Zollinger-Ellison syndrome(Gastrinoma)
1. ¿øÀÎ ¹× º´ÀÎ
pancreas head¿¡ ÁÖ·Î ¹ß»ýÇϸç 1mm-20cmÀÌ»ó±îÁö ´Ù¾çÇÏ´Ù
1/2-1/3ÀÌ multiple gastrinomaÀ̸ç pancreas»Ó¸¸ À̳ª¶ó duodenal wall¿¡¼µµ ÈçÈ÷
¹ß°ßµÇ´Âµ¥ duodenal gastrinoma´Â ¹Ý¼ö¿¡¼ ´ÜÀÏÁ¾±«À̸ç duodenumÀÇ 1st, 2nd partÀÇ
submucosa¿¡ ÈçÈ÷ ¹ß»ýÇÑ´Ù.
90%°¡ anatomic triangle(gastrinoma triangle)³»¿¡ ¹ß°ßµÇ´Âµ¥ ±× °æ°è´Â ´ÙÀ½°ú °°´Ù.
superiorly : cystic duct¿Í CBD junction
inferiorly : duodenum 2nd, 3rd portionÀÇ junction
medially : pancrea body & neck junction
2/3 : malignant (±×·¯³ª ¼¼È÷ ÀÚ¶õ´Ù)
¹ßÇö½Ã 1/3¿¡¼ metastatic disease(ÁÖ·Î regional LN, liver)
20-60% : MEN 1 syndromeÀÇ ÇÑ ±¸¼º¿ä¼Ò·Î¼ »ý±ä´Ù.
cf. MEN 1 syndrome : parathyroid hyperplasia, pituitary, pancreas islet)
MEN 1ÀÇ 90%¿¡¼ hyperparathyroidismÀ» º¸ÀÌ¸ç ¹Ý¼ö¿¡¼ gastrinoma°¡ µ¿¹ÝµÈ´Ù.
gastrin-secreting islet cell tumor´Â multiple hormoneÀ» ÇÔÀ¯Çϴµ¥ ÈçÈ÷ clinically silent
Çϸç ÀÌÁß °¡Àå ÈçÇÑ °ÍÀº ACTH(1/3)ÀÌ´Ù.
2. ÀÓ»óÀû Ư¡
peptic ulcerÀÇ 0.1-1%À» Â÷ÁöÇϸç 30-60¼¼¿¡ È£¹ßÇÑ´Ù.
ulcer¿¡ H.pylori´Â ÈçÈ÷ (-)
1) duodenumÀÇ 1st portion°ú stomach¿¡ ulcer¸¦ º¸À̸ç(75%¿¡¼)
ÈçÈ÷ singleÀÌ´Ù. (multipleÀϼöµµ ÀÖ´Ù)
2) diarrhea(40%)
´ë·®ÀÇ HClÀÌ proximal duodenumÀ¸·Î ¹æÃâµÊÀ¸·Î½á ¼ÒÀåÁ¡¸·¿¡ ºÎ½Ä¼º º¯È¸¦ ÀÏÀ¸ÄÑ
¹ß»ýÇÑ´Ù.
3) steatorrhea : ¼³»çº¸´Ù´Â ´ú ÈçÇÏ´Ù.
pancreatic lipase°¡ inactivationµÇ°í luminal bile acid°¡ °¨¼ÒÇÔÀ¸·Î½á
micelleÇü¼º Àå¾Ö·Î Àå³» Áö¹æÈí¼öÀÇ Àå¾Ö°¡ »ý°Ü¼ ¹ß»ýÇÑ´Ù.
3. Áø´Ü
BAO > 15 mEq/h(90%)
BAO/MAO > 60% (normal <60%) - ±×·¯³ª ÀÌ°Í ´Üµ¶À¸·Ð Àǹ̰¡ ¾ø´Ù.
1) fasting serum gastrin level > 200 ng/L(1000ÀÌ»óÀº Àß ¾È¿Ã¶ó°£´Ù)
2) provocation test
¨ç secretin injection test - most valuable, 95%¿¡¼ (+)
2 U/kg IVÈÄ 2, 5, 10, 15, 20, 25, 30ºÐ¿¡ sample
10ºÐ³»¿¡ 200 ng/LÀÌ»óÀ¸¸é ¾ç¼º
¨è calcium infusion test
¨é feeding of a standard meal
3) localization
¨ç CT : 30%
¨è USG : 20%
¨é hepatic venous gastrin sampling after IA secretin injection
- duodenal gastrinoma locaization
¨ê radiolabeled octreotide scan : useful, ÀÛÀº primary or metastatic tumorã´Âµ¥ À¯¿ë
4. Ä¡·á
1) complete surgical resection of tumor
2) total gastrectomy - gastric surgery°¡ ¿ä±¸µÇ´Â °æ¿ì procedure of choice
3) H2 blocker: gastric acid¸¦ °¨¼Ò½Ã۴µ¥ È¿°úÀûÀ̸ç Áõ»óÀ» È£Àü½Ã۰í ulcer healing
½ÃŲ´Ù(80-85%).
4) omeprazole & lansoprazole - most effective drug
60mg qd ½ÄÀü
¼ö¼úÀ» ÇÒ¼ö¾ø°Å³ª °ÅºÎÇÒ ¶§, metastatic, nonresectable gastrinomaÀÏ ¶§ »ç¿ë
5) CTx : limited
6) Interferon & octreotide : limited
* gastrectomy°¡ tumor progression¿¡´Â ¿µÇâÀ» ¹ÌÄ¡Áö ¾Ê´Â´Ù.
Antral G cell hyperplasia
secretin injection test¿¡¼ ZES¿¡¼ Ư¡ÀûÀ¸·Î º¸ÀÌ´Â serum gastrinÀÇ Å« Áõ°¡°¡
º¸ÀÌÁö ¾Ê´Â´Ù.