Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors

Table 1 Somatic genetic alterations observed in pancreatic acinar cell carcinoma specimens n (%)

Gene	Ref.	Number of patients	Number of patients with mutation (frequency )
TP53	Jiao et al[12]	23	3 (13)
	Chmielecki et al[26]	44	10 (23)
BRAF/RAF11	Jiao et al[12]	23	3 (13)
	Chmielecki et al[26]	44	11 (25)
	Bergmann et al[25]	42	0 (0)
SMAD4	Jiao et al[12]	23	6 (26)
	Chmielecki et al[26]	44	6 (26)
BRCA2	Jiao et al[12]	23	1 (4)
	Chmielecki et al[26]	44	9 (20)
	Furukawa et al[27]	7	3 (43)
CDK2NA	Jiao et al[12]	23	4 (17)
	Chmielecki et al[26]	44	6 (14)
MMR/MSI	Liu et al[24]	36	5 (14)
	Bergmann et al[25]	42	2 (5)
RB1	Jiao et al[12]	23	3 (13)
	Chmielecki et al[26]	44	5 (11)
APC and CTNNB1	Jiao et al[12]	23	2 (9)
	Abraham et al[5]	17	4 (24)
	Chmielecki et al[26]	44	4 (9)
BRCA1	Jiao et al[12]	23	0 (0%)
	Chmielecki et al[26]	44	4 (9)
JAK1	Jiao et al[12]	23	4 (17)
MEN1	Jiao et al[12]	23	1 (4)
	Chmielecki et al[26]	44	3 (7)
GNAS	Jiao et al[12]	23	2 (9)
	Chmielecki et al[26]	44	2 (5%)
FAT	Furukawa et al[27]	7	4 (57)
Allelic Loss on Chromosome 11p	Abraham et al[5]	12	6 (50)

¹Including BRAF gene fusion and point mutations.

Table 2 Chemotherapeutic regimens that showed activity in patients with pancreatic acinar cell carcinomas (9, 16, 18, 19, 22)

Regimen	Ref.	Total number of patients	Best responses
Gemcitabine	Lowery et al[6]	3	SD at 1 yr
Gemcitabine + erlotinib	Lowery et al[6]	4	PR at 5 mo, SD at 10 mo
Gemcitabine + irinotecan	Lowery et al[6]	2	SD at 25 mo
Cisplatin + irinotecan	Lowery et al[6]	1	PR at 12 mo, POD at 25 mo
Gemcitabine + cisplatin	Lowery et al[6]	2	PR at 4 mo
FOLFIRI	Lowery et al[6]	4	PR at 1.5 mo, POD at 11 mo
Gemcitabine + capecitabine	Lowery et al[6]	1	SD then POD at 9 mo
Gemcitabine + docetaxel + capecitabine	Lowery et al[6]	2	SD at 11 mo
Gemcitabine + oxalipatin	Lowery et al[6]	5	PR at 6 mo, POD at 15 mo
Capecitabine + erlotinib	Lowery et al[6]	1	SD at 15 mo, stopped because of toxicity
Folfirinox	Schempf et al[30]	1	PR with regression of primary disease and liver mets
Cisplatin + S11	Furukawa et al[27]	1	CR with resolution of Liver mets. NED after 5 yr
Panitumumab2	Morales et al[40]	2	Clinically stable at 4 mo
Liposomal doxorubicin3	Armstrong et al[29]	1	PR for ≥ 1 yr. Treatment discontinued due to cardiac toxicity risk
Docetaxel + irinotecan + cetuximab	Cananzi et al[41]	1	PR for 7 mo

¹BRCA-2 mutated; ²KRAS wild-type gene;

³The patient was treated with liposomal doxorubicin. DNA microarray and IHC analyses of a biopsy of liver metastases showed elevated topoisomerase II expression and growth inhibition by doxorubicin (a topoisomerase II inhibitor) in vitro in a cell line derived from the patient’s tumor. FOLFIRI: 5-FU/Leucovorin/Irinotecan; FOLFIRINOX: 5-FU/Leucovorin/Oxaliplatin/Irinotecan; PR: Partial Response; POD: Progression of disease; SD: Stable disease; CR: Complete Response; NED: No evidence of disease.