/metastasis of the patient's previously excised pigmented adrenocortical neoplasm.
Keywords:Adrenal  pigmented adrenocortical carcinoma  black adenoma  differential diagnosis  late recurrence
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