1. A case of BRCA1-mutated giant pancreatic acinar cell carcinoma successfully treated with modified FOLFIRINOX therapy and radical resection.
- Author
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Watanabe T, Nagaoka Y, Kimura N, Fukasawa M, Shirai Y, Hirano K, Shibuya K, Yoshioka I, Hamashima T, and Fujii T
- Subjects
- Humans, Aged, Male, Mutation, Tomography, X-Ray Computed, BRCA1 Protein genetics, Neoadjuvant Therapy, Pancreatic Neoplasms drug therapy, Pancreatic Neoplasms genetics, Pancreatic Neoplasms pathology, Pancreatic Neoplasms surgery, Pancreatic Neoplasms diagnostic imaging, Carcinoma, Acinar Cell drug therapy, Carcinoma, Acinar Cell genetics, Carcinoma, Acinar Cell pathology, Carcinoma, Acinar Cell surgery, Carcinoma, Acinar Cell diagnostic imaging, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Fluorouracil therapeutic use, Fluorouracil administration & dosage, Leucovorin therapeutic use, Oxaliplatin therapeutic use, Irinotecan therapeutic use, Pancreatectomy methods
- Abstract
Pancreatic acinar cell carcinoma (PACC) is a rare type of pancreatic cancer; further, its pathogenesis and treatment strategies remain unclear. We report the case of a 70-year-old man who presented with a chief complaint of abdominal distention. Computed tomography scans revealed a large lobulated mass (tumor diameter: 150 mm) in the pancreatic body tail, which was diagnosed as a PACC through endoscopic ultrasonography fine needle aspiration. The other imaging modalities did not reveal distant metastases, and the tumor was classified as resectable. Neoadjuvant chemotherapy was planned after staging laparoscopy ruled out microscopic distant metastasis. First-line chemotherapy with gemcitabine + nab-paclitaxel failed due to tumor growth and worsening abdominal distention. Evaluation using the BRACAnalysis
® device indicated that the patient was positive for BRCA1 mutation. Second-line modified FOLFIRINOX (mFFX) resulted in a marked decrease in elastase 1 levels; moreover, a partial antitumor response was observed, which prompted radical resection. After distal pancreatectomy, the patient has survived for 3.5 years without recurrence. BRCA-mutated pancreatic cancer is more likely to respond to mFFX, including platinum, and BRCA mutations have been reported to be highly prevalent in PACC. It is important to evaluate the presence of BRCA mutations in patients with PACC prior to treatment., (© 2024. Japanese Society of Gastroenterology.)- Published
- 2024
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