Erotokritou A, Gerharz C D, Sagir A
Department of Gastroenterology, Academic Teaching Hospital Bethesda Duisburg, Heerstr, 219 47053, Duisburg, Germany.
Department of Pathology, Academic Teaching Hospital Bethesda Duisburg, Heerstr. 219, 47053, Duisburg, Germany.
J Med Case Rep. 2018 Jun 30;12(1):185. doi: 10.1186/s13256-018-1733-9.
Agenesis of the dorsal pancreas is very rare. Less than 70 cases have been reported to date. Some of these cases had an association with a tumor. The literature of agenesis of the dorsal pancreas and agenesis of the dorsal pancreas-associated pancreatic neoplasia is limited. Here we report the second case of a pancreatic neuroendocrine tumor in a setting of agenesis of the dorsal pancreas.
A 71-year-old man, originally from North Africa, with a history of insulin-dependent diabetes mellitus, presented with a 2-month history of nonspecific abdominal symptoms. Contrast-enhanced computed tomography demonstrated an almost 3 cm round, quite well-defined and homogeneous tumor formation in the area between the neck and absent body and tail of his pancreas. The mass was confirmed by endoscopic ultrasound. Our patient underwent computed tomography-guided biopsy of the mass which provided proof of a neuroendocrine tumor. He underwent pancreas resection because of the presence of a neuroendocrine tumor. Seven months later his glycated hemoglobin increased from 6.9 to 8.7%.
Diagnosis of agenesis of the dorsal pancreas is based on imaging techniques like computed tomography, magnetic resonance cholangiopancreatography, or endoscopic ultrasound. Endoscopic ultrasound-guided fine-needle aspiration can be helpful for the histological diagnosis of the tumor. The hypothesis of the association between pancreatic neoplasia and agenesis of the dorsal pancreas leads us to the suggestion that every patient with diagnosed agenesis of the dorsal pancreas should be observed with a focus on the early detection of potential malignancy.
背侧胰腺发育不全极为罕见。迄今为止，报道的病例不足70例。其中一些病例与肿瘤有关。关于背侧胰腺发育不全及背侧胰腺发育不全相关胰腺肿瘤的文献有限。在此，我们报告第二例背侧胰腺发育不全合并胰腺神经内分泌肿瘤的病例。
一名71岁男性，原籍北非，有胰岛素依赖型糖尿病病史，出现非特异性腹部症状2个月。增强计算机断层扫描显示，在其胰腺颈部与缺如的体部和尾部之间区域有一个近3厘米的圆形、边界相当清晰且均匀的肿瘤形成。通过内镜超声证实了该肿块。我们的患者接受了计算机断层扫描引导下的肿块活检，证实为神经内分泌肿瘤。由于存在神经内分泌肿瘤，他接受了胰腺切除术。七个月后，他的糖化血红蛋白从6.9%升至8.7%。
背侧胰腺发育不全的诊断基于计算机断层扫描、磁共振胰胆管造影或内镜超声等成像技术。内镜超声引导下细针穿刺有助于肿瘤的组织学诊断。胰腺肿瘤与背侧胰腺发育不全之间存在关联的假说使我们建议，对于每例诊断为背侧胰腺发育不全的患者，应重点观察以早期发现潜在恶性病变。
