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55

A MALIGNANT GASTROINTESTINAL STROMAL TUMOR OF GALLBLADDER IMMUNOREACTIVE FOR PDGFRA AND NEGATIVE FOR CD 117 ANTIGEN (C-KIT)

Thursday, 25 November, 2010 - 08:20
Group 3 - 1

Εισαγωγή

GISTs of the gallbladder are extremely rare tumors. Only four malignant, two benign and one GIST-like tumor of the gall bladder have ever been described. The four malignant GISTs were all positive for CD 117 antigen (c-kit). We present for the first time a malignant gastrointestinal stromal tumor of the gallbladder, immunoreactive for platelet derived growth factor receptor alpha (PDGFRA) and negative for CD 117 antigen (c-KIT).

Full Text

Introduction

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GISTs of the gallbladder are extremely rare tumors. Only four malignant, two benign and one GIST-like tumor of the gall bladder have ever been described. The four malignant GISTs were all positive for CD 117 antigen (c-kit). We present for the first time a malignant gastrointestinal stromal tumor of the gallbladder, immunoreactive for platelet derived growth factor receptor alpha (PDGFRA) and negative for CD 117 antigen (c-KIT).
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Case Report

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A 72-yr-old woman was admitted to the First Department of Surgery, University of Athens with symptoms suggestive of obstructive jaundice: fever and chills, dark urine, intermittent right upper quadrant (RUQ) abdominal pain and jaundice. Clinical examination revealed mild tenderness in the RUQ only with no palpable masses.

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Ultrasound demonstrated a thickened gallbladder wall, bile stones and moderate common bile duct dilatation. Bile duct lithiasis was suspected and an ERCP
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was performed. This demonstrated an almost complete obstruction at the level of the cystic duct and common hepatic duct confluence. However the underlying pathology could not be identified and an exploratory laparotomy was decided. This revealed a large irregular hard mass at the site of the gallbladder. The mass was adherent to the duodenum involving the confluence of the cystic duct with the common hepatic duct and the retroduodenal part of the common bile duct. Cholecystectomy was performed along with wedge resection of the gallbladder bed. The common bile duct and common hepatic were excised en bloc with the specimen ensuring free surgical margins along the extrahepatic billiary tree. Lymph node dissection was performed of the hepatoduodenal ligament, the hepatic artery lymph nodes, and the retropancreatic lymph nodes. The enteric-billiary continuity was established through a retrocolic Roux-en-Y hepatico-jejunostomy. The postoperative course was uneventful and the patient was discharged ten days post operation.
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images/image1290024402284.png

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Tumor cells are diffusely immune-positive for PDGFRA (X200)
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Discussion

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There have been a small number of gallbladder GISTs described in the literature, of which only four were malignant and all were Kit positive. To our knowledge this presents the first ever recorded presentation of such a GIST. The significance of the Kit negative genotype has implications on the response to further management. The identification of specific cellular markers has led to the development of effective targeted agents, namely tyrosine kinase inhibitor (TKI) therapy (e.g. imatinib). These have had dramatic effects in prolonging progression free survival in advanced unresectable disease. Most Kit positive tumors are sensitive to imatinib however the majority (80%) of PDGFRA mutations are resistant to treatment.
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Conclusion

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Apart from this being a previously undocumented case it highlights the challenges in establishing the diagnosis, prognosis and most effective management for this unpredictable tumour.
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