Adult pancreatoblastoma: Systematic review of the literature and case report of a young adult patient

Table 1 Tumor location

Tumor location	Frequency1	Ref.
Pancreatic head	37/79	[4,6-27]
Pancreatic body	13/79	Case from this study[4,14,24,28-36]
Pancreatic tail	27/79	[6,7,9,11,14,16,17,24,28,37-49]
Ampulla	2/79	[50,51]

¹Number of cases with designated location over the total number of cases that mention tumor location.

Table 2 Tumor size

Tumor size (cm)	Ref.
1 cm to 3.9 cm	[7,9,14,24,26,29,30,35,50,51]
4.0 cm to 9.9 cm	Case from this study[4,7-16,18,19,21-25,27-29,31,34,36,38,40,43,46-48]
10 cm and larger	[7,9,11,17,20,32,33,37,42,44,45,49,52,53]

Table 3 Tumor areas of metastasis/invasion

Area of metastasis/invasion	Ref.
Adrenal	[17]
Bone	[11]
Breast	[24]
Brain	[39]
Colon/small bowel	[9,20,24,44]
Kidney	[17]
Liver	Case from this study[6-12,14-17,24,25,28,29,32,33,36,38-41,43,44,49,50]
Lung	[6,7,9,17,18,24,28]
Peritoneal carcinomatosis	Case from this study[6,7,28]
Spleen	[17,28,46]
Stomach	[9,28,32,44]

Table 4 Tumor histological features

Histological features	Ref.
Acinar cell groups/differentiation	Case from this study[6,9-11,13,14,28-31,37,40,49,52]
AE1/AE3 (cytokeratin)	[7,10,13,29,31,42,50]
A-hematoxylin	[50]
Alpha-1-antitrypsin	[30]
Alpha-1-chymotrypsin	[30]
Basophilic cells	[37]
Bland nuclei cells	[6]
Blast-like cells	[7]
Board sheets of cells separated by fibrous bands	Case from this study[29]
Chromogranin	Case from this study[6,7,9,28-30,41,52,53]
Chymotrypsin	[4,7,9,17,26,28,29,41]
Cyclin-D1	[28]
EMA squamoid corpuscles	[28]
Eosinophilic cytoplasmic granules/cells	[6,29,30,50]
Epithelioid	[11,13,30,40]
Fibrous bands	Case from this study[6,8,28]
Fine chromatin	[28]
Granular appearance	[41,50]
Grooved nuclei	[7,28]
High nuclear to cytoplasmic ratio	[42,50]
INSM1	[28,52]
Ki-67 > 40%	[8,28,31,38,50]
Focal nuclear molding	[7]
Nested/trabecular growth	[28,30,39,52]
Neuroendocrine component/differentiation	[28,37,52]
NSE	[30,31,38]
Pancytokeratin	[39,54]
Papilae with rare stromal fragments	[42]
Polarization and granular cytoplasm	[30]
Polygonal/spindled cells	[30]
Prominent nucleoli	[8,42]
Pseudopapillae	[7]
Rosette-like formations	Case from this study[52,54]
Squamous corpuscles/nests/differentiation/clusters/morules	[9-13,28,29,39-41,49,50,52,53]
Synaptophysin	Case from this study[9-11,27-30,38,41,43,52]
Trypsin	[4,6,7,9,17,26,28,29,41,52]
Undifferentiated small cells	Case from this study[11]

AE1: Anti-epithelial antigen 1; AE3: Anti-epithelial antigen 3; INSM1: Insulinoma-associated protein 1; NSE: Neuron-specific enolase.

Table 5 Tumor genetic features

Genetic features	Ref.
11p.15.5 mutation	[29]
13q14.2 (RB1)	[40]
6p22	[40]
9p21 (CDKN2A/B) mutation	[29,40]
APC mutation	Case from this study[6,8,12,14,29,40]
B72.3	[17,43]
BCL10	[12,28,29,42,49]
CDKN2A loss (chr 9p)	[6]
CTNNB1 mutation	[6,28,40,42,50,54]
cyclin D1	[37]
DDR2	[37]
EWSR1-FLI1 fusion	[6]
FANCD2	[37]
FGF3	[6]
FGF4	[6]
FGFR1 c1081+1G>T (somatic)	[6]
FGFR2	[37]
FGFR20INA fusion	[6]
INSM1	[28,29,52]
KMT2C	[37]
LAMP1	[37]
MEN1 mutation	[28]
MLH1	[52]
MLH6	[52]
MSH2	[52]
NSD3/WHSc1 L1 mutations	[40]
NSE	[7,40]
PIK3CA mutation	[29]
PMS2	[52]
RB1 Loss of exons18-27	[39]
SMAD4 (DPC4) mutation	[28,29]
SMARCA4	[28]
SMARCB1	[28]
SSTR2 mutation	[31]
Wnt/beta-catenin pathway mutation	[6,7,12,29,37,40,41,43]

Table 6 Tumor treatment

Treatments	Ref.
Surgery	Case from this study[4,6,9-35,37,39-53]
Chemotherapy regimens containing cisplatin, carboplatin, doxorubicin, 5 fluorouracil, mitomycin, bleomycin, gemcitabine, vindesine	Case from this study[6,9-19,23,24,26-28,32,33,36,37,39,40,43,44,46,48,49,52,53]
Radiation	Case from this study[6,9,15-18,23,24,28,33,40,48]
Stem cell transplant	[6,39]
Chemoembolization, ablation	[6,8,9,11,24,28,33]