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Deoxycorticosterone-secreting adrenocortical carcinoma
Akihiro Yamamoto1, Takushi Naroda1, Susumu Kagawa1
1Department of Urology, School of Medicine, The University of Tokushima, Tokushima.
Endocrine Pathology
|March 7, 2020
Summary
This case report details a 66-year-old man with a DOC-secreting adrenocortical carcinoma, presenting with hypertension and hypokalemia. The tumor showed aggressive histological features and specific enzyme markers, leading to fatal metastasis.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Adrenocortical carcinoma is a rare and aggressive malignancy.
- Tumors secreting deoxycorticosterone (DOC) are uncommon causes of mineralocorticoid excess.
Purpose of the Study:
- To report a rare case of a DOC-secreting adrenocortical carcinoma.
- To describe the clinical, histological, and immunohistochemical features of the tumor.
Main Methods:
- Case presentation of a 66-year-old male patient.
- Clinical data including blood pressure, electrolytes, and hormone levels.
- Histopathological and immunohistochemical analysis of the resected adrenal mass.
Main Results:
- The patient presented with hypertension, hypokalemia, suppressed plasma renin activity (PRA), and elevated serum DOC levels.
- The resected adrenal mass weighed 230g and showed features of malignancy including necrosis, atypical mitoses, and vascular invasion.
- Immunohistochemistry revealed positivity for P450 C21 (21-hydroxylase) and P450 C17 (17α-hydroxylase) in tumor cells.
Conclusions:
- DOC-secreting adrenocortical carcinoma can present with significant mineralocorticoid excess symptoms.
- The tumor exhibited aggressive pathological features and specific enzyme expression patterns.
- The patient succumbed to metastatic disease nine months post-surgery.
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