Large Virilizing Benign Adrenal Rest Tumor of Retroperitoneum

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 1-36-Adrenal Incidentaloma & Carcinoma
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-26
Sravanthi Nagavalli*1, Mary D Le1, Luciano Barajas2 and Andrew George Gianoukakis3
1Harbor-UCLA Medical Center, Torrance, CA, 2Harbor-UCLA Medical Center and Los Angeles Biomedical Research Center, Torrance, 3Harbor UCLA Med Ctr, Torrance, CA
Background: In adults, the presence of adrenal rest tissue and its neoplastic transformation is rare. We present a case of a large virilizing, retroperitoneal adrenal rest tumor in a young female.

Clinical case: A 24 year old female presented with amenorrhea and hirsutism of 4 years duration. Menarche occurred at age 11 and menstrual cycles were initially regular. After 8 years, cycles became irregular followed by amenorrhea for the past four years. The patient denied voice changes and was normotensive. PE disclosed coarse terminal hair on face, extremities, trunk, abdomen and pelvic area along with male pattern baldness, right flank fullness and clitoromegaly.  Serum electrolytes were normal. Total testosterone (TT)= 977 ng/dl (n: <10-75 ng/dl ), FSH= 5.8 mIU/ml (n: 3.85-5.12 mIU/ml), LH= 6.3 mIU/ml (n: 4.21-72.24 mIU/ml), Estradiol= 16 pg/ml (n:21-649 pg/ml), Prolactin= 18.49 ng/ml (n:5.18-26.5 ng/ml ),DHEAS= 2876 mcg/dl (n:45-320 mcg/dl ), 17 OHP =63 (n:<285 ng/dl ). 24 hr urine cortisol=35.6 mcg (n: 4-50 mcg). CT revealed a 13 cm solid heterogeneous right-sided abdominal mass, normal adrenal glands, and mildly enlarged pathologic retroperitoneal lymph nodes. Diagnosis of adrenal carcinoma was considered.  Intraoperatively, a retroperitoneal mass intruding into the right renal hilum was noted. A 16 x 13 x 4.5 cm/540 gram unencapsulated mass was resected along with the right kidney. Histologically, the mass was consistent with adrenal tissue. Areas of nuclear atypia and very rare mitoses were noted. Immunohistochemistry was positive for inhibin but negative for AE1/AE3, α-actin, S100 and HMB45. The pathological diagnosis was adrenal cortical neoplasm favoring adenoma with atypia. Alternate diagnoses such as adrenal cortical carcinoma, pheochromocytoma, or renal cell carcinoma were not supported by the immunohistochemical profile. Lymph nodes, right kidney and adrenal gland were uninvolved.  Thus, the mass likely originated from retroperitoneal adrenal rests. Postoperatively, at 2 weeks: TT =46 ng/dl and DHEAS=237 mcg/dl. Hair growth and frequency of depilation decreased. Regular menses resumed one month after surgery.

Conclusion: When encountered in the setting of adrenal lesions, a high level of DHEAS raises suspicion for malignancy. Surprisingly, this virilizing adrenal rest tumor histologically favored a benign tumor with atypia. The patient will be monitored closely with biochemical and imaging surveillance.

Nothing to Disclose: SN, MDL, LB, AGG

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