To the Editor: In the last decade, next-generation sequencing(NGS)-based molecular profiling has shed light on the molecular landscape of biliary tract cancer(BTC), revealing the presence of several potentially action...To the Editor: In the last decade, next-generation sequencing(NGS)-based molecular profiling has shed light on the molecular landscape of biliary tract cancer(BTC), revealing the presence of several potentially actionable mutations [1]. According to Clinical Trials.gov, there are at least 40 phase I to IV ongoing trials aimed at evaluating the role of targeted treatments in BTC, as monotherapy or in combination with other anticancer agents [2]. Early encouraging results have been observed with therapies targeting IDH mutations, FGFR fusions, HER family and, more recently, BRAF mutations [3].展开更多
BACKGROUND Pancreatic mucinous cystadenocarcinoma(MCAC)is a rare malignancy with a poor prognosis when it presents metastases at diagnosis.Due to its very low incidence,there are no clear recommendations for the treat...BACKGROUND Pancreatic mucinous cystadenocarcinoma(MCAC)is a rare malignancy with a poor prognosis when it presents metastases at diagnosis.Due to its very low incidence,there are no clear recommendations for the treatment of advanced disease.Olaparib(an oral PARP inhibitor)has been approved for the maintenance treatment of patients with metastatic pancreatic adenocarcinoma harbouring germline BRCA1/2 mutations.Herein,we report the first case of a germline BRCA1 mutated unresectable MCAC which was effectively treated with olaparib.CASE SUMMARY A 41-year-old woman,without personal or family history of cancer,was diagnosed with ovarian and peritoneal metastases of MCAC.She underwent 12 cycles of gemcitabine plus oxaliplatin(GEMOX)obtaining a partial response and allowing radical surgery.One year later,local recurrence was documented,and other 12 cycles of GEMOX were administered obtaining a complete response.Seven years later,another local recurrence,not amenable to surgical resection,was diagnosed.She started FOLFIRINOX(oxaliplatin,irinotecan,leucovorin and fluorouracil),obtaining a partial response after 8 cycles.Given the excellent response to platinum-based chemotherapy,BRCA testing was performed,and a BRCA1 germline mutation was detected.She was switched to maintenance olaparib due to chemotherapy-related toxicities and achieved an almost complete metabolic response,with a reduction in the diameter of the lesion,after three months of therapy.CONCLUSION The current case suggests the beneficial effect of olaparib in BRCA mutated MCAC.However,further studies are required.展开更多
Dear Editor:In the last two decades,the indications of liver transplantation(LT)for primary and secondary hepatobiliary malignancies have been increasingly expanded.Although this attractive option still represents the...Dear Editor:In the last two decades,the indications of liver transplantation(LT)for primary and secondary hepatobiliary malignancies have been increasingly expanded.Although this attractive option still represents the“last court of appeal”in cancer patients,the role of LT is well established in hepatocellular carcinoma(HCC),where transplantation has also demonstrated a benefit for selected patients affected by peri-hilar cholangiocarcinoma,intrahepatic cholangiocarcinoma,and neuroendocrine tumors[1].Recently,the interest in LT in liver-limited stage IV colorectal cancer(CRC)has increased due to recent advances in transplantation techniques that have led to a re-evaluation of this approach.展开更多
文摘To the Editor: In the last decade, next-generation sequencing(NGS)-based molecular profiling has shed light on the molecular landscape of biliary tract cancer(BTC), revealing the presence of several potentially actionable mutations [1]. According to Clinical Trials.gov, there are at least 40 phase I to IV ongoing trials aimed at evaluating the role of targeted treatments in BTC, as monotherapy or in combination with other anticancer agents [2]. Early encouraging results have been observed with therapies targeting IDH mutations, FGFR fusions, HER family and, more recently, BRAF mutations [3].
文摘BACKGROUND Pancreatic mucinous cystadenocarcinoma(MCAC)is a rare malignancy with a poor prognosis when it presents metastases at diagnosis.Due to its very low incidence,there are no clear recommendations for the treatment of advanced disease.Olaparib(an oral PARP inhibitor)has been approved for the maintenance treatment of patients with metastatic pancreatic adenocarcinoma harbouring germline BRCA1/2 mutations.Herein,we report the first case of a germline BRCA1 mutated unresectable MCAC which was effectively treated with olaparib.CASE SUMMARY A 41-year-old woman,without personal or family history of cancer,was diagnosed with ovarian and peritoneal metastases of MCAC.She underwent 12 cycles of gemcitabine plus oxaliplatin(GEMOX)obtaining a partial response and allowing radical surgery.One year later,local recurrence was documented,and other 12 cycles of GEMOX were administered obtaining a complete response.Seven years later,another local recurrence,not amenable to surgical resection,was diagnosed.She started FOLFIRINOX(oxaliplatin,irinotecan,leucovorin and fluorouracil),obtaining a partial response after 8 cycles.Given the excellent response to platinum-based chemotherapy,BRCA testing was performed,and a BRCA1 germline mutation was detected.She was switched to maintenance olaparib due to chemotherapy-related toxicities and achieved an almost complete metabolic response,with a reduction in the diameter of the lesion,after three months of therapy.CONCLUSION The current case suggests the beneficial effect of olaparib in BRCA mutated MCAC.However,further studies are required.
文摘Dear Editor:In the last two decades,the indications of liver transplantation(LT)for primary and secondary hepatobiliary malignancies have been increasingly expanded.Although this attractive option still represents the“last court of appeal”in cancer patients,the role of LT is well established in hepatocellular carcinoma(HCC),where transplantation has also demonstrated a benefit for selected patients affected by peri-hilar cholangiocarcinoma,intrahepatic cholangiocarcinoma,and neuroendocrine tumors[1].Recently,the interest in LT in liver-limited stage IV colorectal cancer(CRC)has increased due to recent advances in transplantation techniques that have led to a re-evaluation of this approach.
