Pigmented adrenocortical carcinoma: Case report and review |
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Authors: | Jordan L Geller MD Paul C Azer MD Lawrence M Weiss MD Dr Richard B Mertens MD PhD |
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Institution: | (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 |
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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. |
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Keywords: | Adrenal pigmented adrenocortical carcinoma black adenoma differential diagnosis late recurrence |
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