Zhao Zitong, Sim Chiang Khi, Mantoo Sangeeta
Department of Anatomical Pathology, Division of Pathology, Singapore General Hospital, Singapore, Singapore.
Pediatric Surgery, Mount Elizabeth Medical Centre, Singapore, Singapore.
Diagn Pathol. 2017 Jul 5;12(1):49. doi: 10.1186/s13000-017-0643-2.
Heterotopic pancreas most commonly occurs in the upper gastrointestinal tract of adults, usually as an incidental finding. It seldom occurs at the umbilicus, and even rarely in the pediatric age group.
Here we present a case of heterotopic pancreatic tissue in the omphalomesenteric duct remnant of a 9-month-old baby girl. She presented with redness at the base of the umbilicus associated with occasional mild wetness. A urachal fistula was suspected by ultrasound. Histology from subsequent resection revealed fibrous tissue with heterotopic pancreatic tissue and accompanying small bowel mucosa. The patient's umbilical redness resolved after the surgery.
Upon literature search, we found only 17 other cases of heterotopic pancreas reported in the umbilicus. They described a high male to female ratio, frequent association with omphalomesenteric duct remnant and presentation of umbilical discharge. The Heinrich system is frequently used to classify heterotopic pancreas into 3 types, based on the presence of acini, islets and ducts. Several mechanisms have been proposed on the pathogenesis of heterotopic pancreas, including misplacement, metaplasia and totipotent cell theories. Heterotopic pancreas can manifest clinically with diseases of the pancreas, including malignant transformation, reported as high as 12.7% in a series. Awareness of this finding in the biopsy aids the suitable treatment decisions for the patient.
异位胰腺最常见于成人上消化道,通常为偶然发现。很少发生于脐部,在儿童年龄组中更为罕见。
在此,我们报告一例9个月大女婴卵黄管残余处的异位胰腺组织病例。她表现为脐根部发红,伴有偶尔轻度潮湿。超声怀疑为脐尿管瘘。后续切除组织的组织学检查显示为纤维组织,伴有异位胰腺组织及附带的小肠黏膜。术后患儿脐部发红消退。
经文献检索,我们仅发现另外17例脐部异位胰腺的病例报告。这些报告描述了男性与女性的高比例、与卵黄管残余的频繁关联以及脐部排出物的表现。海因里希系统常根据腺泡、胰岛和导管的存在情况将异位胰腺分为3型。关于异位胰腺的发病机制已提出多种学说,包括错位、化生和全能细胞学说。异位胰腺可在临床上表现为胰腺疾病,包括恶性转化,在一组病例中报告的发生率高达12.7%。活检时认识到这一发现有助于为患者做出合适的治疗决策。