Two different adrenal adenomas causing Cushing's syndrome.

A nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, tumors originating from ectopic adrenal tissue are very rare, as is secretion of aldosterone, cortisol, or androgens from such tissue, and adenomatous transformation resulting in glucocorticoid excess is an extremely rare phenomenon.1 We present an interesting case of a tumor arising in presumed ectopic adrenal tissue causing Cushing’s syndrome in a 52-year-old woman who already had an adrenal tumor, and both were managed by retrolaparoscopy. We observed this dynamic case for over 10 months after operation. In this light, we review the etiology, location, and possible pathophysiology of ectopic or aberrant adrenocortical tumor and search for effective and minimally invasive treatment to help future work. A 52-year-old woman developed moderate hypertension without any symptoms 10 years ago. She began to markedly gain weight of a central character, and a dorsal-cervical fat pad appeared, followed by muscle weakness and fatigue, easy bruisability, and bluish stretch marks on the abdomen. The medical history was otherwise relevant for osteoporosis and hemiparesis by earlier encephalorrhagia. Physical examination on admission revealed a yellow middle-aged woman with a moon face and marked central obesity. The blood pressure was 177/115 mm Hg. Facial plethora was present, and the abdomen was obese, exhibiting bluish striae. There were scattered ecchymoses and a dorsalcervical fat pad. The muscle strength of her right side was rather weak. The 8:00AM, 4:00PM, and 12:00PM plasma cortisol levels tested 3 times in one day were higher than the normal and the diurnal variation of cortisol lost. The 24 hour urinary free cortisol was 268.7 ug (normal, 20.26-127.55). An overnight 1mg dexamethasone suppression test revealed a plasma cortisol level of 603.60 nmol/L, and 24 hour urinary free cortisol was 252.67 ug. After the administration of 2 mg dexamethasone per day for 2 days, the plasma cortisol was 638.40 nmol/L, and the 24 hour urinary free cortisol was 243.55 ug. After the administration of 8 mg dexamethasone for 2 days, plasma cortisol was 684.20 nmol/L, and 24 hour urinary free cortisol was 259.21 ug. Blood chemistries, complete blood count, urinalysis, and the other related hormones including renin, angiotensin, aldosterone, epinephrine, norepinephrine, and total testosterone were within normal range. The abdominal CT scan revealed a 1.5×1.1×1.0 cm mass located near the left adrenal gland and there was no other mass. A repeated abdominal CT scan after 7 months, revealed a 1.5×1.2×1.0 cm mass in the same position and a 3.0×1.5×1.0 cm newly found pararenal lesion adjacent to the hilum almost riding on the renal artery with clear edge. No enlarged lymph node, vascular or organic invasion was obvious (Figure 1). In light of the information above, the diagnosis of corticotropinindependent Cushing’s syndrome caused by a primary adrenocortical adenoma combined with an ectopic adrenocortical adenoma was highly suspected. Because of the poor condition including high blood pressure, osteoporosis, hemiparesis, and possible infection, she was planned for laparoscopic exploration and removal of the masses for minimally invasive purpose. Complete resection of both the tumors was successfully performed by retroperitoneal-laparoscopy. Both the resection specimens were oval masses with the entire capsule surrounded by a rim of fat. The pathological results demonstrated a 1.5×1.0×1.0 cm round shaped primary adrenocortical adenoma weighing 3.0 g combined with a 3.0×2.0×2.0 cm regular shaped ectopic adrenocortical adenoma weighing 7.0 g. The pathological report was “adrenal cortical adenomas surrounded by adrenal tissue and fat without capsular or vascular invasion. The one adjacent to the left renal hilum is apt to arise from the remnant of the adrenal gland and the fat containing adrenal gland.” Further immunohistochemistry (Table 1) demonstrated the benign results of both tumors and eliminated the possibility of malignance or metastasis. The postoperative course was uneventful. No complications were observed either intraoperatively or postoperatively. She was discharged home on glucocorticoids 7 days after the operation. Close followup persisted after her discharge from hospital. She did well on reducing dosages of glucocorticoids regularly, Clinical Note