Case Report
Urologia Internationalis
Urol Int 1998;61:251-253
Received: August 27, 1998 Accepted: November 4, 1998
Ken-ichi Egoshi Motoyuki Masai Koichi Nagao Haruo Ito
Department of Urology and Pathology, Teikyo University Ichihara Hospital, Ichihara, Japan
11-Deoxycorticosterone-Producing Adrenocortical Carcinoma
Key Words
11-Deoxycorticosterone Adrenocortical carcinoma Immunohistochemistry
Abstract
A woman presented with a history of weight loss and muscle weakness. A laboratory test revealed hypokalemia and elevation of plasma 11-deoxycorti- costerone (DOC). CT showed a left adrenal mass. A left adrenalectomy was performed. The histological and immunohistochemical diagnosis showed a DOC-producing adrenocortical carcinoma. This cancer is very rare; only 10 cases including the present case have appeared in the literature.
Introduction
Primary aldosteronism is widely known to be associat- ed with hypermineralocorticism, but rarely does overpro- duction of a mineralocorticoid other than aldosterone cause aldosteronism-like symptoms. The 11-deoxycorti- costerone(DOC)-producing tumor is the cause of one such disease. In the present report, we describe a patient with a DOC-producing adrenocortical cancer.
Case Report
A 60-year-old female presented with a history of weight loss (15.0 kg) and muscle weakness. She had been hypertensive for 20 years, and was given nifedipine. On admission to our hospital, her blood pressure was 164/108 mm Hg. Other physical examinations revealed no abnormal findings. A laboratory test revealed hypokal- emia (1.6 mEq/l) together with an increase in erythrocyte sedimenta- tion rate (73 mm/h). Endocrinologically, plasma renin activity and the plasma aldosterone level were normal, but plasma DOC (6.64 ng/ ml), 11-deoxycortisol (2.78 ng/ml) and urinary excretion of 17-
OHCS (8.6 mg/24 h) were high. Investigation of the left adrenal venous blood sample revealed elevations in DOC (55.6 ng/ml) and 11-deoxycortisol (14.5 ng/ml). CT showed a left adrenal mass (10 x 10 cm), but no metastases or invasion to adjacent tissues were noticed. An adrenal scintigram showed no apparent abnormalities.
A left adrenalectomy was performed, and a 11 × 8.5 × 4.5 cm (430 g) encapsulated adrenal tumor was removed. The cut face of the tumor showed hemorrhage and necrosis in places. Histologically, the tumor was mainly composed of atypical cells with eosinophilic cyto- plasm. Capsular invasion, sinusoidal invasion, atypical mitosis and slight nuclear pleomorphism were observed. Histological diagnosis was adrenocortical carcinoma. Immunohistochemically, a marked enhancement of cytochrome P-450 scc, P-450 c21, P-450 3ßHSD activities were indicated in the cytoplasm of cancer cells (fig. 1), in which active conversions into pregnenolone, progesterone, 11-deoxy- cortisol and DOC had occurred (fig. 2). In contrast, enhancement of P-450 c11, P-450 c17 activities were scanty in cells which showed poor conversions into aldosterone and cortisol (fig. 1, 2). These observations are consistent with a DOC-producing adrenocortical tumor.
Following the operation, hypertension, hypokalemia and ele- vation of plasma DOC and 11-deoxycortisol normalized (DOC <0.3 ng/ml). No adjuvant therapy was instituted up to 20 months after surgery, and no recurrence was observed.
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A
B
C
D
E
Discussion
DOC is a natural product of the adrenal gland as a pre- cursor of corticosterone and aldosterone [1]. DOC-pro- ducing tumors cause primary aldosteronism-like symp- toms, show low plasma aldosterone but very high DOC levels. The DOC-producing tumor is very rare; to our knowledge since the first case was reported in 1974 [2], only 20 cases including the present case have appeared in the literature [3-5]. Of these, 8 cases were asymptomatic and the tumors were found by chance at medical check- ups. The clinical presentations were muscle weakness (6 cases), abdominal pain (3 cases), weight loss (3 cases), fatigue (3 cases) and palpable abdominal mass (1 case). The weight of the excised tumors varied from 1.6 to 1,380 (mean 326 g). Histological diagnoses were malignant in 10 cases.
The DOC-producing tumors caused scant symptoms, reflecting the weak hormonal activity by DOC, thus they
were difficult to notice in the early stage of the disease. In particular, malignant cases were almost progressive with poor prognosis.
To date, no safe and effective treatment has been fully developed for adrenocortical carcinoma. Therefore, at present the best hope for this disease is early diagnosis to ensure a good treatment outcome.
Cholesterol
1 (++)
Pregnenolone
5 (土)
17OH-Pregnenolone (3.5ng/ml)
5 (土)
DHEA (2.2ng/ml)
2) (++)
2 (++)
2 (++)
Progesterone (2.8ng/ml )
6-+170H-Progesterone (±)
(0.5ng/ml)
5 (±)
Androstenedione
3 (++)
3 (+)
DOC (6.64ng/ml)
11-Deoxycortisol (2.78ng/ml )
4 (±)
Corticosterone (2.28ng/ml)
4 (土)
Aldosterone (68pg/ml)
Cortisol (9.9g/dl)
References
1 Ayres PJ, Eichorn J, Hechter O, Saba N, Tait SAS: Some studies on the biosynthesis of aldo- sterone and other adrenal steroids. Acta Endo- crinol 1960;33:27-58.
2 Powell-Jackson JD, Calin A, Fraser R, Gra- hame R, Mason P, Missen GAK, Powell-Jack- son PR, Wilson A: Excess deoxycorticosterone secretion from adrenocortical carcinoma. Br Med J 1974;ii:32-33.
3 Kelly WF, Ohare MJ, Loizou S, Davies D, Laing I: Hypermineralocorticism without ex- cessive aldosterone secretion: An adrenal carci- noma producing deoxycorticosterone. Clin En- docrinol 1982;17:353-361.
4 Kondo K, Saruta T, Saito I, Yoshida Y, Mu- rayama H, Matsuki N: Benign deoxycorticoste- rone-producing adrenal tumor. JAMA 1976; 236:1042-1044.
5 Irony I, Biglieri EG, Perloff D: Pathophysiolo gy of deoxycorticosterone-secreting adrenal tu- mors. J Clin Endocrinol Metab 1987;65:836- 840.
Copyright: S. Karger AG, Basel 1998. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Copyright: S. Karger AG, Basel 1998. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.