Adrenal Ganglioneuroma: 20-year experience in a tertiary referral cancer center

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-22
Hassan Shawa*, Khaled M Elsayes, Sanaz Javadi, Ajaykumar Morani, Michelle D Williams, Steven Gerard Waguespack, Naifa Lamki Busaidy, Rena Vassilopoulou-Sellin, Camilo Jimenez and Mouhammed Amir Habra
The University of Texas MD Anderson Cancer Center, Houston, TX
Background: Adrenal ganglioneuroma (AGN) is a rare, differentiated, and usually benign neurogenic tumor. Because only limited clinical information, mostly derived from small case series, is available for AGN, we collected and reviewed data for AGN cases seen at a large institution over a 20-year period.

Objectives: To describe the clinical, biochemical, radiologic, and pathologic features of AGNs.

Methods: We retrospectively reviewed data for AGN cases seen at a single tertiary referral center between 1993 and 2012.

Results: Thirty four patients with AGNs were identified. Median age at diagnosis was 32.5 yr (range, 1.7–74 yr), and the female: male ratio was 2:1.  Fourteen AGNs (41%) were detected incidentally, but 10 patients (29%) presented with abdominal, flank, or back pain/discomfort. The median tumor size was 8 cm (range, 1.5–20 cm). Eight patients had a concurrent adrenal composite tumor including 5 five with pheochromocytoma (PC).  Most of AGNs were nonfunctional. Two patients had multiple endocrine neoplasia type 2 (MEN2A) syndrome (with a C634 proto-oncogene mutation), and one patient had spontaneous malignant transformation. On computed tomography imaging, AGNs were homogenous and well circumscribed, with relatively low attenuation on an unenhanced scan, a median density of 32.5 HU (range, 25–46 HU), and slight enhancement on post-contrast venous phase, with a median density of 40 HU (range, 27–114 HU). Three tumors had extra-adrenal extension. Of six adult patients followed for a median duration of 28.5 months (range, 12–60 months), none had any tumor growth. Nineteen patients had no recurrence or abdominal metastasis during a median follow-up of 43 months (range, 2–80 months).               

Conclusions: We herein described the largest AGN series reported to date.  AGNs are rare neoplasms that are usually nonfunctional and can mimic primary or secondary adrenal malignancies as well as PC on imaging. The natural history of AGNs is usually benign, although local extra-adrenal extension and spontaneous malignant transformation rarely occur.  

Nothing to Disclose: HS, KME, SJ, AM, MDW, SGW, NLB, RV, CJ, MAH

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Sources of Research Support: The University of Texas MD Anderson Cancer Center Core Grant (CA16672).