Abstract：
Pancreatic 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.
The 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.
Studies 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.
The 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.
Studies 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.
摘要：
背景：胰腺腺泡细胞癌是一种罕见的恶性肿瘤。缺乏关于最佳治疗策略的高质量证据。我们介绍了一名52岁男性，患有BRAFV600E突变的PACC，在使用BRAF/MEK抑制剂进行化疗后完全缓解。
方法：患者表现为上腹部疼痛，盗汗,和减肥。CT扫描显示胰腺肿瘤从胰腺头部延伸到身体。组织学检查发现腺泡细胞癌。由于肿瘤无法手术，开始使用FOFIRNIOX进行化疗,最初显示疾病有轻微消退.由于严重的副作用,该方案不得不停止。分子分析确定了BRAFV600E突变，因此患者开始服用BRAF和MEK抑制剂(达拉非尼/曲美替尼).16个月后,CT扫描显示几乎完全缓解，整体健康状况明显改善。
结论：研究表明，多达四分之一的PACCs携带BRAF突变，因此可能对BRAF/MEK抑制剂治疗敏感。这为治疗这种罕见但恶性的肿瘤提供了新的治疗途径。
方法：患者表现为上腹部疼痛，盗汗,和减肥。CT扫描显示胰腺肿瘤从胰腺头部延伸到身体。组织学检查发现腺泡细胞癌。由于肿瘤无法手术，开始使用FOFIRNIOX进行化疗,最初显示疾病有轻微消退.由于严重的副作用,该方案不得不停止。分子分析确定了BRAFV600E突变，因此患者开始服用BRAF和MEK抑制剂(达拉非尼/曲美替尼).16个月后,CT扫描显示几乎完全缓解，整体健康状况明显改善。
结论：研究表明，多达四分之一的PACCs携带BRAF突变，因此可能对BRAF/MEK抑制剂治疗敏感。这为治疗这种罕见但恶性的肿瘤提供了新的治疗途径。
