Long-term outcome of pancreatic function following oncological surgery in children: Institutional experience and review of the literature

Table 1 Our scheduled follow-up for pancreatic neoplasms after surgery in children

	T1	T2	T3
Clinical examination (physical examination, symptoms, weight, height, BMI)	Every 4 mo	Every 6 mo	Once a year
Routine lab tests (CBC, liver and kidney function tests, amylase, lipase, bilirubin, albumin, etc.)	Every 4 mo	Every 6 mo	Once a year
Vitamin dosage (A, D, E, clotting test)	Every 4 mo	Every 6 mo	Once a year
Serological markers (according to the underlying histology) (alpha-FP, Chromogranin-A)	Every 4 mo	Every 6 mo	Once a year
Assessment of exocrine pancreatic function (fecal elastase)	Every 4 mo	Every 6 mo	Once a year
Assessment of endocrine pancreatic function (fasting blood glucose, glycated hemoglobin, C-peptide)	Every 4 mo	Every 6 mo	Once a year
Imaging (abdominal US)	Every 4 mo	Every 6 mo	Once a year
Surgical, diabetological and oncological consulting	Every 4 mo	Every 6 mo	Once a year
Nutritional assessment (3-d recall dietary assessment and diet optimization by dietitians)	Every 4 mo	Every 6 mo	Once a year

T1: First year after diagnosis; T2: Second year after diagnosis; T3: Third year to 10 yr after diagnosis (end of follow-up); BMI: Body mass index; CBC: Complete blood count; alpha-FP: alpha-Fetoprotein; US: Ultrasound.

Table 2 Main demographic and clinical characteristics of our patients

No.	Sex	Age	Histology	Symptoms	Tumor site	Type of surgery	Length of follow-up	Exocrine pancreatic failure	Endocrine pancreatic failure	Vitamin D insufficiency
1	F	12	SPT	Weight loss	HEAD	PPPD	10.4 yr	Yes	Yes	Yes
2	M	9.8	SPT	Occasional diagnosis	BODY	Distal Pancreatectomy	6.4 yr	No	No	No
3	F	17.3	SPT	AP	HEAD	PPPD	3.9 yr	Yes	No	Yes
4	M	15.5	Neuroendocrine tumor	AP	HEAD	PPPD	2.3 yr	No	No	No
5	F	6.6	Acinar cell carcinoma	AP+ weight loss	HEAD	PPPD	11.5 yr	Yes	Yes	Yes
6	M	4.11	Acinar cell carcinoma	Not available	HEAD	PD	12.3 yr	Yes	No	Yes
7	F	14.3	SPT	Weight loss	TAIL	Distal Pancreatectomy	2 yr	No	No	Yes
8	F	13.2	SPT	AP	HEAD	PPPD	9 mo	No	No	Yes
9	F	3 mo	Insulinoma	Hypoglycemia	HEAD	Cephalic Pancreatectomy	11 yr	No	No	Yes
10	F	11	SPT	AP	BODY/TAIL	Distal Splenopancreatectomy	2 yr	No	No	No
11	M	10	Neuroendocrine tumor	Screening in Tuberous Sclerosis	BODY/TAIL	Distal Pancreatectomy	9 yr	No	No	No
12	F	15.5	SPT	No symptoms	BODY/TAIL	Distal Splenopancreatectomy	2.5 yr	No	No	No
13	F	13.8	SPT	AP	HEAD	PPPD	10 yr	No	No	No
14	F	11.6	SPT	No symptoms	BODY/TAIL	Distal Splenopancreatectomy	3.4 yr	No	No	Yes
15	F	16.2	SPT	AP+ weight loss	TAIL	Distal Pancreatectomy	3.8 yr	No	No	No
16	F	7 mo	Insulinoma	Hypoglycemia	HEAD	PPPD	1 mo	No	No	No

F: Female; M: Male; Age: Years at diagnosis; SPT: Solid pseudopapillary tumor; AP: Abdominal pain; PD: Pancreaticoduodenectomy; PPPD: Pylorus preserving pancreaticoduodectomy; Vitamin D insufficiency: < 30 ng/mL.

Table 3 Summary of the reports included in the literature review

Ref.	Country	Length of follow-up (yr)	n of cases/ M/F (%)	Mean or median age (yr)	Exocrine insufficiency (%)	Endocrine insufficiency (%)
Present	Italy	5.7	16 (25/75)	10.7	25	12.5
Cheng et al[26], 2020	China	3.1	104 (31/69)	9.9	8.6	1
Vasudevan et al[27], 2020	United States	2.8	65	13	23	3
Mizuno et al[28], 2018	Japan	30	1 (M)	12	0	0
Divarcı et al[29], 2017	Turkey	3.6	5 (0/100)	15	0	0
d’Ambrosio et al[30], 2014	Italy	2.1	5 (40/60)	7	20	20
Laje et al[31], 2013	United States	6.7	6 (17/83)	15	17	0
Scandavini et al[32], 2018	Sweden	6.6	13 (23/77)	11.4	31	7,7
Lindholm et al[33], 2017	United States	4.7	12 (42/58)	9	83.3	0
Park et al[34], 2016	Korea	10.5	18 (25/75)	10.5	75	12.5
Muller et al[35], 2012	France	4.2	216 (44/56)	8.9	6.25	0
Speer et al[36], 2012	United States	1.4	11 (36/64)	14	9	0
Yazbeck et al[37], 2010	Lebanon	-	1 (F)	13	100	0

¹Two patients excluded due to pancreatic trauma.

²Two patients excluded due to congenital pancreatic malformation and calcifying pancreatitis.

F: Female; M: Male.

Table 4 Overall main characteristics of the reviewed studies

Onset symptoms (%)	Histology (%)	Type of surgery (%)
Abdominal pain (50)	SPT (64)	PD (61)
Palpable mass (17)	Pancreatoblastoma (13)	Distal/central pancreatectomy (30)
Nausea/emesis (16)	Neuroendocrine tumors (7)	Tumor enucleation (9)
Occasional diagnosis (8)	Neuroblastoma (4)
Jaundice (8)	Rhabdomyosarcoma/sarcoma (4)
Diarrhea (1)	Acinar cell carcinoma (4)
	Pancreatic islet cells cancer (1)
	Other (3)

SPT: Solid pseudopapillary tumor; PD: Pancreaticoduodenectomy.

Table 5 Pancreatic function and neoplasm site/surgery

	Overall	Head (%)	Body/tail (%)
	235	149 (63)	106 (37)
Exocrine insufficiency	35	31 (89)	4 (11)
Endocrine insufficiency	5	4 (80)	1 (20)
	Overall	PD (%)	Other surgery (%)
	140	106 (76)	34 (24)
Exocrine insufficiency	30	28 (93)	2 (7)
Endocrine insufficiency	5	4 (80)	1 (20)

PD: Pancreaticoduodenectomy.