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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 4  |  Issue : 1  |  Page : 57-59

Synchronous GIST of jejunum and neuroendocrine tumor of duodenum: A rare case report


1 Consultant Pathologist, HistoLab, Coimbatore, Tamil Nadu, India
2 Consultant Gastroenterologist, Liver and Gastro Care, Coimbatore, Tamil Nadu, India
3 Department of Pathology, Karuna Medical College, Palakkad, Kerala, India

Date of Web Publication14-Jul-2014

Correspondence Address:
Pradeep L Kumar
Department of Pathology, Karuna Medical College, Vilayodi, Chittur, Palakkad - 678 534, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.136720

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  Abstract 

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of gastrointestinal tract, although it comprises only 0.1-3% of all gastrointestinal neoplasms. The majority of the neuroendocrine tumors of small intestine occur in adults and most of them are located in ileum followed by jejunum and duodenum. We present a case of synchronous GIST of jejunum and neuroendocrine tumor of duodenum in an elderly adult male patient without any associated syndrome. Although a Synchronous occurrence of gastrointestinal tumors without any coexisting syndromes is rare, a complete workup should be done to rule it out in any gastrointestinal tumors. To the best of our knowledge this is the first case to be reported in literature.

Keywords: GIST, neuroendocrine tumour, synchronous


How to cite this article:
Janardhan K, Kumar VS, Kumar PL. Synchronous GIST of jejunum and neuroendocrine tumor of duodenum: A rare case report. Arch Int Surg 2014;4:57-9

How to cite this URL:
Janardhan K, Kumar VS, Kumar PL. Synchronous GIST of jejunum and neuroendocrine tumor of duodenum: A rare case report. Arch Int Surg [serial online] 2014 [cited 2022 Jul 5];4:57-9. Available from: https://www.archintsurg.org/text.asp?2014/4/1/57/136720


  Introduction Top


Gastrointestinal stromal tumor (GIST) is the most common nonepithelial tumor of gastrointestinal tract. [1] GISTs are the most common mesenchymal tumors of gastrointestinal tract, although it comprises only 0.1-3% of all gastrointestinal neoplasms. [2]

The synchronous occurrence of GIST and other primary gastrointestinal malignancies is uncommon. [3],[4] More than 60% of the GIST occurs in stomach [5] and 37% in jejunum. [6] The percentage of clinically malignant cases is higher in GIST of small intestine than in stomach. [7]

The majority of the neuroendocrine tumors of small intestine (29%) occurs in adults and most of them are located in ileum (20%), followed by jejunum (3%) and duodenum (3%). [8],[9] We present a case of synchronous GIST of jejunum and neuroendocrine tumor of duodenum not associated with any syndrome. To the best of our knowledge this is the first case to be reported in literature.


  Case Report Top


A 73-year-old male patient presented to surgery outpatient department with dyspepsia, dull aching epigastric pain, vomiting, and with 6 kg weight loss since 3 months. There was no history of fever or bleeding per rectum. Patient is a known hypertensive on medication and without any other associated illness. General physical examination findings were within normal limits. Abdominal examination showed vague fullness in the epigastric region. No definite mass was palpable clinically. Cardiovascular, respiratory, and central nervous systemic examination findings were within normal limits.

On laboratory investigations, no significant findings were seen. Upper gastrointestinal endoscopy showed small nodule of 3 mm in the first part of duodenum. Computed tomography (CT) scan of abdomen showed neoplastic lesion measuring 14 × 9 cm in jejunum [Figure 1]a. A clinical diagnosis of lymphoma and a differential of GIST were made. Surgical resection of the mass with segment of jejunum was done with single layer end-to-end jejunojejunal anastomosis and endoscopic excision biopsy of duodenal lesion was done.
Figure 1: Photograph showing (a) computed tomography (CT) image and (b) gross appearance of jejunal gastrointestinal stromal tumor (GIST)

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Macroscopic examination of resected specimen showed a conglomerate mass of small intestine measuring 24 cm in length, comprising of mass measuring 14 10 cm in the antimesenteric border [Figure 1]b. On cross-section, extensive cystic and fleshy areas were seen. Multiple metastatic tumor nodules were seen in the mesentery measuring 0.5-1 cm, which on histology showed similar picture as in jejunum. No lymph nodes were identified. Microscopic features were of high grade GIST with mitotic count of >8/50 HPF [Figure 2]a. Immunohistochemically, sections were positive for CD 117 (c-kit) confirming the diagnosis [Figure 2]b.
Figure 2: Photomicrography showing (a) jejunal GIST (hematoxylin and eosin (H and E), 100) and (b) CD 117 positivity

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Biopsy of the duodenal nodule showed features of low grade neuroendocrine tumor with synaptophysin positivity on immunohistochemistry supporting the diagnosis [Figure 3]a and b. Cytogenetic study for 17q11.2 mutation was done to rule out neurofibromatosis I, which was negative. The diagnosis of synchronous high-grade, high-risk GIST of jejunum and low-grade neuroendocrine tumor of duodenum was given.
Figure 3: Photomicrography showing (a) duodenal neuroendocrine tumor (H and E, ×100) and (b) synaptophysin positivity

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Postoperative chemotherapy with imatinib 40 mg once a day for 6 months was given and followed-up. Patient had no recurrence for 6 months.


  Discussion Top


The incidence of GIST is very low that is two in 100,000; while jejunal GIST is very rare. [10] GIST accounts for 0.1-3% of all gastrointestinal tumors. [11] As per the literature, jejunum is the rarest site for GIST. Patients with GIST most commonly present with bleeding from mucosal ulceration and abdominal pain. [12] GIST are not homogenous group of neoplasms; however, immunohistochemically some show differentiation towards nerve, histiocyte, smooth muscle, and few undifferentiated. It also shows strong site-dependent genetic heterogeneity. [13]

GIST is a major or minor component in certain rare syndromes and several inherited syndromes like multiple endocrine neoplasia. [13] The main differential diagnosis for GIST are leiomyoma and schwannomas. [14] It is very crucial to differentiate schwannoma and leiomyoma from GIST as these are biologically benign with excellent prognosis. [15]

In our case, GIST was of high grade and immunohistochemically positive for CD 117 (c-kit). Over 95% of GISTs have mutation in c-kit or platelet-derived growth factor receptor (PDGFR)-a gene. The treatment of c-kit positive GIST cells with imatinib results in withdrawal of growth factor support, leading to death of the tumor cell. [16]

In our case, patient was on postoperative chemotherapy with imatinib, with no recurrence.

Synchronous occurrence of jejunal GIST with neuroendocrine tumor of duodenum is very rare. Following an extensive review of the literature this is the first case to be reported to the best of our knowledge. The clinical and endoscopic features were not unusual. On microscopy, GIST of jejunum was of high grade and neuroendocrine tumor of duodenum was of low grade. There were no associated syndromes.

Although a Synchronous occurrence of gastrointestinal tumors without any coexisting syndromes is rare, a complete workup should be done to rule it out in any gastrointestinal tumors.


  Conclusion Top


Synchronous GIST of the jejunum and neuroendocrine tumors of the duodenum are very rare. They may also occur as a component of the rare and inherited syndrome like neurofibromatosis I.

 
  References Top

1.Ohtake S, Kobayashi N, Kato S, Kubota K, Endo I, Inayama Y, et al. Duodenal gastrointestinal stromal tumor resembling a pancreatic neuroendocrine tumor in a patient with neurofibromatosis type I (von Recklinghausen's disease): A case report. J Med Case Rep 2010;4:302.  Back to cited text no. 1
    
2.Kaur R, Bhalla S, Nundy S, Jain S. Synchronous gastric gastrointestinal stromal tumor(GIST) and other primary neoplasms of gastrointestinal tract: Report of two cases. Ann Gastroenterol 2013;26:1-4.  Back to cited text no. 2
    
3.Ferreira SS, Werutsky G, Toneto MG, Alves JM, Piantá CD, Breunig RC, et al. Synchronous gastrointestinal stromal tumours (GIST) and other primary cancers: Case series of a single institution experience. Int J Surg 2010;8:314-7.  Back to cited text no. 3
    
4.Nemes C, Rogojan L, Surdea-Blaga T, Seicean A, Dumitrascu DL, Ciuce C. Gastrointestinal stromal tumour (GIST) associated with synchronous colon adenocarcinoma - a case report. J Gastrointest Liver Dis 2012;21:101-3.  Back to cited text no. 4
    
5.Chen ZM, Wang HL. The Intestine, Appendix and anus. In: Humphrey PA, Dehner LP, editors. The Washington manual of surgical pathology, South Asian ed. New Delhi: Wolters Kluwer; 2009. p. 182-207.  Back to cited text no. 5
    
6.Wilson JM, Melvin DB, Gray GF, Thorbjarnarson B. Primary malignancies of small intestine. A report of 96 cases and review of literature. Ann Surg 1974;180:175-9.  Back to cited text no. 6
[PUBMED]    
7.Tworek JA, Appelman HD, Singleton TP, Greenson JK. Stromal tumours of jejunum and ileum. Mod Pathol 1997;10:200-9.  Back to cited text no. 7
    
8.Bordi C, D'Adda T, Assoni C, Canavese G, Brandi ML. Gastrointestinal endocrine tumours: Recent developments. Endocr Pathol 1998;9:99-116.  Back to cited text no. 8
    
9.Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumours. Cancer 1997;79:813-29.  Back to cited text no. 9
    
10.Kramer K, Siech M, Sträter J, Aschoff AJ, Henne-Bruns D. GI hemorrhage with fulminant shock induced by jejunal gastrointestinal stromal tumor (GIST) coincident with duodenal neuroendocrine carcinoma (NET) +neurofibromatosis (NF) - case report and review of the literature. Z Gastroenterol 2005;43:281-8.  Back to cited text no. 10
    
11.Miettinen M, Lasota J. Gastrointestinal stromal tumors definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-12.  Back to cited text no. 11
    
12.Suster S. Gastrointestinal stromal tumors. Semin Diagn Pathol 1996;13:297-313.  Back to cited text no. 12
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13.Perry CG, Young WF Jr, McWhinney SR, Bei T, Stergiopoulos S, Knudson RA, et al. Functioning paraganglioma and gastrointestinal stromal tumor of the jejunum in three women: Syndrome or coincidence. Am J Surg Pathol 2006;30:42-9.  Back to cited text no. 13
    
14.Levy AD, Quiles AM, Miettinen M, Sobin LH. Gastrointestinal schwannomas: CT features with clinicopathologic correlation. Am J Roentgenol 2005;184:797-802.  Back to cited text no. 14
    
15.Sarlomo-Rikala M, Miettinen M. Gastric schwannomas - a clinicopathological analysis of six cases. Histopathology 1995;27:355-60.  Back to cited text no. 15
    
16.Dhulla AK, Kaushal V, Dhankar R, Atri R, Singh H, Marwah N. The inside mystery of jejuna gastrointestinal stromal tumor: A rare case report and review of literature. Case Rep Oncol Med 2011;2011:985242.  Back to cited text no. 16
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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