Incidentally Diagnosed Adrenocrtical Carcinoma with Intraperitoneal Metastases

Presentation Number: SAT 383
Date of Presentation: April 1st, 2017

Ameya Hodarkar*1 and Leo Tchong2
1Lahey Medical Center, Burlington, MA, 2Lahey Hospital and Medical Center, Burlington, MA

Abstract

Background: Unilateral tumors or masses of the adrenal gland are common and categorized as either functional or silent and as either benign or malignant. The majority of adrenocortical tumors are benign, nonfunctioning adenomas. Adrenocortical carcinomas (ACCs) are rare, often aggressive tumors that may be functional and cause Cushing's syndrome or virilization. But most are nonfunctional and present as an abdominal mass or an incidental finding. The incidence is approximately 1-2 per million population per year. Here we present a case of ACC which was diagnosed due to ascites and vague abdominal discomfort.

Clinical Case: A 60 year old woman with hypothyroidism presented to her primary care provider with abdominal pain. Abdominal exam revealed distension with discomfort on palpation. No organomegaly or masses were identified. A CAT scan of the abdomen and pelvis with contrast was done which revealed ascites, peritoneal masses and a large left ovarian mass suspicious for ovarian malignancy. Incidentally, a left adrenal 4.3 cm mass was also identified. She underwent total hysterectomy and bilateral oophorectomy. An omentectomy, peritoneal mass biopsy, and lymph node sampling were also performed. The ovarian mass was diagnosed to be a fibroma. However, the peritoneal biopsies, revealed poorly differentiated carcinoma consistent with ACC.

She was found to have normal plasma metanephrines and normetanephrines at 0.15 and 0.88 nmol/L respectively. Urine metanephrines and normetanephrines were elevated mildly at 0.91 and 0.71mg/day perhaps due to the stress of the diagnosis. Morning cortisol was normal at 18 μg/dL and the ACTH was also normal at 49 pg/mL. Her electrolytes were normal except for mild hyponatremia. Sodium was 132mmol/L and potassium was 4.2 mmol/L. The patient was started on chemotherapy with etoposide, cisplatin, and doxorubicin along with mitotane. The patient was not able to tolerate the side effects of mitotane and chose to only be on platinum based chemotherapy agents.

Conclusion: This case highlights that patients with non-functioning ACC can present with clinical manifestations of tumor growth such as abdominal or flank pain or with constitutional symptoms like weight loss, anorexia. They may even be detected incidentally on imaging performed for a different reason. One needs to have a high index of suspicion to diagnose ACC given the low incidence rate. A careful history and physical examination should be performed to exclude pheochromocytoma, hyperaldosteronism, hyperandrogenism, and Cushing's syndrome. Complete surgical resection is the treatment of choice, but in cases with metastasis, chemotherapy and mitotane is needed. Five-year survival is approximately 45 to 60 percent for early stage disease, and 10 to 25 percent for advanced stage disease. As a result early diagnosis is key to better outcomes.

 

Nothing to Disclose: AH, LT