Case Report
Copyright ©The Author(s) 2022.
World J Gastroenterol. Dec 7, 2022; 28(45): 6421-6432
Published online Dec 7, 2022. doi: 10.3748/wjg.v28.i45.6421
Table 1 Clinicohistopathologic features of the pancreatic acinar cell carcinoma patients in this dataset (n = 11)
Characteristic
Number of patients (%)
Male11 (100)
Median age at diagnosis, yr65 (56.5-74.0)
Median tumor size, cm7.0 (2.7-16.4)
Histology
Pure acinar6 (55)
Mixed acinar-neuroendocrine3 (27)
Mixed acinar-ductal2 (18)
Primary tumor site
Head/uncinate4 (37)
Body2 (18)
Tail5 (45)
Stage
Resectable2 (18)
Locally advanced2 (18)
Metastatic7 (64)
1st line treatment
Surgery only1 (9)
Surgery and chemotherapy2 (18)
Preoperative chemoradiotherapy and surgery1 (9)
Chemotherapy only7 (64)
Palliative chemotherapy
Modified FOLFIRINOX6 (60)
Gemcitabine with Nab-paclitaxel3 (30)
Gemcitabine1 (10)
Table 2 Germline and somatic molecular profiles of the pancreatic ductal adenocarcinoma patients in this study
Year of diagnosis
Age at diagnosis
Germline testing
Somatic testing
Driver mutation
Personal or family history of malignancy
Disease staging
Surgery and perioperative chemotherapy
TTP after surgery (mo)
First line systemic therapy
Second line systemic therapy
Best treatment and clinical status
OS (mo)
201465BRCA1, BRCA2 negativeNot performedNilFamily history of leukemia, colorectal, ovarian, and prostate cancersT3N0M0 R1Neoadjuvant mFFX with radiotherapy followed by Whipple procedure1.5Gemcitabine for 4 cyclesBSCPD, DOD28.2
MMR IHC intact
201463Not performedNot performedNilNilT3N1bM0 R0Whipple procedure with perioperative mFFX for 12 cycles10.4BSCNilPD, DOD23.2
201474CDKN2A pathogenic variant c.159G>C, p.Met53IleNot performedNilFamily history of melanoma and PDACStage IVNilNAmFFX for 3 cyclesBSCPD, DOD4.6
Personal history of malignant melanoma
201560Not performedNot performedNilThoracic cancerT2N0M0 R0Distal pancreatectomy with adjuvant Gemcitabine for 2 cycles which were discontinued due to toxicities10.7GnP 18 moN/ASD, DOD36.0
201766WGS and RNA seqWGS and RNA seq KRAS, SMAD4NilStage IVNilNAmFFX for 8 cyclesBSCSD, DOD13.4
KRAS, SMAD4, CDKN2A TP53, ATM, TGFBR2, and KDM6A
MMR IHC intact
201864Not performedNot performedNilNilT3N0M0 R0Whipple procedure. No adjuvant therapy10.6GnP for 4 cyclesBSCPD, DOD32.3
20206191 gene panelWGS and RNA seqBRAFFamily history of PDACStage IVNilNAGnP for 16 cyclesCobimetinib 60 mg OD PO (enrolled on CAPTUR trial PR, AWD20.4 ongoing
SND1-BRAF fusion
MMR IHC intact
202170 (case described)12 gene panelNot performedBRCA2Family history of ovarian cancerStage IVNilNAmFFX for 11 cyclesMaintenance Olaparib 150 mg twice dailyPR, AWD11.5 ongoing
BRCA2
Likely pathogenic variant
c.4356delinsCA, p.Gln1452Hisfs*8MMR IHC intactPersonal history of renal cell cancer and NSCLA
20216591 gene panelNot performedNilFamily history of head and neck cancerStage IVNilNAmFFX for 14 cycles, followed by maintenance FOLFIRIRP-3500 in combination with Gemcitabine (enrolled on RP-3500-01 trial)SD, ADW13.4 ongoing
ATM
Pathogenic variant
c.8418+5_8418+8del
202171BRCA1, BRCA 2Not performedBRCA2Family history of breast cancersStage IVNilNA5FU with Oxaliplatin for 12 cycles, downsized to Whipple procedureN/APR, AWD12.1 ongoing
BRCA2 pathogenic variant c.8904delC, p.Val2969Cysfs*7
20215712 gene panel negativeNot performedNilFamily history of non-Hodgkins LymphomaStage IVNilNAOngoing mFFX; had 16 cycles N/APR, AWD12.3 ongoing