Nationwide questionnaire survey on pediatric pancreatic tumors in Japan

Table 1 Characteristics of each disease

	SPN	Pancreatoblastoma	Pancreatic endocrine tumor	Non-epithelial tumor	Pancreatic cancer	Metastatic pancreatic tumor
Number	164	16	14	9	7	3
Age (month)	146 (75-191)	58 (0-156)	76.5 (0-206)	107 (18-187)	102 (12-155)	36 (33-129)
Male:female	36:128	9:7	6:8	4:5	5:2	2:1
Year of diagnosis
2000-2010	48	6	2	3	3	2
2011-2021	116	10	12	6	4	1
Stage
Localized	160	8	14	8	5	2
Distant	4	8	0	1	2	1
Location
Head	41	6	5	6	3	2
Body	23	2	3	1	1	1
Tail	72	1	2	0	2	0
Head + body	4	2	1	0	1	0
Body + tail	24	4	3	1	0	0
Other	0	1	0	1	0	0
Tumor size (mm)	50 (10-110)	90 (25-170)	16.5 (6-25)	60 (30-127)	110 (58.4-122)	30 (12-60)
			6 cases unknown size

Age at onset, sex, presence of distant disease, location, and tumor size are presented; 149 (70.0%) of the 213 patients are from 2011 onward. SPN: Solid pseudopapillary neoplasm.

Table 2 Symptoms per disease (trigger of detection) with overlapping symptoms, n (%)

	SPN	Pancreatoblastoma	Pancreatic endocrine tumor	Non-epithelial tumor	Pancreatic cancer	Metastatic pancreatic tumor
Number	164	16	14	9	7	3
Fever	5 (3.0)	0 (0)	1 (7.1)	1 (11.1)	0 (0)	1 (33.3)
Nausea/vomiting	60 (36.6)	2 (12.5)	0 (0)	4 (44.4)	2 (28.6)	0 (0)
Abdominal pain	108 (65.9)	7 (43.8)	2 (14.3)	6 (66.6)	2 (28.6)	0 (0)
Abdominal mass	11 (6.7)	8 (50.0)	0 (0)	2 (22.2)	4 (57.1)	1 (33.3)
Trauma	31 (18.9)	0 (0)	0 (0)	1 (11.1)	0 (0)	0 (0)
Jaundice	1 (0.6)	2 (12.5)	0 (0)	1 (11.1)	0 (0)	0 (0)
Image abnormality	37 (22.6)	0 (0)	3 (21.4)	0 (0)	1 (14.3)	1 (33.3)
Abnormal blood examination	1 (0.6)	1 (6.3)	8 (57.1)	0 (0)	0 (0)	1 (33.3)
Others	8 (4.9)	5 (31.3)	1 (7.1)	2 (22.2)	0 (0)	0 (0)

The occasion of diagnosis is shown for each tumor. There are duplicate responses. SPN: Solid pseudopapillary neoplasm.

Table 3 Disease-specific surgical procedures and outcomes, n (%)

	SPN	Pancreatoblastoma	Pancreatic endocrine tumor	Non-epithelial tumor	Pancreatic cancer	Metastatic pancreatic tumor
Number	164	16	14	9	7	3
Type of resection
Pancreatoduodenectomy (laparoscopic)	17 (1)	8 (0)	2 (0)	2 (0)	2 (0)	1 (0)
Distal pancreatectomy (laparoscopic)	105 (48)	7 (0)	7 (1)	1 (0)	2 (0)	1 (0)
Total pancreatectomy (laparoscopic)	0 (0)	1 (0)	0 (0)	0 (0)	0 (0)	0 (0)
Enucleation (laparoscopic)	35 (6)	0 (0)	4 (1)	4 (1)	1 (0)	0 (0)
Other (laparoscopic)	7 (0)	0 (0)	1 (0)	2 (0)	2 (0)	1 (0)
Involvement of adult gastrointestinal surgeons
Operator	29	4	5	0	2	0
Assistant	39	4	1	0	1	0
None	96	8	8	9	4	3
Complication	33	3	2	1	4	0
Pancreatic fistula	28	1	2	0	4	0
Others	5	2	0	1	0	0
Outcome
Alive without recurrence	155	10	14	7	2	1
With recurrence	8	2	0	1	1	0
Death	0	4	0	1	4	2

The surgical technique, complications, and outcomes for each tumor are presented. The extent to which adult gastrointestinal surgeons are involved is also presented. SPN: Solid pseudopapillary neoplasm.

Table 4 Comparison of disease frequencies between our study and the Surveillance, Epidemiology, and End Results, National Cancer Database, United Kingdom National Registry of Childhood Tumors, and TREP database, n (%)

	Our study	SEER	National Cancer Database	United Kingdom National Registry of Childhood Tumors	TREP database
Age, year	≤ 18	≤ 19	≤ 19	≤ 18	≤ 18
Country	Japan	United States	United States	United Kingdom	Italy
Time	2000-2021	1975-2018	2004-2014	1971-2000	2000-2009
Number	213	195	109	25	21
Solid pseudopapillary neoplasm	164 (77.0)	63 (32.3)	33 (30.3)	8 (32)	12 (57.1)
Pancreatoblastoma	16 (7.5)	22 (11.3)	17 (15.6)	11 (44)	4 (19.0)
Pancreatic endocrine tumor	14 (6.6)	66 (33.8)	30 (27.5)	0	3 (14.3)
Sarcoma	2 (0.9)	44 (22.6)	7 (6.4)	0	0
Pancreatic adenocarcinoma	1 (0.5)		17 (15.6)	6 (24)	2 (9.5)
Acinar cell carcinoma	6 (2.8)		0	0	0
Metastatic pancreatic tumor	3 (1.4)	0	0	0	0
Others	7 (3.3)	0	5 (4.6)	0	0

A comparison of multicenter case summaries of pediatric pancreatic tumors presents the distribution of the disease in each report. SEER: Surveillance, Epidemiology, and End Results.

Table 5 Comparison of solid pseudopapillary neoplasm between our study and the Surveillance, Epidemiology, and End Results

	Our study (2000-2021)	SEER (1975-2018)	P value
	n = 164	n = 63
Age at diagnosis			< 0.05
< 14 years	153	21
> 14 years	11	42
Sex			< 0.05
Male	36	6
Female	128	57
Race			< 0.05
White	0	47
Others	164	16
	All Asian
Primary site
Head	41	21	0.246
Body	23	7	0.665
Tail	72	21	0.176
Other	28	0	< 0.05
Unknown	0	14	-
Localized	160	45	< 0.05
Regional and distant	4	18	< 0.05
Surgery			< 0.05
Yes	164	59
No	0	4
5-year overall survival	100%	100%

The age at onset was more likely to be lower in Japan than in other countries. All 164 patients with solid pseudopapillary neoplasm in our study survived. All 63 patients with solid pseudopapillary neoplasms aged < 19 years also survived. SEER: Surveillance, Epidemiology, and End Results.