Abstract

Situs inversus is a rare congenital condition in which the major visceral organs are reversed from their normal positions. Usually, it involves complete transposition (right to left reversal) of all of the abdominal organs and only a small percentage is associated with GIST which is the rare mesenchymal tumors of the alimentary tract. Nowadays GISTs represents 0.1% to 3% of all gastrointestinal malignancies, making it a diagnostic challenge. Lesions are frequently located in stomach and proximal small intestine but rarely elsewhere in the abdomen. They are believed to result from mutations of proto-oncogenes c-Kit or platelet-derived growth factor receptor alpha polypeptide, this increase tyrosine kinase receptor activity, leading to uncontrolled proliferation of stem cells that differentiate into cells of Cajal. They can occur at any age but predominantly in middle-aged people and in elderly. We report a very rare case of stomach GIST in Situs inversus totalis. A 56-yearold male presented to our hospital complaining of pain in the epigastrium with melena, findings suggested a gastric GIST. Cytologic and immunohistochemistry analysis confirm diagnosis of GISTs. The reverse location of the internal organs caused no significant difficulties for surgical treatment.

Highlights

  • The most common is Gastrointestinal Stromal Tumors Group (GIST), which arise from mesenchymal stem cells programmed to differentiate into interstitial cells of Cajal in the myenteric plexus [1]

  • When heart is located on the right side of rib cage, this condition is called the transposition of internal organs with dextrocardia and situs inversus totalis

  • Interstitial cells of Cajal form a net in a muscular wall of gastrointestinal tract and regulate its autonomic peristaltic activity, in other words they appear to be pacemaker cells providing the connection between smooth muscle cells and nerve endings

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Summary

Background

When heart is located on the right side of rib cage, this condition is called the transposition of internal organs with dextrocardia and situs inversus totalis. The body of the stomach had a dumbbell tumor of a size of 9 × 14 cm with smooth contours, antral part of the gastric antrum had no significant. Findings were conclusive of morpho-immunohistochemical pattern corresponding with GIST tumors of low degree of malignancy As it was a local form of GIST of the stomach without nodal metastases and hematogenous metastases to the liver, radical surgery was done and there were no complications after surgery. After subtotal distal gastric resection by Billroth II, was done in in 2012 (28 months after operation), x-ray of the stomach stump, CT of the chest and abdomen (Figures 5-11) and other data supported no relapse or progression.

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