Near complete remission of an inoperable pancreatic acinar cell carcinoma after BRAF‐/MEK‐inhibitor treatment—A case report and review of the literature

Author:

von Fritsch Lennart1ORCID,von Bubnoff Nikolas2ORCID,Weber Klaus3,Kirfel Jutta4,Schreiber Cleopatra4,Keck Tobias1,Wellner Ulrich1

Affiliation:

1. Department of Surgery University Hospital of Schleswig‐Holstein, Campus Luebeck Lübeck Germany

2. Department of Hematology and Oncology University Hospital of Schleswig‐Holstein, Campus Luebeck Lübeck Germany

3. Luebecker Onkologische Schwerpunktpraxis Lübeck Germany

4. Institute of Pathology, University Hospital Schleswig‐Holstein, Campus Luebeck Lübeck Germany

Abstract

AbstractIntroductionPancreatic acinar cell carcinomas are rare malignant neoplasms. High‐quality evidence about the best treatment strategy is lacking. We present the case of a 52‐year‐old male with a BRAFV600E‐mutated PACC who experienced a complete remission after chemotherapy with BRAF‐/MEK‐inhibitors.CaseThe patient presented with upper abdomen pain, night sweat, and weight loss. CT scan showed a pancreatic tumor extending from the pancreas head to body. Histological workup identified an acinar cell carcinoma. As the tumor was inoperable, chemotherapy with FOFIRNIOX was initiated and initially showed a slight regression of disease. The regimen had to be discontinued due to severe side effects. Molecular analysis identified a BRAFV600E mutation, so the patient was started on BRAF‐ and MEK‐inhibitors (dabrafenib/trametinib). After 16 months, CT scans showed a near complete remission with a markedly improved overall health.DiscussionStudies suggest that up to one‐fourth of PACCs carry a BRAF mutation and might therefore be susceptible to a BRAF‐/MEK‐inhibitor therapy. This offers a new therapeutic pathway to treat this rare but malignant neoplasm.

Publisher

Wiley

Reference26 articles.

1. HrubanRH PitmanMB KlimstraDS American Registry of Pathology Armed Forces Institute of Pathology.Tumors of the Pancreas.2007.

2. Acinar Cell Carcinoma of the Pancreas

3. CT and MRI features of acinar cell carcinoma of the pancreas with pathological correlations

4. Metastatic Acinar Cell Carcinoma of the Pancreas

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