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Huong D. Nguyen, MD; Thanh D. Hoang, DO; Vinh Q. Mai, DO; Patrick W. Clyde, MD, FACP, FACE; Babette C. Glister, MD; K. M. Mohamed Shakir, MD, FRCP, MACP, MACE From the Division of Endocrinology, National Naval Medical Center, Bethesda, Maryland
Case Presentation: A 49-year-old woman with a history of hypertension and newly diagnosed type 2 diabetes mellitus pre- sented with progressively worsening weakness and malaise during the previous 3 months. She also reported facial and ab- dominal hair growth, bilateral lower extremity edema, enlarging abdominal girth, male-pattern scalp hair loss, intermittent palpitations, and sweating. She had had no easy bruising, proximal muscle weakness, or headache. Menopause had occurred at age 40 years. On examination, the patient was slightly hypertensive. She appeared well developed and somewhat plethoric. Her coarse facial hair had been shaved. She had no moon facies, buffalo hump, supraclavicular fullness, striae, or ecchymoses. Her abdomen was distended with a 15-cm firm mass on the right side. She had severe pitting edema in both lower extremities. There was coarse hair in the midabdominal area and on the back. Laboratory tests revealed the following results (reference ranges shown parenthetically): total testosterone, 471 ng/dL (2 to 45); dehydroepiandrosterone sulfate, 276 ug/dL (35 to 256); cortisol after 1 mg overnight dexamethasone suppression, 36 ug/dL; midnight cortisol, 33 ug/dL; and cortisol at 7 AM, 33 ug/ dL; adrenocorticotropic hormone, <5 pg/mL; normal urine metanephrines and catecholamines; plasma aldosterone concentra- tion, <1 ng/dL; renin activity, 2.9 ng/ml per hour; and estradiol, 886 pg/mL. Abdominal computed tomographic scans were obtained (Fig. 1 and 2). What is the diagnosis?
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Fig. 2
Answer: Adrenocortical carcinoma. Typically, adrenocortical carcinoma manifests as a heteroge- neous adrenal mass larger than 10 cm with irregu- lar margins and areas of necrosis (1). Moreover, it may show evidence of vascular or capsular invasion. Adrenocortical carcinoma can secrete steroids, steroid precursors, and mineralocorti- coids. Cosecretion of androgens and corticoste- roids suggests the presence of a malignant tumor (1,2). Benign, functional adrenal tumors normally secrete only one class of hormone. The primary treatment of adrenocortical carcinoma manifesting in early stages is surgical resection; however, 20% of cases of adrenocortical carcinoma are unresect- able because of advanced disease. Mitotane alone
or in combination with etoposide, doxorubicin, and cisplatin is the primary therapy for metastatic adrenocortical carcinoma (3). Overall 5-year survival with complete resection is approximately 32% to 48% (1). For those patients with distant metastatic involvement, the prognosis is poor, with a 5-year survival rate of 0% (2).
Our patient had evidence of cosecretion of testosterone and cortisol. In addition, her estradiol level was extremely elevated, which may have resulted from peripheral testosterone conversion or direct tumor secretion. Because of pulmonary metastatic lesions, she was classified as having stage IV disease and subsequently referred for chemotherapy.
In conclusion, in postmenopausal women who present with symptoms and signs of hypercortisolism and hyperandrogen- ism, adrenocortical carcinoma should be included in the differential diagnoses.
DISCLAIMER
The views expressed in this material are those of the authors and do not necessarily reflect the official policy or position of the US Department of the Navy, the US Department of Defense, or the US government.
DISCLOSURE
The authors have no multiplicity of interest to disclose.
REFERENCES
1. Wandoloski M, Bussey KJ, Demeure M.J. Adrenocortical cancer. Surg Clin North Am. 2009;89:1255-1267.
2. Strosberg JR, Hammer GD, Doherty GM. Management of adrenocortical carcinoma [with quiz]. J Natl Compr Canc Netw. 2009; 7:752-759.
3. Berruti A, Terzolo M, Sperone P, et al. Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocorti- cal carcinoma: a large prospective phase II trial. Endocr Relat Cancer. 2005;12:657-666.
Address correspondence to Dr. Huong D. Nguyen, Division of Endocrinology, National Naval Medical Center, 8901 Wisconsin Avenue, Bethesda, MD 20889.
E-mail: huong.nguyen@med.navy.mil.
Published as a Rapid Electronic Article in Press at http://www.endocrinepractice.org on August 16, 2010. DOI: 10.4158/EP10171.VV Copyright @ 2010 AACE.
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