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A Case Report: A Rare Case of Multiple Chest Wall Schwannomas Associated with Uterine Leiomyoma and Infertility

Received: 12 November 2017     Accepted: 14 December 2017     Published: 8 January 2018
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Abstract

Schwannomatosis is a tumor predisposition syndrome characterized by development of multiple schwannomas that are slow-growing encapsulating neurogenic tumors. It is a recurrent condition and patients lack usually the classic cutaneous stigmata, vestibular schwannomas or brain parenchyma tumors. Many papers have incriminated the mutation of the SMARCB1 gene in this disease which has been also observed in cases of uterine leiomyoma. This report describes a rare case of multiple chest wall schwannomas associated to a uterine fibroma. A genetic study done did not show any SMARCB1 gene mutation. Subsequently a further genetic counselling and a whole exome sequencing were recommended especially that the patient underwent many trials of failed IVF and two miscarriages despite resection of all the tumors.

Published in International Journal of Cardiovascular and Thoracic Surgery (Volume 4, Issue 1)
DOI 10.11648/j.ijcts.20180401.11
Page(s) 1-4
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2018. Published by Science Publishing Group

Keywords

Schwannomatosis, Leiomyoma, Infertility, Genetic Mutation

References
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[4] Hulsebos TJM, plompAS, WoltermanRA et al. Germline mutation of IN1/SMARCB1 in familial schwannomatosis. Am J Hum Genet. 2007; 80: 805-810.
[5] Plotkin SR. Blakely JO, Evans DG et al. Update from the 2011 International Schwannomatosis workshop from genetics to diagnostic criteria. Am J Med Genet A. 2013; 161: 405-416.
[6] Smith MJ, KulkarniA, RustadC et al. Vestibular schwannomas occur in schwannomatosis and should not be consideredan exclusion criteria for clinical diagnosis. Am JMed Genet A. 2012; 158A: 215-218.
[7] Hulsebos TJM, Kenter S, Siebers-Renelt U, Hans V, Wesseling P, Flucke U. SMARCB1 involvement in the development of leiomyoma in a patient with schwannomatosis. Am J Surg Pathol. 2014; 38 (3): 421-425.
[8] Bazzi C, SestiniR, ProvenzanoA et al. Shwannomatosis associatd with multiple meningiomas due t a familial SMARCB1 mutation. Neurogenetics. 2001; 11: 73-80.
[9] Dane B, Danec, Basaran S etol. Vaginal schwannoma in a case with uterine Myoma. Ann Diagn Pathol. 2010; 14: 137-139.
[10] Gorlin RJ, Koutlas IG. Multiple Schwannomas, multiple nevi and multiple vaginal leiomyomas: a new dominant syndrome. Am J Med. Genet 1998; 78: 76-81.
[11] McClenethanJH, BloomRJ. Peripheral tumors of the intercostal nerves. AnnThoracSurg. 2004; 78: 713-714.
[12] AthanassiadiK, KalavrouziotisG, RondogianniD, LoutsidisA, HatzimichalisA, Bellenis I. Primary chest wall tumors: early and long term results of surgical treatment. Eur J cardiothorac surg. 2001; 19: 589-593.
[13] Sebanathan S, Salama FD, Morgan WE, Harvey JA. Primary chest wall tumors. Ann thorac surg 2004; 78: 713-714.
[14] Bousamra M. Neurogenic tumors of the mediastinumin: Pearson FG, Cooper JL, DeslauriersJ, Ginsberg RJ, Heibert CA, Patterson GA, Urshel HC, editors. Thoracic surgery. 2nd ed, Philadelphia, PA: Churchill living-stone; 2002: 1732-1738.
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  • APA Style

    Kawkabani Nadine, Darwish Rula, Boustros Omar, Bejjani Simon, El Achkar Nancy, et al. (2018). A Case Report: A Rare Case of Multiple Chest Wall Schwannomas Associated with Uterine Leiomyoma and Infertility. International Journal of Cardiovascular and Thoracic Surgery, 4(1), 1-4. https://doi.org/10.11648/j.ijcts.20180401.11

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    ACS Style

    Kawkabani Nadine; Darwish Rula; Boustros Omar; Bejjani Simon; El Achkar Nancy, et al. A Case Report: A Rare Case of Multiple Chest Wall Schwannomas Associated with Uterine Leiomyoma and Infertility. Int. J. Cardiovasc. Thorac. Surg. 2018, 4(1), 1-4. doi: 10.11648/j.ijcts.20180401.11

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    AMA Style

    Kawkabani Nadine, Darwish Rula, Boustros Omar, Bejjani Simon, El Achkar Nancy, et al. A Case Report: A Rare Case of Multiple Chest Wall Schwannomas Associated with Uterine Leiomyoma and Infertility. Int J Cardiovasc Thorac Surg. 2018;4(1):1-4. doi: 10.11648/j.ijcts.20180401.11

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  • @article{10.11648/j.ijcts.20180401.11,
      author = {Kawkabani Nadine and Darwish Rula and Boustros Omar and Bejjani Simon and El Achkar Nancy and Abou Khalil Bassam},
      title = {A Case Report: A Rare Case of Multiple Chest Wall Schwannomas Associated with Uterine Leiomyoma and Infertility},
      journal = {International Journal of Cardiovascular and Thoracic Surgery},
      volume = {4},
      number = {1},
      pages = {1-4},
      doi = {10.11648/j.ijcts.20180401.11},
      url = {https://doi.org/10.11648/j.ijcts.20180401.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcts.20180401.11},
      abstract = {Schwannomatosis is a tumor predisposition syndrome characterized by development of multiple schwannomas that are slow-growing encapsulating neurogenic tumors. It is a recurrent condition and patients lack usually the classic cutaneous stigmata, vestibular schwannomas or brain parenchyma tumors. Many papers have incriminated the mutation of the SMARCB1 gene in this disease which has been also observed in cases of uterine leiomyoma. This report describes a rare case of multiple chest wall schwannomas associated to a uterine fibroma. A genetic study done did not show any SMARCB1 gene mutation. Subsequently a further genetic counselling and a whole exome sequencing were recommended especially that the patient underwent many trials of failed IVF and two miscarriages despite resection of all the tumors.},
     year = {2018}
    }
    

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    AB  - Schwannomatosis is a tumor predisposition syndrome characterized by development of multiple schwannomas that are slow-growing encapsulating neurogenic tumors. It is a recurrent condition and patients lack usually the classic cutaneous stigmata, vestibular schwannomas or brain parenchyma tumors. Many papers have incriminated the mutation of the SMARCB1 gene in this disease which has been also observed in cases of uterine leiomyoma. This report describes a rare case of multiple chest wall schwannomas associated to a uterine fibroma. A genetic study done did not show any SMARCB1 gene mutation. Subsequently a further genetic counselling and a whole exome sequencing were recommended especially that the patient underwent many trials of failed IVF and two miscarriages despite resection of all the tumors.
    VL  - 4
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Author Information
  • Cardiac Surgery Anesthesia Department, Saint Georges Hospital University Medical Center, Beirut, Lebanon

  • Cardiac Surgery Anesthesia Department, Saint Georges Hospital University Medical Center, Beirut, Lebanon

  • Cardio-Thoracic Surgery Department, Saint Georges Hospital University Medical Center-University of Balamand, Beirut, Lebanon

  • Cardio-Thoracic Surgery Department, Saint Georges Hospital University Medical Center-University of Balamand, Beirut, Lebanon

  • General Anesthesia Department, Saint Georges Hospital University Medical Center-University of Balamand, Beirut, Lebanon

  • Cardio-Thoracic Surgery Department, Saint Georges Hospital University Medical Center, Beirut, Lebanon

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