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Endocrine

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05.07.2019 | Original Article

Primary adrenal schwannoma: a series of 31 cases emphasizing their clinicopathologic features and favorable prognosis

verfasst von: Jun Zhou, Dandan Zhang, Wencai Li, Luting Zhou, Haimin Xu, Saifang Zheng, Chaofu Wang

Erschienen in: Endocrine | Ausgabe 3/2019

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Abstract

Purpose

Primary adrenal schwannoma (PAS) is a very rare benign tumor, and most of them have been described in case reports. This study aimed to analyze their distinct clinicopathologic features and follow-up data through the largest series yet.

Methods

Clinicopathologic features of 31 primary adrenal schwannomas were retrospectively studied. Imaging and histologic features were re-evaluated and summarized. Immunohistochemical markers were measured, including S100, SOX10, AE1/AE3, EMA, SMA, Desmin, HMB45, GLUT1, and Ki67. Follow-up of all cases was performed.

Results

All the tumors were clinically misdiagnosed as nonfunctioning adrenal adenoma (NAA; 23/31), aldosterone-producing adenoma/aldosteronoma (APA; 3/31), cortisol-producing adenoma (CPA; 3/31), or pheochromocytoma (PCC; 2/31). Some 87% (27/31) presented with adrenal incidentaloma, and 13% (4/31) had a clinical symptom or unregulated hormone levels. They comprised conventional (19/31), cellular (7/31), plexiform (2/31), ancient (1/31), epithelioid (1/31) and microcystic/reticular variants (1/31) and had various histologic features. Immunohistochemically, all tumors (31/31) were positive for S100 and Sox10, with a low Ki-67 proliferative index. In the long-term follow-up (mean, 53 mo.; median, 56 mo.), none had evidence of recurrence and metastasis. Univariate analysis showed that OS and DFS were not associated with age; sex; tumor side, size, or number; adrenal-related symptoms; gross feature (solid vs. cystic); or any histologic feature (P > 0.9999).

Conclusion

PAS is an extremely rare tumor and mostly appears as an incidentaloma. Clinically, it tends to be misdiagnosed as other common adrenal tumors. This tumor has a benign biologic behavior and prognosis, without correlations with clinical or histologic parameters.

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Titel: Primary adrenal schwannoma: a series of 31 cases emphasizing their clinicopathologic features and favorable prognosis
verfasst von: Jun Zhou
Dandan Zhang
Wencai Li
Luting Zhou
Haimin Xu
Saifang Zheng
Chaofu Wang
Publikationsdatum: 05.07.2019
Verlag: Springer US
Erschienen in: Endocrine / Ausgabe 3/2019
Print ISSN: 1355-008X
Elektronische ISSN: 1559-0100
DOI: https://doi.org/10.1007/s12020-019-01992-z

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