Rodrigues Pedro, Oliveira Rui Caetano, Oliveira Carlos Miguel, Cipriano Maria Augusta
Pathology, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.
Pathology, Hospitais da Universidade de Coimbra, Coimbra, Portugal.
BMJ Case Rep. 2017 Oct 23;2017:bcr-2017-221999. doi: 10.1136/bcr-2017-221999.
Pancreatic dorsal agenesis (PDA) is a rare congenital anomaly, usually asymptomatic, that can present with abdominal pain, pancreatitis, diabetes mellitus and jaundice. Pancreatic tumours in PDA background are extremely rare, and when they occur are mainly pancreatic ductal adenocarcinoma. We present a case of a 48-year-old female patient with incidental detection of a 26×20 mm nodular lesion of the cephalic pancreas on ultrasound. Surgery was performed and gross examination revealed PDA with a tumour developed around the Wirsung duct. Histology showed a neuroendocrine tumour G1 with neural and vascular invasion. Two and half years later, the patient is alive and without tumour relapse. Awareness of the association of PDA and pancreatic tumours is fundamental in order to develop personalised follow-up programmes.
胰腺背侧发育不全（PDA）是一种罕见的先天性异常，通常无症状，可表现为腹痛、胰腺炎、糖尿病和黄疸。PDA背景下的胰腺肿瘤极为罕见，一旦发生，主要是胰腺导管腺癌。我们报告一例48岁女性患者，超声偶然发现胰头有一个26×20毫米的结节性病变。进行了手术，大体检查显示为PDA，在主胰管周围有一个肿瘤。组织学显示为G1级神经内分泌肿瘤，伴有神经和血管侵犯。两年半后，患者存活，无肿瘤复发。认识到PDA与胰腺肿瘤的关联对于制定个性化的随访计划至关重要。
