Korean J Gastroenterol.  2014 Nov;64(5):307-310. 10.4166/kjg.2014.64.5.307.

Massive Upper Gastrointestinal Bleeding from Multiple Gastrointestinal Stromal Tumor in a Neurofibromatosis Patient

• Kim SB
• Chung WC

• KMID: 2164436
• DOI: http://doi.org/10.4166/kjg.2014.64.5.307

Abstract

No abstract available.

MeSH Terms

Endoscopy, Digestive System
Gastrointestinal Hemorrhage/*etiology
Gastrointestinal Stromal Tumors/complications/*diagnosis/radionuclide imaging
Humans
Jejunum/pathology
Male
Middle Aged
Neurofibromatosis 1/complications/*diagnosis/pathology
Proto-Oncogene Proteins c-kit/metabolism
Tomography, X-Ray Computed
Proto-Oncogene Proteins c-kit

Figure

• 

  Fig. 1. Initial esophagogastroduod-enoscopy. Mass-like lesions with overlying normal mucosa and central ulceration are seen in the 2nd and 3rd portion of the duodenum.

• 

  Fig. 2. Abdominal computed tomography. Two well-enhancing lesions (arrows) are observed in the third portion of the duodenum.

• 

  Fig. 3. Gastrointestinal bleeding scan with Tc-99m labeled red blood cell (RBC). An area of accumulation of Tc-99m RBC is noted at left upper abdomen with increased amount of activity and distal migration, suggesting bleeding from upper jejunum. ANT, anterior.

• 

  Fig. 4. Histologic examination of the biopsy specimen. (A) Spindle shaped cells arranged in fascicular pattern are seen (H&E, ×100). (B) Tumor cells are positive for CD 117 on immunohistochemical staining (×100).

Reference

References

1. Riccardi VM. Neurofibromatosis: clinical heterogeneity. Curr Probl Cancer. 1982; 7:1–34.

2. Relles D, Baek J, Witkiewicz A, Yeo CJ. Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases. J Gastrointest Surg. 2010; 14:1052–1061.

3. North K. Neurofibromatosis type 1. Am J Med Genet. 2000; 97:119–127.

4. Basile U, Cavallaro G, Polistena A, et al. Gastrointestinal and retroperitoneal manifestations of type 1 neurofibromatosis. J Gastrointest Surg. 2010; 14:186–194.

5. Kim ET, Namgung H, Shin HD, et al. Oncologic manifestations of neurofibromatosis type 1 in Korea. J Korean Surg Soc. 2012; 82:205–210.

6. Gottfried ON, Viskochil DH, Couldwell WT. Neurofibromatosis Type 1 and tumorigenesis: molecular mechanisms and therapeutic implications. Neurosurg Focus. 2010; 28:E8.

7. Andersson J, Sihto H, Meis-Kindblom JM, Joensuu H, Nupponen N, Kindblom LG. NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic, and genotypic characteristics. Am J Surg Pathol. 2005; 29:1170–1176.

8. Miettinen M, Lasota J. Gastrointestinal stromal tumors-defi-nition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001; 438:1–12.

9. Miettinen M, Fetsch JF, Sobin LH, Lasota J. Gastrointestinal stromal tumors in patients with neurofibromatosis 1: a clinicopathologic and molecular genetic study of 45 cases. Am J Surg Pathol. 2006; 30:90–96.

10. Choi W, Hong SD, Kim HN, et al. Two cases of neuroendocrine carcinoma and GIST in a patient with neurofibromatosis type 1. Korean J Med. 2011; 81:786–791.

11. Seo SO, Oh HJ, Kim KH, et al. A case of duodenal GIST accompanied with neurofibromatosis-1, presenting with gastrointestinal bleeding. Korean J Gastrointest Endosc. 2007; 35:254–257.

12. Han SH, Park SH, Cho GH, et al. Malignant gastrointestinal stromal tumor in a patient with neurofibromatosis type 1. Korean J Intern Med. 2007; 22:21–23.