ENDOCRINE PRACTICE Rapid Electronic Article in Press
Rapid Electronic Articles in Press are preprinted manuscripts that have been reviewed and accepted for publication, but have yet to be edited, typeset and finalized. This version of the manuscript will be replaced with the final, published version after it has been published in the print edition of the journal. The final, published version may differ from this proof. DOI:10.4158/EP-2017-0122 C 2017 AACE.
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EP-2017-0122
VISUAL VIGNETTE: ADRENAL MASS AND CLITOROMEGALY
Run Yu, MD, PhD
From: Division of Endocrinology, UCLA David Geffen School of Medicine, Los Angeles, California
Corresponding address: Run Yu, MD, PhD Division of Endocrinology UCLA David Geffen School of Medicine 200 Medical Plaza Dr, Suite 530 Los Angeles, CA 90095 Email: runyu@mednet.ucla.edu
Case presentation: A 58-year-old female presented with abdominal mass. One month before presentation, she had had bilateral flank pain and epigastric abdominal pain for several weeks. Abdominal CT at an outside hospital showed a large solid mass (10.4 x 7.8 x 8.9 cm) interposed between the superior aspect of the stomach and the spleen and extensive para-aortic lymphadenopathy. Her past medical history included type 2 diabetes, anxiety, depression, obesity, and bariatric surgery. She did not appear DOI:10.4158/EP-2017-0122 C 2017 AACE.
Cushingoid. FDG PET/CT showed a large hypermetabolic left abdominal mass (axial CT image, asterisk) with left supraclavicular, retrocrural, and retroperitoneal lymphoadenopathy, and the CT part incidentally identified an enlarged clitoris (19 x 54 mm) (axial CT image, arrow) (Figure 1). What is the diagnosis?
Answer: Virilizing adrenocortical carcinoma. Further questioning revealed facial hair growth, frequent thoughts on sex, and clitoral enlargement in the previous months. She declined pelvic examination. Clitoromegaly is defined by a clitoral index (product of dimeter and length) >35 mm2 and is a sign of hyperandrogenism (1). This patient’s clitoral index was 1026 mm2 thus she had clear and prominent clitoromegaly. A large adrenal mass accompanied with prominent clitoromegaly makes virilizing adrenocortical carcinoma the only plausible diagnosis (2). Although clitoromegaly can be assessed clinically, it is very challenging for non-gynecologists to do pelvic examination in clinical practice due to privacy concern, inconvenience, and patient reluctance as in our case. The clitoris can be examined in detail by pelvic CT or MRI but is often neglected by radiologists and clinicians. As pelvic CT or MRI is commonly performed in patients suspected to have adrenal or pelvic mass, it is important to examine the clitoris on CT or MRI for “free” evidence of hyperandrogenism. On CT, the clitoris glans and shaft appear as a cylindrical tissue in the perineum; the clitoral index can be measured on axial views (3). Hormonal testing after the FDG PET/CT showed that total testosterone level was 461 ng/dL (normal range 2- 45), free testosterone 133 pg/mL (0.1 - 6.4),
dehydroepiandrosterone (DHEA) sulfate 10600 ng/ml (100-1600), androstenedione 1458 ng/dL (20-75), 24-hour urine free cortisol 1295.4 mcg/d ( ⇐ 45.0), and ACTH 3 pg/mL
(4-48). Biopsy of the left supraclavicular lymph node showed high-grade epithelioid neoplasm, steroidogenic, with neuroendocrine differentiation; Ki-67 labeling index was >50%. The hormonal and biopsy results confirmed the diagnosis.
References
1. Tagatz GE, Kopher RA, Nagel TC, Okagaki T. The clitoral index: a bioassay of androgenic stimulation. Obstet Gynecol. 1979;54:562-564.
2. Miller BS, Else T. Personalized care of patients with adrenocortical carcinoma: a comprehensive approach. Endocr Pract. 2017;23:705-715.
3. Hosseinzadeh K1, Heller MT, Houshmand G. Imaging of the female perineum in adults. Radiographics. 2012;32:E129-E168.
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