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

Table 1 Demographic and clinical characteristics of 30 patients with pancreatic extragastrointestinal stromal tumors identified from literature published between January 2004 and May 2014

Ref.	Year	Country	Age (yr)	Sex	Clinical presentation	Examination	Radiologic tools	Tumor location	Tumor size (cm)
Tian et al[4]	2014	China	61	M	Incidental finding	Abdominal mass	CT	Tail	60 × 80
			60	M	Incidental finding	NS	CT	Head	60 × 50
Paklina et al[11]	2013	Russia	38	F	Abdominal discomfort	NS	CT	Head	90
Serin et al[1]	2013	Turkey	30	M	Abdominal distension	NS	US + CT	Tail	130
Soufi et al[16]	2013	Morocco	39	M	Weight loss + abd pain + constipation	Distension	CT + endoscopy	Head	90 × 70 × 50
Wegge et al[2]	2012	USA	55	M	Haematemesis + haematochezia	Non-specific	CT + MRCP + endoscopy	Head	46 × 45 × 44
Babu et al[13]	2012	China	55	F	Upper abdominal pain	Non-specific	CT + US	Head	50 × 40 × 30
Kim et al[3]	2012	Korea	55	M	Abdominal discomfort	Non-specific	CT + MR	Tail	130 × 90 × 85
Čečka et al[9]	2011	Czech	74	F	Abdominal mass	Palpable mass	US + CT	Tail	110 × 80 × 40
Vij et al[14]	2011	India	35	M	Weight loss + abdominal discomfort	Non-specific	US + CT	Head	80 × 60
Rao et al[7]	2011	India	40	M	Weight loss + abdominal pain + anemia	Non-specific	US + CT	Head + Body	65 × 60
Yang et al[15]	2011	China	55	M	Abdominal discomfort	Abdominal mass	CT + MR	Body + Tail	178 × 196
Barros et al[12]	2011	Brasil	63	F	Abdominal pain + ponderal loss	NS	NS	NS	NS
			81	F	Difficult gastric emptying + ponderal loss	NS	NS	NS	100
Joshi et al[17]	2010	USA	84	M	Weight loss + abdominal distension	Distension	CT	Entire pancreatic tissue	340 × 240 × 270
Crisan et al[18]	2010	Romania	61	M	Weight loss + fever + intense sweating	Diffuse tenderness	CT X	Tail + Body	140
Saif et al[19]	2010	USA	31	M	Weight loss + abdominal pain + anemia	NS	CT + MR + endoscopy	Head	56 × 51 × 42
Padhi et al[8]	2010	India	42	F	Weight loss + abdominal pain	Palpable mass	CT + MR	Body + Tail	350 × 300 × 250
Harindhanavudhi et al[20]	2009	USA	63	F	Fatigue + weakness+anemia	Non-specific	CT + EUS	Body	160 × 110
Trabelsi et al[21]	2009	Tunisia	52	F	Epigastric pain	Palpable mass	US + CT	Head	105 × 80 × 30
Goh et al[10]	2009	Singapore	58	M	Incidental finding	NS	NS	Head	90
Showalter et al[23]	2008	USA	72	F	Incidental finding	NA	MR	Tail	70
Yan et al[24]	2008	USA	47	M	Nausea + vomiting + (hepatitis B)	Splenomegaly	CT + EUS	Uncinate process	24 × 21
Ganesh et al[25]	2008	UK	76	F	Weight loss + abdominal pain	Diffuse tenderness	CT + endoscopy	Tail + body	NS
Daum et al[27]	2005	Czech	70	F	Incidental finding	Palpable mass	CT	Head	100 × 80 × 60
Krska et al[28]	2005	Czech	38	F	Abdominal pain + fatigue	Tenderness	CT + US + EUS + CT + endoscopy	Head + Body	170 × 120
Pauser et al[29]	2005	USA	51	M	Incidental finding	NS	US + CT + endoscopy	Tail	30
			54	F	Abdominal discomfort	NS	US	Body	20
Neto et al[30]	2004	Brasil	67	F	Weight loss + abd pain + gastric bloating	NS	NS	Body + Tail	200 × 190 × 120
Yamaura et al[31]	2004	Japan	54	F	Incidental finding	Palpable mass	US + CT + MR + angiography	Tail	140 × 120 × 80

US: Ultasonography; CT: Computed tomography; MR: Magnetic resonance; EUS: Endoscopic ultrasound; MRCP: Magnetic resonance cholangiopancreatography; X: Partial thrombosis detected in both portal vein and inferior vena cava at the level of left renal vein; NA: Not-available; NS: Not-stated; M: Male; F: Female; UK: United Kingdom; USA: United States of America.

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.