Premium
Adrenal ganglioneuroma: features and outcomes of 27 cases at a referral cancer centre
Author(s) -
Shawa Hassan,
Elsayes Khaled M.,
Javadi Sanaz,
Morani Ajaykumar,
Williams Michelle D.,
Lee Jeffrey E.,
Waguespack Steven G.,
Busaidy Naifa L.,
VassilopoulouSellin Rena,
Jimenez Camilo,
Habra Mouhammed Amir
Publication year - 2014
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.12320
Subject(s) - medicine , ganglioneuroma , magnetic resonance imaging , tertiary referral centre , radiology , hounsfield scale , pheochromocytoma , cancer , pathology , surgery , computed tomography , neuroblastoma , genetics , biology , cell culture
Background Adrenal ganglioneuroma ( AGN ) is a rare neurogenic tumour that can mimic other adrenal neoplasms. Limited information, mostly derived from small cases series, is available for AGN . Methods A retrospective review for AGN s seen at a tertiary referral centre describing important features to distinguish AGN from other adrenal neoplasms. Results Of 53 ganglioneuromas, 27 were AGN s. Median age was 31 years (range, 1·7–64 years) and median tumour size was 8 cm (range, 1·5–20 cm). Seventeen AGN s (63%) were detected incidentally and nine patients (33%) presented with abdominal/back discomfort. Catecholamine levels, available for 21 patients, were normal. On computed tomography ( CT ), most AGN s were homogenous and well circumscribed with a median density of 32·5 Hounsfield units ( HU ) on unenhanced CT ; 40 HU on postcontrast venous phase; and 66·5 HU on delayed postcontrast phase. On magnetic resonance imaging ( MRI ), AGN s had hypo‐intense signal on T1‐weighted images with heterogeneous hyperintense signal on T2‐weighted images. In four patients, there was no tumour growth during median follow‐up of 48 months (range, 21–60 months). One patient had malignant peripheral nerve sheath tumour arising from AGN . Thirteen patients with resected AGN had no recurrence during a median follow‐up of 50 months (range, 2–135 months). Conclusions We herein describe the largest AGN series reported to date. Isolated AGN s do not produce catecholamines and have CT imaging characteristics that can help in distinguishing them from other adrenal and para‐adrenal neoplasms. The natural history of AGN s is usually benign, although local extra‐adrenal extension or malignant transformation can rarely occur.