Hirao S, Sho M, Kanehiro H, Hisanaga M, Ikeda N, Tsurui H, Nakajima Y, Nakano H
First Department of Surgery, Nara Medical University, Japan.
Hepatogastroenterology. 2000 Jul-Aug;47(34):1159-61.
We present a rare case of pancreatic adenocarcinoma in a 47-year-old man with the Peutz-Jeghers syndrome. The patient underwent pancreatoduodenectomy with partial resection of the portal vein. We also review the current literature concerning Peutz-Jeghers syndrome associated with malignant tumors, especially pancreatic cancer. To our knowledge, this is the first report of a Peutz-Jeghers syndrome patient with pancreatic cancer having pancreatoduodenectomy and pathologically diagnosed with invasive ductal adenocarcinoma of the pancreas. The Peutz-Jeghers syndrome patients with pancreatic cancer were relatively young. As the pancreatic cancer in these patients was advanced and most were unresectable at diagnosis, the prognoses of these patients were extremely poor. Surgical resection offers the only chance for cure or long-term survival for Peutz-Jeghers syndrome patients, if the tumor is localized without distant metastasis. Therefore, screening even for young patients with Peutz-Jeghers syndrome is necessary for early detection of cancer.
我们报告一例47岁患有黑斑息肉综合征（Peutz-Jeghers syndrome）的男性胰腺腺癌罕见病例。该患者接受了胰十二指肠切除术，并部分切除了门静脉。我们还回顾了当前有关与恶性肿瘤相关的黑斑息肉综合征的文献，尤其是胰腺癌。据我们所知，这是首例黑斑息肉综合征合并胰腺癌患者接受胰十二指肠切除术并经病理诊断为胰腺浸润性导管腺癌的报告。患有胰腺癌的黑斑息肉综合征患者相对年轻。由于这些患者的胰腺癌已属晚期，且大多数在诊断时无法切除，这些患者的预后极差。如果肿瘤局限且无远处转移，手术切除是黑斑息肉综合征患者获得治愈或长期生存的唯一机会。因此，即使是年轻的黑斑息肉综合征患者也有必要进行筛查以早期发现癌症。
