Publication: Bulky gastrinoma of the common bile duct: Unusual localization of extrapancreatic gastrinoma case report
Abstract
Zollinger-Ellison Sendromu serum gastrin düzeyinde yükseklik, artmış mide asiti, yaygın gastrointestinal ülserasyonlar ve ishal ile karakterizedir. Gastrinomalar sıklıkla (%75) sporadik olarak görülür ve gastrinoma üçgeninde yerleşirler. Cerrahiye gönderilen Zollinger-Ellison Sendromu hastalarının %5.6 kadarı ekstraduodenal, ekstrapankreatik ve ekstranodal gastrinomadır. Hastamız 44 yaşında kadın, 12 yıldır bulantı ve diyare şikâyetleri mevcuttu. Abdominal bilgisayarlı tomografi ve manyetik rezonans görüntülemede homojen, düzgün sınırlı, 6x7 cm solid kitle, karaciğer-sağ böbrek ve inferior vena cava arasında yerleşmiş olduğu görüldü. Somatostatin reseptör pozitişiği bu kitlenin nöroendokrin tümör olduğunu düşündürdü. Hastanın serum gastrin düzeyi çok yüksek bulundu ve hasta cerrahiye verildi. Cerrahi sırasında kitlenin 8x6x5 cm boyutlarında iyi kapsüllü ve koledoktan köken aldığı görüldü. Bu vakanın özelliği çok nadir görülen bir yerleşimde koledoktan köken almış olması ve çok büyük olduğu halde kapsül invazyonu ve metastaz yapmamış olmasıdır.
Zollinger-Ellison syndrome is characterized by elevated levels of serum gastrin associated with increased gastric acid secretion, gastrointestinal ulcerations and diarrhea. Most gastrinomas (75%) occur sporadically and are located within the gastrinoma triangle. Extraduodenal, extrapancreatic and extranodal gastrinomas have been shown in 5.6% of the patients with Zollinger-Ellison syndrome who underwent surgery. We report a 44-year-old female who had been complaining of nausea and diarrhea for 12 years. Abdominal computed tomography and magnetic resonance imaging detected a homogeneous, regular-shaped 6 x 7 cm solid mass, located between the liver, right kidney and inferior vena cava. Somatostatin receptor positive scanning led us to investigate a neuroendocrine tumor. Serum gastrin level was found very high and the patient underwent surgery. Local excision of the tumor was performed, and an 8x6x5 cm, well-capsulated, solitary mass, originating from the common bile duct was removed. The unique feature of our case is the unusual localization, and although the tumor was very large in size, only capsule invasion was observed with no evidence of distant metastasis.
Zollinger-Ellison syndrome is characterized by elevated levels of serum gastrin associated with increased gastric acid secretion, gastrointestinal ulcerations and diarrhea. Most gastrinomas (75%) occur sporadically and are located within the gastrinoma triangle. Extraduodenal, extrapancreatic and extranodal gastrinomas have been shown in 5.6% of the patients with Zollinger-Ellison syndrome who underwent surgery. We report a 44-year-old female who had been complaining of nausea and diarrhea for 12 years. Abdominal computed tomography and magnetic resonance imaging detected a homogeneous, regular-shaped 6 x 7 cm solid mass, located between the liver, right kidney and inferior vena cava. Somatostatin receptor positive scanning led us to investigate a neuroendocrine tumor. Serum gastrin level was found very high and the patient underwent surgery. Local excision of the tumor was performed, and an 8x6x5 cm, well-capsulated, solitary mass, originating from the common bile duct was removed. The unique feature of our case is the unusual localization, and although the tumor was very large in size, only capsule invasion was observed with no evidence of distant metastasis.