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Appendiceal schwannoma — report of a case and literature review

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Abstract

Appendiceal tumours encompass a wide spectrum of differential diagnoses and frequently present with clinical features of appendicitis. We report the case of a 43-year-old woman who presented with epigastric pain, dyspepsia and bloating. An atypical right para-iliac mass was detected on abdominal ultrasound, and computed tomography (CT) identified an appendiceal tumour. The tumour subtype remained indeterminate following Gallium-68 Dotatate positron emission tomography (PET); however, an appendiceal neuroendocrine tumour was suspected. Surgical resection with laparoscopic en bloc appendicectomy and limited caecectomy was performed, and histopathological assessment confirmed an appendiceal schwannoma. The report is followed by a review of the literature. To our knowledge, there have been fourteen reported cases of appendiceal schwannoma. The preoperative diagnosis can be challenging and appendiceal schwannoma had not been suspected in any of the reported cases, while a suspected diagnosis of neuroendocrine tumour or gastrointestinal stromal tumour was common. Definitive diagnosis requires immunohistochemical assessment and S100 is the hallmark. No personal or family history of underlying neurofibromatosis (NF) type 1 or type 2 has been reported to date. As for other gastrointestinal schwannomas, complete surgical resection is the recommended treatment for appendiceal schwannoma. Following this, despite lack of long-term follow-up, no cases of recurrence have been reported thus far.

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Authors and Affiliations

  1. Department of Academic Surgery, The Royal Marsden NHS Foundation Trust, Fulham Road, London, SW3 6JJ, UK

    Joseph P. Doyle, Ricky H. Bhogal & Vasileios K. Mavroeidis

  2. Department of Pathology, The Royal Marsden NHS Foundation Trust, London, SW3 6JJ, UK

    Monica Terlizzo

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  1. Joseph P. Doyle
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  2. Ricky H. Bhogal
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  3. Monica Terlizzo
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  4. Vasileios K. Mavroeidis
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Contributions

RB and VM gave substantial contributions to the conception or the design of the manuscript. JD participated in drafting the manuscript. RB, VM and MT revised it critically. All authors read and approved the final version of the manuscript.

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Correspondence to Joseph P. Doyle.

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Doyle, J.P., Bhogal, R.H., Terlizzo, M. et al. Appendiceal schwannoma — report of a case and literature review. Ir J Med Sci 192, 1801–1805 (2023). https://doi.org/10.1007/s11845-022-03184-7

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  • DOI: https://doi.org/10.1007/s11845-022-03184-7

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