PDF(Pubmed)
Abstract：
Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic tumor type, and ruptured pancreatic tumors are rarer. Computed tomography (CT) in a 48-year-old man incidentally revealed a raptured pancreatic tail tumor. The patient was treated conservatively because he was asymptomatic, and his general condition was stable. After a detailed examination, the pancreatic tumor was diagnosed as raptured PACC. Considering the potential infiltration of tumor cells into the hematoma within the omental sac, our decision is to initiate chemotherapy as the primary course of action. A liquid biopsy was performed, and comprehensive genomic profiling of circulating tumor DNA showed a tumor BRCA2 mutation. Chemotherapy with modified FOLFIRINOX (mFFX) was selected as the first treatment. After seven courses of mFFX, the primary tumor diminished remarkably. At this time, the radical resection was performed via distal pancreatectomy with simultaneous resection of the gastric wall and colon, which had adhered strongly to the tumor. Histopathological examination revealed that the tumor had shrunk to less than 5% of its original size due to chemotherapy (Grade 3 of Evans Classification). Devising treatment strategies for ruptured pancreatic malignant tumors is challenging due to the worsening general condition caused by severe abdominal symptoms and intra-abdominal bleeding. In this context, this case-report documents a rare instance of raptured PACC with a tumor BRCA2 mutation that underwent radical resection following mFFX treatment.
摘要：
胰腺腺泡细胞癌（PACC）是一种罕见的胰腺肿瘤类型，破裂的胰腺肿瘤更罕见。一名48岁男子的计算机断层扫描（CT）偶然发现胰腺尾部肿瘤。病人因无症状而接受保守治疗,一般情况稳定.经过详细的检查，胰腺肿瘤被诊断为PACC破裂。考虑到肿瘤细胞可能浸润到网膜囊内的血肿中，我们的决定是将化疗作为主要的治疗方案.进行了液体活检，循环肿瘤DNA的综合基因组分析显示肿瘤BRCA2突变。选择使用改良FOLFIRINOX(mFFX)的化疗作为第一治疗。经过七门mFFX课程，原发肿瘤明显缩小。此时,根治性切除术是通过远端胰腺切除术同时切除胃壁和结肠，强烈粘附在肿瘤上。组织病理学检查显示,由于化疗(Evans分类的3级),肿瘤缩小至其原始大小的5%以下。由于严重的腹部症状和腹腔内出血引起的全身状况恶化，因此针对破裂的胰腺恶性肿瘤制定治疗策略具有挑战性。在这种情况下，本病例报告记录了一例罕见的PACC伴肿瘤BRCA2突变,在mFFX治疗后接受了根治性切除.
