Extra­adrenal plexiform infiltration in mature ganglioneuromas are predictive of gonadal type steroidogenesis and interstitial Leydig cells

Background: Mature adrenal ganglioneuromas (MAGNs) are benign neoplasms of the combined neural crest, schwannian, and connective tissue origin, rarely occurring in the adrenal glands. This study presents the phenotypic and hormonal spectrum of these pre­operatively challenging neoplasms. Design: Demographic, diagnostic, surgical, and pathologic findings of patients who were adrenalectomized as a result of MAGN were retrospectively reviewed from the database of a tertiary referral hospital. Clinical data, as well as follow­up data, were collected retrospectively. All the patients received operative resection. Results: A total of 36 MAGN (24 males, 12 females) were retrieved. Mean age was 39.6 years (range 25–72). Thirty (83.3%) were asymptomatic, four (11.1%) complained of abdominal discomfort, and two (5.6%) had abdominal distension. Mean size of the tumors was 7.23 cm (range, 2.5­15 cm, twenty­four were larger than 5 cm). There was no recurrence, during a mean follow­up of 6.5 years (range, 1­10 years). A whorled appearance corresponding to interlacing bundles of Schwann cells and collagen fibers on histologic specimens was visualized in twelve tumors on T1­ and T2­weighted images. Tumors with markedly high signal intensity on T2­weighted images consisted histologically of a Part of: Poster VI ­ Wednesday PM ­ Endocrine 1:00 PM ­ 4:00 PM significant amount of myxoid stroma and relatively few cellular and fibrous components. Tumors with intermediate to high signal intensity consisted of numerous cellular and fibrous components and little myxoid stroma. The extra­adrenal extension was seen in 9 (6 males, three females) with a multifocal perivascular plexiform appearance and dystrophic calcification. These neoplasms were associated with gonadal type urinary steroid profile in women and interstitial Leydig cells. Conclusion: Pre­operative diagnosis of MAGNs remains difficult, in particular, those neoplasms with extra­adrenal extension. The presence in extra­adrenal neoplasms of dystrophic calcification, lobulated plexiform appearances, and gonadal type steroid in young females suggest the presence of Leydig cells, which is usually overlooked in males. A complete resection should be recommended once malignancy cannot be entirely excluded by pre­operative analyses.