Prolonged Survival in a Patient with a Pancreatic Acinar Cell Carcinoma

Abstract

Pancreatic acinar cell carcinoma (ACC) is a rare entity. Herein we present the case of a 50-year-old male patient with an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion and multiple metastases in the liver and spleen. A liver biopsy showed a pancreatic ACC. The patient received 9 cycles of gemcitabine plus oxaliplatin (GEMOX regimen), which had to be stopped because of a persistent grade 2 neuropathy. A CT scan showed complete response after 14 years. At the age of 61 years, a localized prostatic cancer was diagnosed, treated by prostatectomy. The patient carried a BRCA2 mutation. None of the precedent case reports describe a chemosensibility to the GEMOX regimen. In spite of the lack of study in these patients, chemotherapy with oxaliplatin seems to be the most effective. Long survival can be expected.

Pancreatic acinar cell carcinoma is associated with BRCA2 germline mutations: a case report and literature review

“…Including the current case, a total of 10 ACC patients with germline BRCA2 mutations have now been reported (Table 2). 7,[17][18][19][20][21] Loss of the wild-type BRCA2 allele, observed in all cases with known zygosity, 7,17 supports the causal relation between BRCA2 germline mutation and the onset of ACC. Moreover, literature review showed that somatic BRCA2 alterations occur in about 12% of ACCs.…”

“…Literature review of ACC in patients with a germline BRCA2 mutation identified nine additional cases (Table 2). 7,[17][18][19][20][21] The mean age at diagnosis was 62 years (SD: 8.7; range: 50-78), which is similar to sporadic cases. 4 Including the current patient, seven males (67%) and three females (33%) were identified, resulting in male-to-female ratio of 2.3:1, corresponding to the male predominance in sporadic ACC.…”

“…Of note, the actual prevalence of both germline and somatic BRCA1/2 mutations in pancreatic ACC can be under-or overestimated mainly due to the rarity of this tumor type, lack of large-scale genomic analyses and possible underrepresentation in literature. Patients with ACC and germline BRCA1/2 alterations were often described in case reports 7,[17][18][19][20]22,23 hindering the possibility to estimate real portion of ACCs emerged due to BRCA1/2 germline mutations. The heterogeneity observed between sequencing studies is also remarkable: only one out of four studies detected somatic BRCA1 mutations, and one WES study did not identify any BRCA1/2 mutation in 22 ACCs.…”

“…7,17 Three out of 10 patients had a previous personal history of another BRCA2-associated malignancy, including breast, ovarian and prostate cancers. 17,18,20 In addition, three patients, including the current, had a family history of BRCA2associated malignancies and/or family member(s) harboring germline BRCA2 mutations. 18,19 Two patients presented with PP as the initial manifestation of ACC.…”

“…17,18,20 In addition, three patients, including the current, had a family history of BRCA2associated malignancies and/or family member(s) harboring germline BRCA2 mutations. 18,19 Two patients presented with PP as the initial manifestation of ACC. 19,20 In addition, literature search identified two ACC patients with BRCA1 germline mutation.…”