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

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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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Funding

The study was supported by the Shanghai science and technology commission (17ZR1417500).

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Author notes

  1. These authors contributed equally: Jun Zhou, Dandan Zhang

Authors and Affiliations

  1. Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China

    Jun Zhou, Luting Zhou, Haimin Xu, Saifang Zheng & Chaofu Wang

  2. Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China

    Dandan Zhang & Wencai Li

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  1. Jun Zhou
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Correspondence to Chaofu Wang.

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Zhou, J., Zhang, D., Li, W. et al. Primary adrenal schwannoma: a series of 31 cases emphasizing their clinicopathologic features and favorable prognosis. Endocrine 65, 662–674 (2019). https://doi.org/10.1007/s12020-019-01992-z

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