Skip to main content

Advertisement

Log in

Small Bowel Obstruction in a Neurofibromatosis Patient—A Rare Presentation of Gastro-Intestinal Stromal Tumors (GISTs): Case Report and Literature Review

  • Case Report
  • Published:
Indian Journal of Surgery Aims and scope Submit manuscript

Abstract

Gastrointestinal stromal tumours (GISTs) are infrequently encountered, mesenchymal tumours of the gastrointestinal tract of varying malignant potential. Multiple (in hundreds) small intestinal GISTs have been reported to occur only very occasionally in patients with type 1 neurofibromatosis (NF1). They are mostly silent, often detected incidentally or when complicated by bleeding or other symptoms. Such a case of multiple jejunal GISTs causing small bowel obstruction in a patient with NF1 is described and relevant literatures are briefly discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

References

  1. Miettinen M, Fetsch JF, Sobin LH, Lasota J (2006) Gastrointestinal stromal tumors in patients with neurofibromatosis 1. Am J Surg Pathol 30(1):90–6

    Article  PubMed  Google Scholar 

  2. Andersson J, Sihto H, Meis-Kindblom JM, Joensuu H, Nupponen N, Kindblom LG (2005) NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic and genotypic characteristics. Am J Surg Pathol 29(9):1170–6

    Article  PubMed  Google Scholar 

  3. Fuller CE, Williams GT (1991) Gastrointestinal manifestations of type 1 neuro-fibromatosis(von Recklinghausen's disease). Histopathology 19:1–11

    Article  CAS  PubMed  Google Scholar 

  4. Crosby JA, Catton CN, Davis A, Courture J, O’ Sullivan B, Kandel R et al (2001) Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database. Ann Surg Oncol 8(1):50–9

    Article  CAS  PubMed  Google Scholar 

  5. Vasiliadis K, Kogopoulos E, Katsamakas M, Karamitosos E, Tsalikidis C, Pringos B et al (2008) Ileoileal intussusception induced by a gastrointestinal stromal tumor. World J Surg Oncol 6:133. doi:10.1186/1477-7819-6-133, Available at: http://www.wjso.com/content/6/1/133

    Article  PubMed Central  PubMed  Google Scholar 

  6. Zailani MH, Naqiyah I, Rohaizak M, Siti-Aishah MA (2007) Gastrointestinal stromal tumours(GIST) in patient with Neurofibromatosis: A case report. Int Med J. 6(1). Available at: http://www.eimjm.com/Vol6-No1/Vol6-No1-C4.htm

  7. Kim SW, Shin HC, Kim IY, Kang K-H, Cho H-D (2009) Multiple gastrointestinal stromal tumours presenting as a haemoperitoneum in a patient with Type 1 neurofibromatosis. Br J Radiol 82:e133–6. doi:10.1259/bjr/25491808

    Article  CAS  PubMed  Google Scholar 

  8. Li FP, Fletcher JA, Heirich MC, Garber JE, Sallan SE, Lewandrowski CC et al (2005) Familial gastrointestinal stromal tumour syndrome: phenotypic and molecular features in a kindred. J Clin Oncol 23(12):2735–43

    Article  CAS  PubMed  Google Scholar 

  9. Tsukuda K, Ikeda E, Takagi S, Miyake T, Muraoka T, Watanabe K et al (2007) Multiple gastrointestinal stromal tumors in neurofibromatosis type 1 treated with laparoscopic surgery. Acta Med Okayama 1:47–50

    Google Scholar 

  10. Karagulle E, Turk E, Yildirim E, Gokturk HS, Kiyici H, Moray G (2008) Multifocal intestinal stromal tumors with jejunal perforation and intra-abdominal abscess: report of a case. Turk J Gastroenterol 19(4):264–7

    PubMed  Google Scholar 

  11. Joo M, Lee HK, Kim H, Kim MK, Chi JG (2004) Multiple small intestinal stromal tumors with Neurofibromatosis-1. Yonsei Med J 45(3):564–7

    PubMed  Google Scholar 

Download references

Acknowledgements

We are thankful to Dr. Palat K. Menon, M.D., Ph.D., Medical Director Quest Diagnostic India Private Limited, for providing photomicrographs in academic interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Subhendu Bikas Saha.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Saha, S.B., Parmar, R. & Mandal, A. Small Bowel Obstruction in a Neurofibromatosis Patient—A Rare Presentation of Gastro-Intestinal Stromal Tumors (GISTs): Case Report and Literature Review. Indian J Surg 75 (Suppl 1), 415–417 (2013). https://doi.org/10.1007/s12262-012-0746-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12262-012-0746-7

Keywords

Navigation