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      Clinical features and outcomes of adrenal schwannoma: a study of 13 cases from a single centre

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          Abstract

          Background

          Adrenal schwannomas (AS) are extremely rare neoplasms. This study shares our experience regarding the diagnosis and operative management of AS.

          Methods

          Clinical details, radiologic, laboratory, and pathologic findings as well as follow-up data were analysed retrospectively for 13 AS patients who accepted surgery at a tertiary referral hospital in China between 1 January 1996, and 31 December 2017.

          Results

          The mean age of the patients at diagnosis was 44.7 ± 13.7 years (range 19–62 years; male: female ratio, 1:1.16), of whom seven patients had unilateral AS on the right side, and the remaining six on the left side. None of the cases were hormonally active. None of the 13 cases were diagnosed as AS by CT imaging before the operation. Among the patients, ten were asymptomatic. The mean preoperative size was 7.1 ± 3.2 cm (range 1.6–12.6 cm). All patients underwent surgery, with open adrenalectomy in five patients and laparoscopy in eight patients. The mean tumor size on pathologic examination was 6.8 ± 3.0 cm (range 3.0–11.7 cm). The surgical specimens were confirmed by pathological examination. During a median follow-up of 60.8 ± 17.7 months, no patients showed recurrence or metastasis.

          Conclusion

          The preoperative diagnosis of AS remains difficult despite the advances in imaging examinations. After complete resection, the prognosis of AS is excellent.

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          Most cited references30

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          The clinically inapparent adrenal mass: update in diagnosis and management.

          Clinically inapparent adrenal masses are incidentally detected after imaging studies conducted for reasons other than the evaluation of the adrenal glands. They have frequently been referred to as adrenal incidentalomas. In preparation for a National Institutes of Health State-of-the-Science Conference on this topic, extensive literature research, including Medline, BIOSIS, and Embase between 1966 and July 2002, as well as references of published metaanalyses and selected review articles identified more than 5400 citations. Based on 699 articles that were retrieved for further examination, we provide a comprehensive update of the diagnostic and therapeutic approaches focusing on endocrine and radiological features as well as surgical options. In addition, we present recent developments in the discovery of tumor markers, endocrine testing for subclinical disease including autonomous glucocorticoid hypersecretion and silent pheochromocytoma, novel imaging techniques, and minimally invasive surgery. Based on the statements of the conference, the available literature, and ongoing studies, our aim is to provide practical recommendations for the management of this common entity and to highlight areas for future studies and research.
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            Neurogenic tumors in the abdomen: tumor types and imaging characteristics.

            There is a broad spectrum of neurogenic tumors that involve the abdomen. These tumors can be classified as those of (a) ganglion cell origin (ganglioneuromas, ganglioneuroblastomas, neuroblastomas), (b) paraganglionic system origin (pheochromocytomas, paragangliomas), and (c) nerve sheath origin (neurilemmomas, neurofibromas, neurofibromatosis, malignant nerve sheath tumors). Abdominal neurogenic tumors are most commonly located in the retroperitoneum, especially in the paraspinal areas and adrenal glands. All of these tumors except neuroblastomas and ganglioneuroblastomas are seen in adult patients. Abdominal neurogenic tumor commonly manifests radiologically as a well-defined, smooth or lobulated mass. Calcification may be seen in all types of neurogenic tumors. The diagnosis of abdominal neurogenic tumor is suggested by the imaging appearance of the lesion, including its location, shape, and internal architecture. Benign and malignant neurogenic tumors are difficult to differentiate unless distant metastatic foci are seen. For malignant tumors, imaging modalities other than computed tomography (CT) and magnetic resonance (MR) imaging may be necessary for staging. However, because most neurogenic tumors in adults are benign, CT and MR imaging can be used to develop a differential diagnosis and help determine the immediate local extent of tumor. Copyright RSNA, 2003.
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              The genomic landscape of schwannoma.

              Schwannomas are common peripheral nerve sheath tumors that can cause debilitating morbidities. We performed an integrative analysis to determine genomic aberrations common to sporadic schwannomas. Exome sequence analysis with validation by targeted DNA sequencing of 125 samples uncovered, in addition to expected NF2 disruption, recurrent mutations in ARID1A, ARID1B and DDR1. RNA sequencing identified a recurrent in-frame SH3PXD2A-HTRA1 fusion in 12/125 (10%) cases, and genomic analysis demonstrated the mechanism as resulting from a balanced 19-Mb chromosomal inversion on chromosome 10q. The fusion was associated with male gender predominance, occurring in one out of every six men with schwannoma. Methylation profiling identified distinct molecular subgroups of schwannomas that were associated with anatomical location. Expression of the SH3PXD2A-HTRA1 fusion resulted in elevated phosphorylated ERK, increased proliferation, increased invasion and in vivo tumorigenesis. Targeting of the MEK-ERK pathway was effective in fusion-positive Schwann cells, suggesting a possible therapeutic approach for this subset of tumors.

                Author and article information

                Journal
                Endocr Connect
                Endocr Connect
                EC
                Endocrine Connections
                Bioscientifica Ltd (Bristol )
                2049-3614
                28 April 2021
                01 May 2021
                : 10
                : 5
                : 543-549
                Affiliations
                [1 ]Department of Urology, Department of Urology, Wuzhou GongRen Hospital , Wuzhou, China
                [2 ]Department of Urology, The First Affiliated Hospital of GuangXi Medical University , Nanning China
                [3 ]Department of Pathology, The First Affliated Hospital of GuangXi Medical University , Nanning, China
                Author notes
                Correspondence should be addressed to W Fu: fuwj66@ 123456aliyun.com

                *(H Huang and Q Ding contributed equally to this work)

                Article
                EC-21-0062
                10.1530/EC-21-0062
                8183624
                33909596
                c6c721f4-5181-445a-a2a3-97cacada3240
                © The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 21 April 2021
                : 28 April 2021
                Categories
                Research

                adrenal schwannoma,clinical features,outcomes
                adrenal schwannoma, clinical features, outcomes

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