Fassari Alessia, Rosso Edoardo, Anafack Jyoti Lakshmi, De Blasi Vito
Department of General Surgery, Centre Hospitalier de Luxembourg, Luxembourg, Luxembourg.
Centre de Chirurgie Digestive, Pôle Santé Sud, Le Mans, France.
Am J Case Rep. 2025 Apr 25;26:e947512. doi: 10.12659/AJCR.947512.
BACKGROUND Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly resulting from incomplete development of the dorsal pancreatic bud, affecting the body and tail of the pancreas. While often asymptomatic, it can present with clinical abnormalities such as abdominal pain, diabetes, or obstructive jaundice, and may be associated with chronic pancreatitis and tumors. We report the third documented case of a high-grade neuroendocrine tumor located at the head of the pancreas in a patient with complete ADP, along with a review of the literature. CASE REPORT An 86-year-old man presented with jaundice caused by a 3-cm pancreatic mass compressing the bile duct, confirmed through imaging to be associated with ADP. Surgical intervention was declined, and a combined laparoscopic and radiological approach was used to place a biliary stent, alleviating symptoms. Histopathological analysis confirmed a poorly differentiated neuroendocrine carcinoma (NEC). Despite initial symptomatic relief, the patient died due to complications 3 months later. CONCLUSIONS This report underscores the diagnostic challenges of ADP and its potential association with pancreatic neoplasms. Improved imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS) facilitate diagnosis and differentiation from similar conditions. The association between ADP and pancreatic tumors should warn us to be watchful with every patient diagnosed with ADP and focus on the early detection of possible malignancies. Further research is needed to clarify the genetic and developmental mechanisms linking ADP to tumorigenesis and to explore targeted therapeutic approaches.
背景 背侧胰腺发育不全(ADP)是一种罕见的先天性异常,由背侧胰芽发育不完全所致,影响胰腺的体部和尾部。虽然通常无症状,但可出现腹痛、糖尿病或梗阻性黄疸等临床异常,且可能与慢性胰腺炎和肿瘤有关。我们报告了第三例记录在案的完全性ADP患者胰腺头部发生高级别神经内分泌肿瘤的病例,并对相关文献进行了综述。
病例报告 一名86岁男性因3厘米胰腺肿块压迫胆管导致黄疸就诊,影像学检查证实与ADP相关。患者拒绝手术干预,采用腹腔镜和放射学联合方法置入胆管支架,缓解了症状。组织病理学分析证实为低分化神经内分泌癌(NEC)。尽管最初症状缓解,但患者3个月后因并发症死亡。
结论 本报告强调了ADP的诊断挑战及其与胰腺肿瘤的潜在关联。计算机断层扫描(CT)、磁共振成像(MRI)和内镜超声(EUS)等改进的成像技术有助于诊断并与类似疾病相鉴别。ADP与胰腺肿瘤之间的关联应提醒我们,对每例诊断为ADP的患者都要保持警惕,关注可能恶性肿瘤的早期发现。需要进一步研究以阐明将ADP与肿瘤发生联系起来的遗传和发育机制,并探索靶向治疗方法。