case presentation - university of colorado denve · case presentation endoscopic image of tumor,...

Case Presentation

Amid Keshavarzi, MDUCHCS

March, 12, 2007

Case PresentationCase Presentation

19 19 y/oy/o male, presented to ER with one week of male, presented to ER with one week of abdominal painabdominal pain

Pain started in upper abdomen then localized to RLQ, Pain started in upper abdomen then localized to RLQ, nausea, vomiting and diarrhea, subjective fevers and nausea, vomiting and diarrhea, subjective fevers and chills, WBC=17,400. chills, WBC=17,400.

Abdominal exam: Peritoneal signsAbdominal exam: Peritoneal signs

CT CT Free fluid and air, diffuse Free fluid and air, diffuse jejunaljejunal wall wall thickening, mesenteric thickening, mesenteric lymphadenopathylymphadenopathy, appendix , appendix was not visualized.was not visualized.


Operating room (10/17/06): Operating room (10/17/06): Open appendectomy : Open appendectomy :

SuppurotiveSuppurotive appendicitisappendicitis-- ((Path: Acute Path: Acute periappendicitisperiappendicitis))

Worsening of abdominal pain on evening Worsening of abdominal pain on evening of POD #1.of POD #1.

Diffuse peritonitis on POD #2Diffuse peritonitis on POD #2

Case PresentationCase PresentationOperating room (10/19/06):Operating room (10/19/06):

-- IO findings:IO findings: JejunalJejunal perforations with diffuse mesenteric perforations with diffuse mesenteric lymphadenopathylymphadenopathy

-- JejunalJejunal resection resection -- 3.5 x 4 cm LN from greater curvature3.5 x 4 cm LN from greater curvature-- IO Serum Gastrin level IO Serum Gastrin level 3538 3538 [136[136--1490 pg/ml]1490 pg/ml]-- Path: Path: TransmuralTransmural inflammation, 12 inflammation, 12 LNsLNs negative for malignancynegative for malignancy

Greater curvature LN: Filled with NE tumorGreater curvature LN: Filled with NE tumor

Work up for MENWork up for MEN--I:I:Serum Ca, PTH, Serum Ca, PTH, ProlactinProlactin

Octreotide scan: Octreotide scan: Focus of increase uptake in Focus of increase uptake in RUQ (3 x 2 cm)RUQ (3 x 2 cm)

Serum Gastrin level:Serum Gastrin level:154 154 pg/mlpg/ml

EGD + EUSEGD + EUSBxBx of of submucosalsubmucosal massmassSuboptimal EUSSuboptimal EUS


Endoscopic Image of tumor, located in the antrum of stomach.

Operating room (1/9/07):Operating room (1/9/07):1)1) LOALOA2)2) TruncalTruncal vagotomyvagotomy with with antralantral resection and resection and

BillrothBillroth I anastomosisI anastomosis3)3) Multiple Multiple serosalserosal and mesenteric biopsiesand mesenteric biopsies4)4) IntraoperativeIntraoperative US of liver and pancreasUS of liver and pancreas


Pathology:Pathology:4 mm 4 mm submucosalsubmucosal NE tumor w/o aggressive NE tumor w/o aggressive behavior behavior HypertrophicHypertrophic gastropathygastropathy and chronic gastritisand chronic gastritisOne One perigastricperigastric LN consistent with LN LN consistent with LN metastasismetastasis


ZollingerZollinger--Ellison Ellison SyndromeSyndrome((GastrinomaGastrinoma))

Historical Facts:Historical Facts:It was described by It was described by ZollingerZollinger and Ellison and Ellison (1955)(1955)OberhelmanOberhelman (1972)(1972)

Etiology:Etiology:NeuroendocrineNeuroendocrine tumortumorNonNon--beta Cells beta Cells Gastrin productionGastrin production

Incidence of ZES:Incidence of ZES:UnknownUnknown0.1% or more of cases of peptic ulcer disease. 0.1% or more of cases of peptic ulcer disease.

Sex & Age: Sex & Age: M/F ratios from 1.5:1 to 2:1. 30M/F ratios from 1.5:1 to 2:1. 30--50 year 50 year old. old.

Mortality/Morbidity: Mortality/Morbidity: Depend on the size and the occurrence of tumor Depend on the size and the occurrence of tumor metastasis. metastasis.

GastrinomaGastrinoma (ZES)(ZES)


Gastrin stimulates the acidGastrin stimulates the acid--secreting cells (secreting cells (FundicFundicparietal cells) parietal cells) increase in acid secretion increase in acid secretion 1) GI1) GImucosal ulceration), 2) Hmucosal ulceration), 2) High secretin level igh secretin level diarrheadiarrhea. .

ZES may occur sporadically (80%) ZES may occur sporadically (80%) or as part of MENor as part of MEN--I (20%). I (20%).

The primary tumor location:The primary tumor location:Duodenum, pancreas, abdominal Duodenum, pancreas, abdominal lymph nodes, lymph nodes, ectopicectopic locations locations ((egeg, stomach, heart, ovary, gall bladder, , stomach, heart, ovary, gall bladder, liver, kidney). liver, kidney).


Symptoms:Symptoms:Dyspepsia (80%)Dyspepsia (80%)GERD (60%)GERD (60%)DysphagiaDysphagia (30%)(30%)Epigastric pain Epigastric pain Diarrhea (40%)Diarrhea (40%)HeartburnHeartburn

Signs:Signs:Peptic ulcer (80Peptic ulcer (80--90%)90%)

Proximal duodenum Proximal duodenum Distal duodenum (14%)Distal duodenum (14%)Jejunum (11%)Jejunum (11%)

Perforated peptic ulcer Perforated peptic ulcer (7(7--10%)10%)GI bleedingGI bleeding

Size: 2Size: 2--9 mm with median of 7.59 mm with median of 7.5

75% located in 75% located in submucosalsubmucosal

LN metastases: 54%LN metastases: 54%

Liver metastases: <1cm Liver metastases: <1cm 4%, 24%, 2--3 cm 3 cm 30%, >3 cm 30%, >3 cm 60%60%


Locations of Locations of GastrinomaGastrinoma

Location of duodenal and pancreatic gastrinomas in 95 patients with ZES without MEN1 found at surgical

explorationNorton JA et al, Surg Onc 12(2003)

DiagnosisDiagnosisBiochemical:Biochemical:

Fasting serum gastrinFasting serum gastrinGastric acid Gastric acid secretorysecretory teststestsProvocative testsProvocative testsSerum calcium level Serum calcium level

Radiological:Radiological:Serotonin receptor Scintigraphy (SRS)Serotonin receptor Scintigraphy (SRS)CT scan/MRICT scan/MRIEndoscopic UltrasoundEndoscopic UltrasoundHepatic venous samplingHepatic venous samplingand intraand intra--arterial injectionarterial injectionof Secretinof Secretin

SurgicalSurgical

Evaluation AlgorithmEvaluation Algorithm

Gastrin LevelGastrin Level

Gastrin Acid Secretary Study (BAO)

Provocation test (Secretin Stimulation Test)

Somatostatin Receptor Scintigraphy (SRS)

CT / MRI

+/- Surgery

RadiologicRadiologic FindingsFindings

RadiologicRadiologic FindingsFindings

Norton JA et al, Surg Onc 12(2003)

Comparison of Comparison of IntraoperativeIntraoperativeDiagnostic TechniquesDiagnostic Techniques


Medical Management Medical Management

Acid Suppression:Acid Suppression:H2 BlockersH2 BlockersPPI PPI OctreotideOctreotide

Chemotherapy (Chemotherapy (ControversialControversial) : ) : In patients with metastatic disease, In patients with metastatic disease, -- StreptozocinStreptozocin + + 55--FluorouracilFluorouracil or or DoxorubicinDoxorubicin + + OmerazoleOmerazole

Surveillance endoscopySurveillance endoscopy

Binstock, A. J. et al. Am. J. Roentgenol. 2001;176:947-951

Pathophysiologic schema for development of different types of gastric

carcinoid tumors

Surgical Surgical ManagementManagement

Indicated for intent to cure , except it is Indicated for intent to cure , except it is contraindicated (e.g. liver metastases, MENcontraindicated (e.g. liver metastases, MEN--I, or I, or medically contraindicated)medically contraindicated)

95% of sporadic 95% of sporadic GastrinomasGastrinomas found at surgery found at surgery with the cure rate of 60with the cure rate of 60--70%70%

Surgery increase the survival in Surgery increase the survival in localizedlocalizedmetastatic liver lesions (80% 5metastatic liver lesions (80% 5--survival rate)survival rate)

Liver metastases is less common in MENLiver metastases is less common in MEN--II

Surgical Surgical ManagementManagementSurgery is indicated in all patients with sporadic disease Surgery is indicated in all patients with sporadic disease and MENand MEN--I with tumor <2cm I with tumor <2cm

Surgical options:Surgical options:DuodenumDuodenum resection with marginresection with marginHead of pancreas Head of pancreas EnucleatingEnucleatingBody and tail of pancreas Body and tail of pancreas Enucleating or formal Enucleating or formal resectionresectionWhipple Whipple Liver wedge/segmental resection or RFALiver wedge/segmental resection or RFA

Role of Role of DuodenotomyDuodenotomy

DuodenotomyDuodenotomy is the most important is the most important surgical intervention: surgical intervention:

increase the surgical cure rate two foldincrease the surgical cure rate two foldIncrease the rate of finding three foldIncrease the rate of finding three foldProlong the survivalProlong the survival

Tumor size is the most common reason of Tumor size is the most common reason of not being able to detect it.not being able to detect it.

Comparison of surgery vs Comparison of surgery vs medical treatmentmedical treatment

Effect of surgery for cure on the development of hepatic Effect of surgery for cure on the development of hepatic metastases and survival in patients with ZES. metastases and survival in patients with ZES.


Post Operative Evaluation and Post Operative Evaluation and ManagementManagement

Prior to discharge: Check at least two fasting gastrin levels Prior to discharge: Check at least two fasting gastrin levels and a secretin provocative test at the time oral gastric and a secretin provocative test at the time oral gastric antisecretoryantisecretory drugs are restarted.drugs are restarted.

Maintained preoperative Maintained preoperative antisecretoryantisecretory treatment and retreatment and re--evaluated at 3evaluated at 3––6 months 6 months postresectionpostresection. .

Fasting gastrin and a secretin test are recommended in yearly Fasting gastrin and a secretin test are recommended in yearly fashion. fashion.

No need for imaging studies (CT, MRI, ultrasound) if the No need for imaging studies (CT, MRI, ultrasound) if the patient is cured at 3patient is cured at 3––6 months. 6 months.

Whether SRS will detect recurrences earlier than biochemical Whether SRS will detect recurrences earlier than biochemical studies is at present unknown. studies is at present unknown.

Biochemical assessment: Fasting gastrin determination and Biochemical assessment: Fasting gastrin determination and secretin test.secretin test.

It is preferable the patient is not taking gastric It is preferable the patient is not taking gastric antisecretoryantisecretory drugs because they can cause drugs because they can cause achlorhydriaachlorhydriaand elevate the fasting gastrin.and elevate the fasting gastrin.

FalseFalse--positive secretin tests can occur in positive secretin tests can occur in achlorhydricachlorhydricpatients. patients.

Even in patients cured of Even in patients cured of postresectionpostresection, some degree of , some degree of gastric acid gastric acid hypersecretionhypersecretion may continue and that a low dose may continue and that a low dose of a gastric of a gastric antisecretoryantisecretory agent may continue to be required. agent may continue to be required.

Whether a prior parietal cell Whether a prior parietal cell vagotomyvagotomy would obviate the need would obviate the need for gastric acid for gastric acid antisecretoryantisecretory treatment in this subgroup of treatment in this subgroup of cured cured hypersecretorshypersecretors is at present unknown. is at present unknown.

Post Operative Evaluation and Post Operative Evaluation and ManagementManagement

ControversiesControversies

Operative role of endoscopic ultrasoundOperative role of endoscopic ultrasound

Role of Whipple resectionRole of Whipple resection

Surgical intervention in patients with MENSurgical intervention in patients with MEN--II

Place of highly selective Place of highly selective vagotomyvagotomy

Use of surgery in patients with advanced, aggressive tumorsUse of surgery in patients with advanced, aggressive tumors

Place of rePlace of re--operation in patients not cured or with relapseoperation in patients not cured or with relapse

Existence of lymph node primary and need for role of routine Existence of lymph node primary and need for role of routine lymph node excision lymph node excision

Questions?Questions?

case presentation - university of colorado denve · case presentation endoscopic image of tumor,...

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