Jun Zhou1, Dandan Zhang2, Wencai Li2, Luting Zhou1, Haimin Xu1, Saifang Zheng1, Chaofu Wang3. 1. Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. 2. Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China. 3. Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. wangchaofu@126.com.
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.
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.
Authors: Jacques W M Lenders; Quan-Yang Duh; Graeme Eisenhofer; Anne-Paule Gimenez-Roqueplo; Stefan K G Grebe; Mohammad Hassan Murad; Mitsuhide Naruse; Karel Pacak; William F Young Journal: J Clin Endocrinol Metab Date: 2014-06 Impact factor: 5.958
Authors: Jung Min Lee; Mee Kyoung Kim; Seung Hyun Ko; Jung Min Koh; Bo Yeon Kim; Sang Wan Kim; Soo Kyung Kim; Hae Jin Kim; Ohk Hyun Ryu; Juri Park; Jung Soo Lim; Seong Yeon Kim; Young Kee Shong; Soon Jib Yoo Journal: Endocrinol Metab (Seoul) Date: 2017-06
Authors: Giuseppina Incampo; Luigi Di Filippo; Erika Maria Grossrubatscher; Paolo Dalino Ciaramella; Stefano Frara; Andrea Giustina; Paola Loli Journal: Endocrine Date: 2022-02-12 Impact factor: 3.633
Authors: Isabelle Fülber; Katharina Peer; Elisabeth Maurer; Detlef K Bartsch; Jannis Görlach; Joachim Nils Göbel; Marion Roeßler; Katharina Holzer Journal: Innov Surg Sci Date: 2020-08-31
Authors: Mussa H AlMalki; Metib Alotaibi; Mohammad Maswood Ahmad; Muhammad Amin Ur Rahman; Turki Alharthi Journal: Case Rep Endocrinol Date: 2020-01-30