Successful BRAF/MEK inhibition in a patient with BRAF^V600E-mutated extrapancreatic acinar cell carcinoma.

ABSTRACT: Pancreatic acinar cell carcinoma (PAC) is a rare disease with a poor prognosis. Treatment options for metastatic PAC are limited and often follow chemotherapeutic regimens for pancreatic ductal adenocarcinoma. Although recurrent genomic alterations, such as BRAF fusions and defects in genes involved in homologous recombination DNA repair, have been described in PAC, data on the clinical efficacy of molecularly guided, targeted treatment are scarce. Here we describe the case of a 27-yr-old patient with BRAF^V600E-mutated PAC who was successfully treated with a combination of BRAF and MEK inhibitors. The patient presented to our clinic with abdominal pain and weight loss. Imaging showed extensive retroperitoneal disease as well as mediastinal lymphadenopathy. Because

SUBMITTER: Busch E 

PROVIDER: S-EPMC7476408 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature