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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.