Adrenal schwannoma: why should endocrinologists be aware of this uncommon tumour?

Incampo, G.; Filippo, Luigi di; Grossrubatscher, Erika; Ciaramella, Paolo Dalino; Frara, Stefano; Giustina, Andrea; Loli, Paola

doi:10.1007/s12020-022-02997-x

Cited by 8 publications

(24 citation statements)

References 79 publications

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“…In agreement with the previous data, we found a female predominance. 2 Most patients in our study were discovered with schwannomas incidentally (92%) and only one patient presented with symptoms of mass effect. In contrast, around 30% of patients presented with symptoms of mass effect in the systematic review.…”

Section: Discussionmentioning

confidence: 69%

“…juxta-adrenal lesions. 2 Adrenal schwannomas were reported to be large with a median size of 6 cm, unilateral, with indeterminate imaging characteristics and cystic component in a third of cases. 2 Sixty-two percent of patients were asymptomatic at the time of discovery, with the rest reporting symptoms of mass effect, such as abdominal pain.…”

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confidence: 99%

“…2 Adrenal schwannomas were reported to be large with a median size of 6 cm, unilateral, with indeterminate imaging characteristics and cystic component in a third of cases. 2 Sixty-two percent of patients were asymptomatic at the time of discovery, with the rest reporting symptoms of mass effect, such as abdominal pain. 2 However, the reported data were likely influenced by publication bias with only more severe cases being published and the inclusion of patients before the widespread availability of crosssectional imaging leading to likely higher proportion of patients presenting with symptoms of mass effect and larger size.…”

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confidence: 99%

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Adrenal and juxta‐adrenal schwannomas: A single‐centre study

Rolak

Dogra

McKenzie

et al. 2023

Clinical Endocrinology

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Objective Adrenal schwannomas and juxta‐adrenal schwannomas are rare tumours. We aimed to summarise their clinical, biochemical and imaging characteristics. Design Single‐centre retrospective study of eligible patients between 1995 and 2022. Patients and Measurements Patients with a histopathologic diagnosis of adrenal or juxta‐adrenal schwannoma. Results Twenty‐four patients were diagnosed with either primary adrenal schwannoma (8, 33%) or juxta‐adrenal schwannoma (16, 67%). Most tumours (21, 88%) were discovered incidentally on imaging. All tumours were unilateral, with 15 (62%) on the left and 9 (38%) on the right. At diagnosis, the median tumour size was 4 cm (range, 2–13 cm). Adrenal schwannomas were smaller when compared to juxta‐adrenal schwannomas (median of 3.1 cm [range, 2–9 cm] vs. 4.6 cm [range, 2.3–13.3 cm], p = .037). On imaging, the tumours were round or oval in shape in 16 (70%), lobulated in 7 (30%), solid in 15 (68%), solid‐cystic in 7 (32%), heterogeneous in 14 (61%) and homogeneous in 9 (39%). The median unenhanced computed tomography attenuation was 30 Hounsfield units (HU) (range, 12–38 HU). Of the 20 patients who underwent complete hormonal testing, all had nonfunctioning tumours. There was no recurrence or new tumour development in our cohort. Conclusions Adrenal and juxta‐adrenal schwannomas are nonfunctioning benign tumours that present with indeterminate radiographic features, including large tumour size and increased unenhanced CT attenuation. We did not find an imaging phenotype that was diagnostic of schwannoma. The diagnosis of this rare tumour is based on biopsy or resection.

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Section: Discussionmentioning

confidence: 69%

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confidence: 99%

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confidence: 99%

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Adrenal and juxta‐adrenal schwannomas: A single‐centre study

Rolak

Dogra

McKenzie

et al. 2023

Clinical Endocrinology

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“…Like other schwannomas, AS may have the following histological features, such as intact capsule, Antoni A and B areas, verocay body, hyalinization, myxoid stroma, and foam cells. The immunohistochemical features of benign AS have the following commonalities: most are positive for S-100 or SOX-10, but negative for keratin, desmin, actin, CD34, and CD117 ( 5 , 20 ). The positive expression of Ki-67 in eight patients with AS was less than 5%, and their low proliferation index suggested that they had the biological nature of laziness.…”

Section: Discussionmentioning

confidence: 99%

“…Due to the low incidence of AS, only a few case reports and small case series have been published so far, and the total number of AS cases is less than 200 ( 5 ). From April 2007 to April 2022, the affiliated hospital of Guizhou Medical University treated 1309 patients who underwent surgical resection of adrenal lesions, of which only eight were diagnosed as AS, accounting for only 0.61%.…”

Section: Introductionmentioning

confidence: 99%

Clinical presentation, management, and research progress of adrenal schwannoma

Zhang

Wen

et al. 2022

Front. Surg.

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ObjectiveThis study shares our experience in managing adrenal schwannoma (AS).MethodsThe clinical data of eight patients with AS in our hospital from April 2007 to April 2022 were analyzed retrospectively.ResultsA total of 1309 patients with adrenal lesions were treated in the affiliated hospital of Guizhou Medical University for 15 years, of which only 8 cases were diagnosed as AS, accounting for 0.61%. Among the eight patients with AS, there were five females and three males, with an average age of 48.63 ± 12.05 years, and the average maximum diameter of the tumor was 6.96 ± 1.83 cm. All patients underwent adrenalectomy and were pathologically diagnosed as AS after the operation. The average follow-up time of eight patients with AS was 60.13 ± 22.33 months, and there was no recurrence or metastasis.ConclusionThe retroperitoneum is an uncommon site for schwannoma tumors, and among adrenal incidentalomas, the schwannoma is rare. The disease lacks specific clinical and imaging features, but correct diagnosis before the pathological examination is very important for clinical management and surgical decision. When imaging examination indicates a slow-growing retroperitoneal mass, schwannoma should be considered. Surgical resection is the main treatment. Pathology is the gold standard for diagnosis. Most of the tumors are benign and have a good prognosis. There is a risk of recurrence after the operation, and it should be monitored actively.

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Adrenal and periadrenal schwannoma: histological, molecular and clinical characterization of an institutional case series

et al. 2023

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Purpose Adrenal schwannoma (AS) and periadrenal schwannoma (PAS) are exceedingly rare Schwann cell tumors that develop from the adrenal medulla and periadrenal peripheral nerves respectively. The underlying genetic events are elusive. Methods We searched our institutional database for AS/PAS cases and reviewed the histology and clinical outcome. Comprehensive molecular work-up was performed. Results We found reports of 4 AS/PAS cases diagnosed between 1992 and 2022 among the 1248 adrenal lesions submitted for histopathology during the same time period (0.32%). Two patients were male, two were female, and the age span was 59–80 years. Median size was 70 mm (range 50–100 mm), and from a radiology perspective, the lesions were initially suspected of malignant lesions originating from either adrenals or kidneys. Hormonal analyses were normal in all cases. Histologically, three cases were annotated as cellular AS or PAS, and one case was annotated as microcystic AS. Molecular characterization using focused next-generation sequencing did not identify SMARCB1 or NF2 mutations, alterations previously associated to schwannoma at other anatomical sites. The postoperative period was without complications for all patients, and follow-up did not show any signs of relapse or metastatic disease. Conclusion AS/PAS are rare neoplasms that are most often benign, and the molecular etiology is most likely not related to mutations in established schwannoma-related genes. Since these tumors may be misinterpreted as malignant, knowledge of this entity is essential for radiologists, endocrinologists, surgeons and pathologists.

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Adrenal schwannoma: why should endocrinologists be aware of this uncommon tumour?

Cited by 8 publications

References 79 publications

Adrenal and juxta‐adrenal schwannomas: A single‐centre study

Adrenal and juxta‐adrenal schwannomas: A single‐centre study

Clinical presentation, management, and research progress of adrenal schwannoma

Adrenal and periadrenal schwannoma: histological, molecular and clinical characterization of an institutional case series

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