|Year : 2014 | Volume
| Issue : 1 | Page : 57-59
Synchronous GIST of jejunum and neuroendocrine tumor of duodenum: A rare case report
Karpagam Janardhan1, Vijaya S Kumar2, Pradeep L Kumar3
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 Publication||14-Jul-2014|
Pradeep L Kumar
Department of Pathology, Karuna Medical College, Vilayodi, Chittur, Palakkad - 678 534, Kerala
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
Gastrointestinal stromal tumor (GIST) is the most common nonepithelial tumor of gastrointestinal tract.  GISTs are the most common mesenchymal tumors of gastrointestinal tract, although it comprises only 0.1-3% of all gastrointestinal neoplasms. 
The synchronous occurrence of GIST and other primary gastrointestinal malignancies is uncommon. , More than 60% of the GIST occurs in stomach  and 37% in jejunum.  The percentage of clinically malignant cases is higher in GIST of small intestine than in stomach. 
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%). , 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|| |
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|| |
The incidence of GIST is very low that is two in 100,000; while jejunal GIST is very rare.  GIST accounts for 0.1-3% of all gastrointestinal tumors.  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.  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. 
GIST is a major or minor component in certain rare syndromes and several inherited syndromes like multiple endocrine neoplasia.  The main differential diagnosis for GIST are leiomyoma and schwannomas.  It is very crucial to differentiate schwannoma and leiomyoma from GIST as these are biologically benign with excellent prognosis. 
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. 
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|| |
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.
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[Figure 1], [Figure 2], [Figure 3]