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Gastric plexiform schwannoma in association with neurofibromatosis type 2

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Abstract

Plexiform schwannoma (PS) is an uncommon variant of schwannoma characterized by a multinodular (plexiform) growth pattern. It comprises up to 5 % of all schwannomas. The association between PS and neurofibromatosis type 1 or type 2 (NF1/NF2) is only rarely reported. Most cases of PS occur in the skin and subcutaneous soft tissue, with only a few reports of digestive tract involvement. We describe an 18-year-old male with NF2 who had bilateral vestibular schwannomas and multiple cutaneous PSs, and a 3-year history of abdominal pain. The patient ultimately underwent a distal gastrectomy for a partially obstructing submucosal antral mass, associated with an overlying ulcer. Histopathologic examination showed the mass to be a PS. The patient is alive and well, without symptoms, 12 months postoperatively. A review of the English language medical literature yielded only ten examples of PS arising in the digestive tract. Our patient is the first to be reported to have a gastric PS, and only the second patient to be reported with a digestive tract PS to have NF2, and the only patient reported to have both digestive tract and cutaneous PSs. Despite its rare occurrence with NF2, the finding of PS at any site should stimulate an examination for other manifestations of this disorder.

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Abbreviations

PS:

Plexiform schwannoma

References

  1. Goldblum JR, Folpe AL, Weiss SW, et al., editors. Enzinger and Weiss’s soft tissue tumors. 6th ed. Philadelphia: Elsevier; 2014.

    Google Scholar 

  2. Berg JC, Scheithauer BW, Spinner RJ, et al. Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region. Hum Pathol. 2008;39(5):633–40.

    Article  PubMed  Google Scholar 

  3. Iwashita T, Enjoji M. Plexiform neurilemmoma: a clinicopathological and immunohistochemical analysis of 23 tumours from 20 patients. Virchows Arch A Pathol Anat Histopathol. 1987;411(4):305–9.

    Article  CAS  PubMed  Google Scholar 

  4. Agaram NP, Prakash S, Antonescu CR. Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. Am J Surg Pathol. 2005;29(8):1042–8.

    PubMed  Google Scholar 

  5. Aktekin A, Ozkara S, Meric K, et al. Plexiform schwannoma of the duodenum accompanying pyloric stenosis: report of a case. Turk J Gastroenterol. 2012;23(4):385–9.

    Article  PubMed  Google Scholar 

  6. Cokelaere K, Sciot R, Geboes K. Esophageal plexiform schwannoma. Int J Surg Pathol. 2000;8(4):353–7.

    Article  PubMed  Google Scholar 

  7. Hirose T, Scheithauer BW, Sano T. Giant plexiform schwannoma: a report of two cases with soft tissue and visceral involvement. Mod Pathol. 1997;10(11):1075–81.

    CAS  PubMed  Google Scholar 

  8. Iida A, Imamura Y, Katayama K, et al. Plexiform schwannoma of the small intestine: report of a case. Surg Today. 2003;33(12):940–3.

    Article  PubMed  Google Scholar 

  9. Jacobson BC, Hirsch MS, Lee JH, et al. Multiple asymptomatic plexiform schwannomas of the sigmoid colon: a case report and review. Gastrointest Endosc. 2001;53(7):801–4.

    Article  CAS  PubMed  Google Scholar 

  10. Kawaguchi S, Yamamoto R, Yamamura M, et al. Plexiform schwannoma of the rectum. Dig Endosc. 2014;26(1):113–6.

    Article  PubMed  Google Scholar 

  11. Miettinen M, Shekitka KM, Sobin LH. Schwannomas in the colon and rectum: a clinicopathologic and immunohistochemical study of 20 cases. Am J Surg Pathol. 2001;25(7):846–55.

    Article  CAS  PubMed  Google Scholar 

  12. Retrosi G, Nanni L, Ricci R, et al. Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2. J Pediatr Surg. 2009;44(7):1458–61.

    Article  PubMed  Google Scholar 

  13. Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 2009;361(4):358–67.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Ammoun S, Cunliffe CH, Allen JC, et al. ErbB/HER receptor activation and preclinical efficacy of lapatinib in vestibular schwannoma. Neuro Oncol. 2010;12(8):834–43.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Nishio J, Mori S, Nabeshima K, et al. Successful enucleation of large multinodular/plexiform schwannoma of the foot and ankle. Springerplus. 2015;4:260.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Rege TA, Wagner AJ, Corless CL, et al. “Pediatric-type” gastrointestinal stromal tumors in adults: distinctive histology predicts genotype and clinical behavior. Am J Surg Pathol. 2011;35(4):495–504.

    Article  PubMed  Google Scholar 

  17. Miettinen M, Makhlouf HR, Sobin LH, et al. Plexiform fibromyxoma: a distinctive benign gastric antral neoplasm not to be confused with a myxoid GIST. Am J Surg Pathol. 2009;33(11):1624–32.

    Article  PubMed  Google Scholar 

  18. Posner MA, McMahon MS, Desai P. Plexiform schwannoma (neurilemmoma) associated with macrodactyly: a case report. J Hand Surg Am. 1996;21(4):707–10.

    Article  CAS  PubMed  Google Scholar 

  19. Bendon CL, Giele HP. Macrodactyly in the setting of a plexiform schwannoma in neurofibromatosis type 2: case report. J Hand Surg Am. 2013;38(4):740–4.

    Article  PubMed  Google Scholar 

  20. Hebert-Blouin MN, Amrami KK, Scheithauer BW, et al. Multinodular/plexiform (multifascicular) schwannomas of major peripheral nerves: an underrecognized part of the spectrum of schwannomas. J Neurosurg. 2010;112(2):372–82.

    Article  PubMed  Google Scholar 

  21. Itenberg S, Turner R, Amin B, et al. Eruptive plexiform schwannomas in a child with neurofibromatosis I. J Drugs Dermatol. 2011;10(10):1210–1.

    PubMed  Google Scholar 

  22. Ko JY, Kim JE, Kim YH, et al. Cutaneous plexiform schwannomas in a patient with neurofibromatosis type 2. Ann Dermatol. 2009;21(4):402–5.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Nagato T, Katada A, Yoshizaki T, et al. Laryngeal plexiform schwannoma as first symptom in a patient with neurofibromatosis type 2. Clin Neurol Neurosurg. 2010;112(6):505–8.

    Article  PubMed  Google Scholar 

  24. Nguyen TV, Matthews MR, Barrera FF, et al. Multiple cutaneous plexiform schwannomas as initial presentation of neurofibromatosis 2 in a 9-year-old. Pediatr Dermatol. 2012;29(4):536–8.

    Article  PubMed  Google Scholar 

  25. Purcell SM, Dixon SL. Schwannomatosis. An unusual variant of neurofibromatosis or a distinct clinical entity? Arch Dermatol. 1989;125(3):390–3.

    Article  CAS  PubMed  Google Scholar 

  26. Raimondo L, Garzaro M, Mazibrada J, et al. Plexiform schwannoma of the posterior pharyngeal wall in a patient with neurofibromatosis 2. Ear Nose Throat J. 2015;94(3):E17–9.

    PubMed  Google Scholar 

  27. Murata Y, Kumano K, Ugai K, et al. Neurilemmomatosis. Br J Dermatol. 1991;125(5):466–8.

    Article  CAS  PubMed  Google Scholar 

  28. Shishiba T, Niimura M, Ohtsuka F, et al. Multiple cutaneous neurilemmomas as a skin manifestation of neurilemmomatosis. J Am Acad Dermatol. 1984;10(5 Pt 1):744–54.

    Article  CAS  PubMed  Google Scholar 

  29. Rongioletti F, Drago F, Rebora A. Multiple cutaneous plexiform schwannomas with tumors of the central nervous system. Arch Dermatol. 1989;125(3):431–2.

    Article  CAS  PubMed  Google Scholar 

  30. Katsumi K, Ogose A, Hotta T, et al. Plexiform schwannoma of the forearm. Skeletal Radiol. 2003;32(12):719–23.

    Article  PubMed  Google Scholar 

  31. Guillemaud J, El-Hakim H, Chauhan N, et al. A pediatric case of neurofibromatosis type 2 presenting as a plexiform schwannoma of the intraparotid facial nerve. Int J Pediatr Otorhinolaryngol Extra. 2006;1(3):220–5.

    Article  Google Scholar 

  32. Sheikh S, Gomes M, Montgomery E. Multiple plexiform schwannomas in a patient with neurofibromatosis. J Thorac Cardiovasc Surg. 1998;115(1):240–2.

    Article  CAS  PubMed  Google Scholar 

  33. Attia EA, Yassin M, Lasheen MA, et al. Multiple isolated cutaneous plexiform schwannomas. Indian J Dermatol Venereol Leprol. 2011;77(5):594–6.

    Article  PubMed  Google Scholar 

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Correspondence to Satoru Kudose.

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All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Informed consent was obtained from all patients for being included in the study.

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Kudose, S., Kyriakos, M. & Awad, M.M. Gastric plexiform schwannoma in association with neurofibromatosis type 2. Clin J Gastroenterol 9, 352–357 (2016). https://doi.org/10.1007/s12328-016-0687-y

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  • DOI: https://doi.org/10.1007/s12328-016-0687-y

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