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  Table of Contents  
LETTER TO THE EDITOR
Year : 2015  |  Volume : 52  |  Issue : 4  |  Page : 510-512
 

“Gastro-Esophageal junction GIST”


Department of Surgery, Jaslok Hospital and Research Centre, Peddar Road, Mumbai, Maharashtra, India

Date of Web Publication10-Mar-2016

Correspondence Address:
S A Bhange
Department of Surgery, Jaslok Hospital and Research Centre, Peddar Road, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.178390

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How to cite this article:
Bhange S A, Sathe S M, Gala A P, Bhansali M S. “Gastro-Esophageal junction GIST”. Indian J Cancer 2015;52:510-2

How to cite this URL:
Bhange S A, Sathe S M, Gala A P, Bhansali M S. “Gastro-Esophageal junction GIST”. Indian J Cancer [serial online] 2015 [cited 2019 Dec 13];52:510-2. Available from: http://www.indianjcancer.com/text.asp?2015/52/4/510/178390


Sir,

We present a case of gastro-intestinal stromal tumor (GIST) involving the esophago-gastric junction (EGJ). GISTs contribute 1-3% of all GI malignancies originating primarily from the interstitial cells of cajal or from their stem cell precursors.

[1] Most commonly, they arise in the stomach and small intestine, rarely in the appendix, gallbladder, pancreas, and the retroperitoneum, but esophago-gastric junction tumors are extremely rare.

These tumors are characterized by expression of the transmembrane receptor tyrosine kinase KIT, which is defined by the CD117 antigen and is the product of the kit proto-oncogene although some GISTs do not or only weakly express this marker as was seen in our case.[2]

The drug imatinib can block signaling via KIT and PDGFRA by binding to the adenosine triphosphate-binding pocket required for phosphorylation and activation of the receptor resulting in inhibition of tumor proliferation and increase in median survival rate.

Our patient was a 60-year-old female who presented with pain in upper abdomen and vomiting after meals since 4-5 months, but no dysphagia. OGD [Figure 1], [Figure 2], [Figure 3] with EUS [Figure 4],[Figure 5] revealed hiatus hernia at 34-38 cm with a subepithelial tumor approximately 5 × 3 cm arising from the muscularis propria, hypoechoic with hyperechoic specs, no capsular invasion, no surrounding lymph nodes. CECT abdomen [Figure 6], [Figure 7], [Figure 8] showed a polypoidal submucosal mass lesion in the anterior and posterior walls of the esophagus, about 6 cm in length, starting just below the left atrium, extending to the esophago-gastric junction and the proximal stomach wall. The overlying mucosa showed irregularity and fissuring. These features were suggestive of either GIST or leiomyoma.
Figure 1: Endoscopy showing Hiatal hernia from 34-38 cm with a subepithelial tumour at 34 cm

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Figure 2: Endoscopy showing Hiatal hernia from 34-38 cm with a subepithelial tumour at 34 cm

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Figure 3: Endoscopy showing Hiatal hernia from 34-38 cm with a subepithelial tumour at 34 cm

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Figure 4: EUS showing Subepithelial tumour arising from muscularis propria, predominantly hypoechoic with hyperechoic specs

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Figure 5: EUS showing Subepithelial tumour arising from muscularis propria, predominantly hypoechoic with hyperechoicspecs

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Figure 6: Oral Contrast CT showing a polypoidal submucosal mass arising from anterior and posterior walls of the esophagus, with few foci of calcification and with some fissuring and irregularity of the overlying mucosa

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Figure 7: Oral Contrast CT showing a polypoidal submucosal mass arising from anterior and posterior walls of the esophagus, with few foci of calcification and with some fissuring and irregularity of the overlying mucosa

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Figure 8: Oral Contrast CT showing a polypoidal submucosal mass arising from anterior and posterior walls of the esophagus, with few foci of calcification and with some fissuring and irregularity of the overlying mucosa

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The tumor was large and multicentric, and hiatus hernia made the left thoracoabdominal approach difficult, hence we proceeded to a total transhiatal esophagectomy. Recovery was uneventful, and patient was discharged on tenth postoperative day. She is currently on oral imatinib 400 mg daily and will follow-up with us three-six monthly.

Cut section of the specimen revealed multi-lobulated homogenous whitish tumor arising from the wall of the stomach, 6 × 6 × 2.5 cm in size, with foci of calcification. Three other identical nodules varying from 0.5-1 cm were arising from the muscularis of the stomach and esophagus. Microscopically, they revealed interlacing bundles of spindle cells showing bland nuclear features with no mitosis or necrosis and clear resection margins [Figure 9],[Figure 10]. All 12 nodes were negative for malignancy. Immunohistochemistry revealed: SMA +++[Figure 12], CD34+++ [Figure 11] with weakly positive CD117.
Figure 9: Hematoxylin and eosinophillic staining interlacing bundles of spindle cells with bland nuclear features. No mitosis or necrosis

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Figure 10: Hematoxylin and eosinophillic staining interlacing bundles of spindle cells with bland nuclear features. No mitosis or necrosis

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Figure 11: CD34 was highly positive for the tumor cells and also the vessels

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Figure 12: SMA was highly positive for the tumor cells and also the vessels

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In the available literature, GISTs of the esophagogastric junction are often classified among gastric or esophageal lesions, without specifying the precise anatomical site. Simultaneous involvement of the stomach and esophagus, as seen in our case, is very rare. The main hurdle to understanding and treating these tumors is their relative rarity and the subsequent shortage of literature. As per NCCN guidelines, surgery should be the initial stage of treatment for a localized and resectable disease. The goal should be complete resection with an intact pseudocapsule. Preoperative imatinib should not be considered if surgical morbidity cannot be improved by reducing the size of the tumor. Radical surgery with negative surgical margins and adjuvant therapy (in case of high mitotic rates) has shown to have increased survival rates, hence, at present, it is not advisable to treat these tumors with laparoscopic/open enucleation alone with adjuvant therapy.[3],[4]


  Acknowledgment Top


We thank the radiology and histopathology departments of Jaslok Hospital and Research Centre and Dr. Pankaj Dhawan (senior gastroenterologist) for their help.

 
  References Top

1.
Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): Gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998;152:1259-69.  Back to cited text no. 1
    
2.
Medeiros F, Corless CL, Duensing A, Hornick JL, Oliveira AM, Heinrich MC, et al. KIT-negative gastrointestinal stromal tumors: Proof of concept and therapeutic implications. Am J Surg Pathol 2004;28:889-94.  Back to cited text no. 2
    
3.
Coccolini F, Catena F, Ansaloni L, Lazzareschi D, Pinna AD. Esophagogastric junction gastrointestinal stromal tumor: Resection vs enucleation. World J Gastroenterol 2010;16:4374-6.  Back to cited text no. 3
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4.
Peparini N, Carbotta G, Chirletti P. Enucleation for gastrointestinal stromal tumors at the esophagogastric junction: Is this an adequate solution? World J Gastroenterol 2011;28;2159-60.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]



 

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