Pancreatic extragastrointestinal stromal tumor: A case report and comprehensive literature review

Table 2 Morphological characteristics, treatments, and outcomes of 30 patients with pancreatic extragastrointestinal stromal tumor identified from literature published between January 2004 and May 2014

Ref.	Cell type	Mitotic count (/50 HPF)	CD117	CD34	Surgical procedures	Recurrence (after surgery)	Outcome (follow-up)	Medical treatment
Tian et al[4]	Spindle	< 5 (intermediate risk)	(+)	(+)	Distal pancreatectomy + splenectomy	No	Alive (36 mo)	No
	Spindle	> 5 (high risk)	(+)	NS	Tumor resection	Yes (liver, 12 mo)	Alive (36 mo)	Gleevec + TACE
Paklina et al[11]	Spindle	1-2 (intermediate risk)	(+)	NS	NS	NS	NS	NS
Serin et al[1]	NS	NS (high risk)	(+)	NS	Distal pancreatectomy + splenectomy	No	Alive (21 mo)	No
Soufi et al[16]	Spindle	< 5 (intermediate risk)	(+)	(+)	Whipple + segmental colectomy	No	Alive (24 mo)	Gleevec
Wegge et al[2]	Spindle	6 (intermediate risk)	(+)	(+)	Whipple	No	Alive (5 mo)	Gleevec
Babu et al[13]	Spindle	6-8 (high risk)	(+)	(+)	Pancreatic head resection	No	Alive (11 mo)	No
Kim et al[3]	Spindle	7 (high risk)	(+)	(+)	Distal pancreatectomy + splenectomy	No	Alive (4 mo)	Gleevec
Čečka et al[9]	Spindle	5 (high risk)	(+)	(+)	Distal pancreatectomy + splenectomy	No	Alive (66 mo)	No
Vij et al[14]	Spindle	12-15 (high risk)	(+)	(-)	Whipple	Yes (liver, 24 mo)a	Alive (48 mo)	Gleevec
Rao et al[7]	Spindleb	8-10 (high risk)	(+)	(+)	Whipple	Yes (liver, 24 mo)	Alive (30 mo)	Gleevec
Yang et al[15]	Spindle	> 30/10 HPF (high risk)	(+)	(+)	Distal pancreatectomy + splenectomy	Yes (intraperitoneal, 24 mo)c	Alive (41 mo)	Gleevec
Barros et al[12]	NS	< 5	(+)	(+)	No	NS	Death (8 mo)	No
	NS	< 5 (intermediate risk)	(+)	(+)	Laparotomy + biopsy	Surgery not performed	Alive (12 mo)	Gleevec
Joshi et al[17]	Spindle	NS	(+)	(+)	None performedd	Surgery not performed	Death (5 d)	No
Crisan et al[18]	Spindle	(high risk)	(+)	(+)	Distal pancreatectomy + splenectomy + partial colectomy + duodenojejunal resection	NS	Alive (3 mo)	NS
Saif et al[19]	Spindlee	48 (high risk)	(+)	(-)	Whipple, pylorus preserving	Yes (liver, 9 mo)	Alive (NS)	Gleevec
Padhi et al[8]	Spindle	6-8 (high risk)	(+)	(+)	Distal pancreatectomy + splenectomy + left hemicolectomy	No	Alive (10 mo)	No
Harindhanavudhi et al[20]	Spindle	< 5 (high risk)	(+)	(+)	Cystojejunostomyf	NS	Alive (NS)	Gleevec
Trabelsi et al[21]	Spindle	6 (high risk)	(+)	(+)	Whipple + partial colectomy	No	Alive (10 mo)	No
Goh et al[10]	Spindle	> 10 (high risk)	(+)	NS	Whipple	No	Alive (58 mo)	NS
Showalter et al[23]	NA	3 (intermediate risk)	(+)	(-)	Distal pancreatectomy + splenectomy - laparoscopic	No	Alive (27 mo)	NS
Yan et al[24]	Spindleg	3 (low risk)	(+)	NS	NS	NS	NS	NS
Ganesh et al[25]	Spindleh	NS	(+)	(+)	No (inoperable)	Surgery no performed	Alive (30 mo)	Gleevec
Daum et al[27]	Spindle	2 (intermediate risk)	(+)	(-)	Whipple	No	Alive (6 mo)	Gleevec
Krska et al[28]	Spindlei	1 (high risk)	(-)	(+)	Partial pancreatectomy	No	Alive (30 mo)	NS
Pauser et al[29]	Spindle	NS	(+)	(+)	Resection	No	Alive (24 mo)	NS
	Spindle	NS	(+)	(+)	Resection	No	Alive (48 mo)	NS
Neto et al[30]	Mixed	120 (high risk)	(+)	(+)	Distal pancreatectomy	Yes (peritoneum)	Alive (NS)	Gleevec
Yamaura et al[31]	Spindle	Few (high risk)	(+)	(+)	Distal pancreatectomy + splenectomy + partial gastric resection	NS	Alive (30 mo)	NS

^aLiver metastasis at postoperative month 24. Metastasectomy performed. Two years followed without recurrence;

^bDiagnosed using USG-guided fine needle aspiration (FNA);

^cIntraperitoneal recurrence at postoperative month 24. Resection performed. Imatinib treatment both before and after resection. Following second resection, followed-up without recurrence;

^dCT-guided liver biopsy diagnosed metastatic EGIST. Clinical status deteriorated prior to surgery and died five days following diagnosis;

^eDiagnosed with Endo-USG (EUS)-guided FNA. Liver lesion diagnosed with CT and PET at postoperative month 9. Biopsy diagnosis was EGIST. Gleevec treatment dose increased to 800 mg. Due to resistance to treatment, was switched to sunitinib;

^fPancreatic mass diagnosed four years ago, patient refused surgical treatment. CT revealed 10-cm enlargement in four years. Diagnosis was made with EUS-guided FNA. Explorative laparotomy revealed pancreatic hemorrhagic cyst; cystojejunostomy performed to obtain an incisional biopsy sample diagnosed high-risk GIST. Patient refused definitive surgical treatment;

^gDiagnosis made with EUS-guided FNA;

^hDiagnosed using USG-guided FNA. Further surgical treatment aborted as the patient was inoperable, and Gleevec treatment was initiated. Clinical follow-up period of 30 mo revealed significant tumor reduction;

ⁱUSG-guided biopsy could not provide diagnosis. CT: Computed tomography; USG: Ultrasonography; EGIST: Extragastrointestinal stromal tumor; PET: Positron emission tomography; TACE: Transcatheter arterial chemoembolization; NA: Not-available; NS: Not-stated.