Acinar Cell Carcinoma of the Pancreas in Korea - Clinicopathologic Analysis of 27 Patients from Korean Literature and 2 Cases from Our Hospital -

Jae Hoon Lee; Kyeong Geun Lee; Hwon Kyum Park; Kwang Soo Lee

doi:10.4166/kjg.2010.55.4.245

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Lee, Lee, Park, and Lee: Acinar Cell Carcinoma of the Pancreas in Korea - Clinicopathologic Analysis of 27 Patients from Korean Literature and 2 Cases from Our Hospital -

Original Article

Korean J Gastroenterol 2010;55(4):245-251.

Published online: 21 February 2010

DOI: https://doi.org/10.4166/kjg.2010.55.4.245

Acinar Cell Carcinoma of the Pancreas in Korea - Clinicopathologic Analysis of 27 Patients from Korean Literature and 2 Cases from Our Hospital -

Jae Hoon Lee, M.D., Kyeong Geun Lee, M.D., Hwon Kyum Park, M.D., Kwang Soo Lee, M.D.

Department of Surgery, Hanyang University College of Medicine, Seoul, Korea

Correspondence to: Hwon Kyum Park, M.D. Department of Surgery, Hanyang University Guri Hospital, 249-1, Gyomun-dong, Guri 471-701, Korea Tel: +82-31-560-2295, Fax: +82-31-566-4409 E-mail: hkpark@hanyang.ac.kr

Received 19 October 2009 Accepted 1 February 2010

Abstract

Background/Aims

Acinar cell carcinoma (ACC) of the pancreas is a rare malignancy. ACC has been considered a cancer with poor prognosis due to frequent metastasis, a high recurrence rate, and low resectability. The aim of this study was to examine the clinical, radiologic and pathologic features of ACC in Korean patients, and surgical outcome was also investigated.

Methods

We reviewed the clinical records of two patients with ACC who had undergone operation in January 1996 and December 2005 at Hanyang University Medical Center. Through searching of medical journal from 1983 to 2009, 27 patients reported on literatures as Korean ACC patients were reviewed together. The clinical, pathohistologic, and radiologic features, treatment, and prognosis were investigated for all 29 patients.

Results

ACC was more common in male, and age at diagnosis ranged from 25 to 68 years (median 54). Symptoms were, mostly abdominal pain and mass. Liver was most common organ of metastasis at diagnosis and recurrence after operation. The mean tumor size was 7.0 cm, and most common location was tail. Of the 29 patients, 22 underwent surgical resection. Excluding 7 cases of not-reported survival, the median survival with operation was 22.4 months compared to 1.5 months with non-operation.

Conclusions

In Korea, the clinical features of ACC include young age, large size, tail location, and nonspecific tumor markers. Surgery should be actively performed in the treatment of ACC regardless of size.

Keywords: Acinar cell carcinoma, Pancreas

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Table 1.

Clinical Characteristics and Operation of Acinar Cell Carcinoma

Case No.^∗	S/A	Symptom	Location	Size (cm)	Amylse/ lipase (U/L)	Increased tumor marker	Meta	Operation
Surgical resection g group
1	F/60	Abdominal pain	Head	1	I/I (375/120)	None	None	PPPD
2	F/48	None	Head	1.2	I/I (120/112)	AFP (89.9 ng/mL)	None	PD
3	M/36	Epigastric pain	Head	2.2	N/N	None	None	PPPD
4	M/54	Jaundice	Head	3	N/N	N-R	None	PD
5	F/25	Jaundice	Head	3	N/N	None	None	PD
6	M/59	Epigastric pain, weight loss	Head	3	N/N	None	None	PD＋ wedge (S5) due to HCC
7	F/62	Jaundice	Head	3	N/N	CA19-9 (258.7 U/mL)	None	PPPD
8	M/60	Jaundice, weight loss	Head	8.2	N/I (170)	CEA (5.51 ng/mL)	None	PD
9	M/59	Indigestion	Body	8	N/N	N-R	None	SP
10	F/50	LUQ mass	Body, tail	12.5	N/I (1188)	None	None	DP＋ TG
11	M/54	LUQ pain	Body, tail	19	N/I (709)	None	None	DP＋ T-colon resection
12	M/39	Flank pain	Tail	3.5	N/I (976)	None	None	TP
13	M/38	Abdominal pain	Tail	5	N/	None	None	DP
14	M/36	Epigastric pain	Tail	5	N/N	None	None	DP
15	M/53	LUQ pain	Tail	5	N/I (2400)	None	None	DP
16	M/60	Epigastric pain	Tail	6	N/N	None	None	DP＋ TG
17	F/66	Hemoperitoneum	m Tail	6	N/N	None	Colon	DP
18	F/49	LUQ pain	Tail	9	N/N	N-R	Liver	DP
19	F/64	Epigastric pain, LUQ mass	Tail	9	N/N	N-R	None	DP＋ TG
20	M/60	Weight loss	Tail	11	N/	CA19-9 (62.4 U/mL)	None	DP
21	M/41	LUQ pain	Tail	12	N/N	None	None	DP
22	F/58	LUQ mass	Tail	18	N/I (245)	CEA (3.5 ng/mL)	Liver	DP
Non-surggical group
23	F/47	Epigastric pain	Head	2	N/N	None	Lung	Open biopsy
24	M/52	Epigastric pain, weight loss	Head	7	N/N	None	Liver	None
25	M/62	Epigastric pain, jaundice	Head	9	N/N	CA19-9 (350 U/mL)	Liver	None
26	M/41	Abdominal pain	Head, body	5	N/N	None	Liver, lung,	None
							bone
27	F/43	Epigastric pain	Body	6.2	N/N	None	Liver	Needle biopsy
28	M/57	Epigastric pain	Body	11	N/N	N-R	None	Needle biopsy
29	M/68	LUQ mass	Tail	10	N/	None	Liver	Open biopsy

S/A, sex/age; Meta, metastasis at presenting; LUQ, left upper quadrant; N/I, normal/increased; N-R, not reported; PD, pancreaticoduodenectomy (Whipple's operation); SP, subtotal pancreatectomy; DP, distal pancreatectomy; TP, total pancreatectomy; TG, total gastrectomy; PPPD, pylus-preserving pancreaticoduodenectomy.

^∗ Number of Case (reference number): 1-2 (our case), 3 (8), 4 (9), 5 (7,10), 6 (11), 7 (8), 8 (12), 9 (13), 10 (14), 11-12 (7,10), 13 (15), 14 (8), 15 (7,10), 16-17 (8), 18 (10), 19 (8), 20 (16), 21 (17), 22 (18), 23-26 (8), 27 (7,10), 28 (10), 29 (19).

Table 2.

CT Finding, Adjuvant Treatment and Follow-up

Case No.	CT findings		Recurrence (postoperative period months)	Chemotherapy/ radiotherapy (RT)	Survival
Case No.	Necrosis/ capsule	Tumor invasion	Recurrence (postoperative period months)	Chemotherapy/ radiotherapy (RT)	Survival
Surgical resection group
1	−/−	None	Liver (29 mo)	Gemcitabine, capecitabine#5	Alive, 63 mo
2	−/−	None	None	None	Alive, 70 mo
3	N-R	None	None	None	Alive, 4 mo
4	N-R	N-R	N-R	N-R	N-R
5	＋/＋	None	None	5-FU#12	Alive, ＞10 years
6	N-R	None	N-R	N-R	N-R
7	N-R	None	N-R	None	D, 22.4 mo
8	N-R	None	Liver (12 mo)	Gemcitabine, cisplatin, 5-FU	D (pneumonia), 30 mo
9	＋/＋	None	N-R	N-R	N-R
10	＋/＋	Omentum	Liver, bone (left. iliac, sternum) 15 mo	5-FU, cisplatin#6/RT (5460 rad)	D, 17 mo
11	＋/＋	Portal vein	Local (4 mo)	5-FU#2/RT (5,400 rad)	D (MI), 9 mo
12	−/−	None	None	5-FU#12	D, 43 mo (lung failure)
13	＋/＋	None	Pancreas (body, 30 mo)	Cisplatin#6	N-R
14	N-R	None	Liver (22 mo)	Xeloda, Gemtan#2, 5-FU, leucovorin, oxaliplatin #4	Alive, 31.5 mo
15	＋/＋	Spleen	N-R	5-FU#2	Follow-up loss
16	N-R	None	Liver (2 mo)	None	D, 4.7 mo
17	N-R	None	Liver, stomach (16 mo)	None	D, 19.4 mo
18	＋/＋	Spleen	N-R	N-R	N-R
19	N-R	None	N-R	None	D. 82.6 mo
20	−/−	T-colon mesentery	Pancreas (head, 4 mo)	None	D, 18 mo
21	＋/−	Spleen	Stomach (3 mo), liver (7 mo)	Gemcitabine #7, capecitabine #14	Alive, 21 mo
22	N-R	None	None	None	D (MI), 9 day
Non-surgical group
23	N-R	Omentum, paraaortic	N-R	None	Alive, 1.4 mo
24	N-R	Duodenum, SMA	No operation	None	D, 2.2 mo
25	N-R	Duodenum, paraaortic	No operation	None	D, 1.0 mo
26	N-R	Transverse colon	No operation	None	D, 1.5 mo
27	＋/＋	None	No operation	5-FU#12/RT (5,400 rad)	D, 19 mo
28	＋/＋	None	N-R	N-R	N-R
29	＋/＋	Peritoneal spread	No operation	N-R	N-R

#, number of chemotherapy cycle; N-R, not reported; D, death; MI, myocardial infarction; mo, months.

Table 3.

Immunohistochemistry of Acinar Cell Carcinoma

Case No.	Positive	Negative
1	α1-antitrypsin, lipase, CEA, cytokeratin, PAS, d-PAS, NSE (focal)	AFP, chromogranin, cytokeratin 7, cytokeratin 20
2	α1-antitrypsin, cytokeratin	Chromogranin, synaptophysin
8	α1-antitypsin
10	PAS	CEA, synaptophysin, chromogranin
21	PAS, d-PAS

PAS, periodic acid stain; d-PAS, diastase periodic acid-schiff; NSE: neuron specific enolase.

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