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

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Abstract

Purpose

Adrenal schwannomas (AS) are rare tumours arising from Schwann cells. Due to the high prevalence of adrenal incidentalomas, running into very rare adrenal tumours has become a possibility for high volume expert centres. So far, the clinical behaviour and the radiological characteristics of AS make the pre-operative diagnosis of AS extremely challenging. Due to limited information available, we wanted to summarise the main features of this tumours, in order to raise the profile of an uncommon disease.

Methods

We performed a MEDLINE and EMBASE research to review the literature. We found 57 case reports and case series and a total of 169 cases, including 2 more cases found in our Institutions. We collected data regarding year of publication, sex, age, and, when available, clinical presentation, hormonal data, radiological features, tumour site and size, treatment, histology and follow-up.

Results

We analysed and discussed the clinical, radiological and pathological characteristics of cases identified, underlying the critical aspects of assessment and management of these tumours which still remain questioned, as, currently, pathologic examination is the only way to make the diagnosis.

Conclusions

The pre-operative diagnosis of AS is more than challenging and pathologic examination is so far the only way to make a certain diagnosis. Therefore, it is important to consider also the AS in the list of possible diagnoses when faced with a large not secreting adrenal tumour, with suspicious radiological features.

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Authors and Affiliations

  1. Institute of Endocrine and Metabolic Sciences, Università Vita-Salute San Raffaele and IRCCS Ospedale San Raffaele, Milan, Italy

    Giuseppina Incampo, Luigi Di Filippo, Stefano Frara, Andrea Giustina & Paola Loli

  2. Endocrine Unit, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy

    Erika Maria Grossrubatscher & Paolo Dalino Ciaramella

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  1. Giuseppina Incampo
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  2. Luigi Di Filippo
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  3. Erika Maria Grossrubatscher
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Contributions

Study conception and design (Paola Loli), literature search, data analysis and interpretation of data (Giuseppina Incampo, Luigi Di Filippo, Erika Grossrubatscher, Paolo Dalino Ciaramella), first draft of the manuscript (Giuseppina Incampo, Paola Loli), critical revision of the work (Paola Loli, Stefano Frara, Andrea Giustina). All Authors approved the version to be published.

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Correspondence to Paola Loli.

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Incampo, G., Di Filippo, L., Grossrubatscher, E.M. et al. Adrenal schwannoma: why should endocrinologists be aware of this uncommon tumour?. Endocrine 75, 684–697 (2022). https://doi.org/10.1007/s12020-022-02997-x

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