Boyer et al[15]2001 |
Abdominal pain |
2 |
Both of two cases were in the head |
A 5.0 cm solid mass with central necrosis; positive for CD117 and CD34; negative for S100 |
Biopsy of liver lesion and partial duodenopancreatectomy; |
NA |
Neto et al[6] 2004 |
Epigastric pain, bloating, weight loss |
1 |
Body |
A 20.0 cm solid cystic mass with necrotic foci; mitotic count (120/50 HPF); positive for CD117 and CD34; negative for cytokeratins 7 and 20, desmin and synaptophysin |
Distal pancreatectomy and splenectomy; treated with imatinib mesylate |
Recurrence with peritoneal and retroperitoneal nodal disease 1 mo after surgery |
Yamaura et al[7]2004 |
Incidental finding |
1 |
Tail |
A 14.0 cm solid mass with cystic degeneration; few mitoses; positive for CD34 and vimentin; negative for SMA and S100 |
Distal pancreatectomy splenectomy, and partial gastrectomy |
No evidence of disease recurrence at 30 mo |
Krska et al[8] 2005 |
Abdominal pain |
1 |
Body and head |
A 17.0 cm mass; mitotic count: 1/50 HPF; positive for CD34 and vimentin; negative for S100, chromogranin, actin, and CD117 |
Partial pancreatectomy |
No evidence of disease recurrence at 30 mo |
Pauser et al[11]2005 |
Incidental finding/abdominal discomfort |
2 |
Tail and body |
A 3.0 cm solid mass with positive for CD117 and CD34, cell negative for SMA;A 2.0 cm solid mass with positive for CD117 and CD34, cell negative for SMA |
Distal pancreatectomy splenectomy, and partial gastrectomy |
No evidence of disease at 24 and 48 mo |
Daum et al[9]2005 |
Incidental finding |
1 |
Head |
A 10.0 cm mass with central hemorrhage mitotic count: 2/50 HPF; negative for CD117, vimentin, actin, S100, CD34, desmin, and cytokeratins |
Whipple procedure; imatinib |
No evidence of disease recurrence at 6 mo |
Showalter et al[10] 2008 |
Incidental finding on workup for back pain |
1 |
Tail |
A 7.0 cm solid mass; mitotic count: 3/50 HPF; positive for CD117; negative for S100 protein and SMA |
Laparoscopic distal pancreatectomy and splenectomy |
No evidence of disease recurrence at 27 mo |
Yan et al[13]2008 |
Nausea and vomiting |
1 |
Uncinate process |
A 2.4 cm pancreatic mass with spindle cells and mild atypia; positive for CD117; negative for desmin |
NA |
NA |
Harindhanavudhi et al[14]2009 |
Fatigue and weakness |
1 |
Body |
A 16.0 cm × 11.0 cm solid cystic mass; positive for CD117, CD34 |
Exploratory laparotomy with cystojejunostomy and biopsy of cyst wall |
NA |
Trabelsi et al[12] 2009 |
Abdominal pain |
1 |
Head |
A 10.5 cm × 8.0 cm × 3.0 cm mass with spindle and epithelioid cells Mitotic: 6/50 HPF. Positive for CD117 (c-Kit) and CD34, negative for SMA, S100, synaptophysin and cytokeratins |
Hemipancreaticoduodenectomy with antrectomy and partial colectomy |
No evidence of disease recurrence at 10 mo |
Vij et al[5] 2011 |
Weakness, postprandial |
1 |
Head |
A 6.5 cm × 6.0 cm solid mass with pleomorphic Spindle cells; mitosis: 12-15/50 HPF; positive for CD117; negative for CD34, SMA, desmin, S100 and cytokeratins |
Whipple procedure; Imatinib on protocol |
Developed liver metastasis 24 mo after surgery |
Rubin et al[16]2011 |
fullness and painabdominal pain |
1 |
Body and tail |
A 25.0 cm × 30.0 cm solid mass positive for CD117, CD34 and VIM; negative for S100, NF, DES, SMA, and CK |
Distal pancreatectomy splenectomy |
NA |
Present case 1 |
Incidental finding |
1 |
Tail |
A 6.0 cm × 8.0 cm solid mass with spindle cells mitotic: < 5/50 HPF; positive for CD117, CD34, and s-100 protein, negative for DOG-1, Desmin, NF, synaptophysin, Chromogranin A and cytokeratin |
Distal pancreatectomy splenectomy |
No evidence of disease recurrence at 36 mo |
Present case 2 |
Incidental finding |
1 |
Head |
A 4.0 cm × 5.0 cm × 6.0 cm solid mass with spindle cells mitosis: > 5/50 HPF. Positive for CD117, S100 protein and DOG-1, negative for SMA, synaptophysin, and cytokeratin |
Pancreatic head tumor resection |
Developed liver metastasis 12 mo after surgery progression-free survival for 36 mo after two times of TACE and oral imatinib |