Ann Hepatobiliary Pancreat Surg.  2017 Nov;21(4):237-242. 10.14701/ahbps.2017.21.4.237.

Extra-gastrointestinal stromal tumor of the pancreas: report of a case

• Kwon HJ

• KMID: 2397806
• DOI: http://doi.org/10.14701/ahbps.2017.21.4.237

Abstract

Gastrointestinal tumors (GISTs) of the pancreas are extremely rare with limited individual case reports and small number of case series. Herein, we report a case of pancreatic extragastrointestinal stromal tumor (EGIST) along with literature review. A 64-year-old female patient was referred to us for treatment of an abdominal mass detected by ultrasonographic examination. The tumor was located in the periamullary region. Under a preoperative diagnosis of a duodenal GIST, we performed a pylorus preserving pancreatoduodenectomy for this lesion. Laboratory examination results were within normal ranges. On pathologic gross examination, the tumor measured at 7 cm in its greatest dimension almost entirely involved the pancreatic head. Its cut surface was rubbery and white. It was surrounded by a thin pseudocapsule and well demarcated. Histopathological examination of the specimen showed a cellular lesion with compressed pancreatic tissue at peripheral. Mitotic count was 5 per 50 high-power fields. Immunohistochemically, neoplastic cells were positive for antibodies against C-KIT (CD117), CD 34, and vimentin. However, smooth-muscle actin reactions with antibodies against S-100 or desmin were negative. Based on above findings, the tumor was finally diagnosed as GISTs originating from the pancreas. The patient has been followed up postoperatively for 72 months. There is no evidence of recurrence. Here we report this case of pancreatic EGIST presenting as a solid neoplasm along with literature review of cases previously described. Our review on pancreatic EGISTs is limited and insufficient to make a conclusion regarding its clinical features. Those manifested large masses tended to have an aggressive biological and clinical behavior. Thus, pancreatic EGISTs need to be carefully differentiated. Adequate surgical intervention is necessary for pancreatic EGISTs.

Keyword

Diagnosis; Gastrointestinal stromal tumor; Pancreas; Pancreatectomy; Pancreatic neoplasm

MeSH Terms

Actins
Antibodies
Desmin
Diagnosis
Female
Gastrointestinal Stromal Tumors
Head
Humans
Middle Aged
Pancreas*
Pancreatectomy
Pancreatic Neoplasms
Pancreaticoduodenectomy
Pylorus
Recurrence
Reference Values
Vimentin
Actins
Antibodies
Desmin
Vimentin

Figure

• 

  Fig. 1 Imaging study findings. (A and B) An abdominal computed tomography (CT) scan showing a well-enhanced mass measuring at 6.5 cm×6.5 cm with central low dense lesion appearing as necrosis (axial & coronal). (C) Positron emission tomography (PET) showing a hypermetabolic lesion measured at 7 cm (SUVmax 4.0) in the pancreatic head without evidence of abnormal hypermetabolic lesion, suggesting lymph node or distant metastasis. (D) Endoscopic ultrasonography (EUS) revealing a well-defined homogenous hypoechoic mass like lesion (EUS-guided biopsy was done) without posterior acoustic shadowing at the pancreatic head. The main pancreatic duct was mildly dilated.

• 

  Fig. 2 Pathologic study findings. (A) On pathologic gross examination, the tumor measured at 7 cm in its greatest dimension involving the pancreatic head. Its cut surface was rubbery and white. Surrounded by a thin pseudocapsule, it was well-demarcated, infiltrating the duodenal wall. (B) The tumor was composed of spindle cells (H&E, ×200). (C) Immunohistochemical examination revealing diffuse and weak CD 117 positivity. (D) Tumor cells showing positive immunoreactivity for CD 34.

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