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Gastroenterology > ZES > Flashcards

ZES Flashcards

1
Q

What is Zollinger-Ellison Syndrome (ZES)?

A

Severe peptic ulcer diathesis secondary to gastric acid hypersecretion due to unregulated gastrin release from a non–β-cell neuroendocrine tumor (NET; gastrinoma)

Initially typified by aggressive and refractory ulceration requiring total gastrectomy for survival enhancement.

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2
Q

What percentage of patients with sporadic ZES can be cured by surgical resection?

A

Up to 40%

This is significant compared to earlier treatment approaches.

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3
Q

At what ages are most patients diagnosed with ZES?

A

Between ages 30 and 50

Females are slightly more commonly affected than males.

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4
Q

What are the two classifications of gastrinomas?

A

Sporadic tumors and those associated with multiple endocrine neoplasia (MEN) type 1

Sporadic tumors account for 80% of gastrinomas.

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5
Q

What has contributed to a delay in the diagnosis of ZES?

A

Widespread availability and use of PPIs

This has led to decreased referrals for gastrinoma evaluation and increased false-positive diagnoses.

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6
Q

How does gastrin stimulate acid secretion?

A

Through gastrin receptors on parietal cells and by inducing histamine release from ECL cells

Gastrin also has a trophic action on gastric epithelial cells.

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7
Q

What are the clinical manifestations of longstanding hypergastrinemia in ZES?

A

Increased gastric acid secretion, peptic ulcer diathesis, erosive esophagitis, and diarrhea

Markedly increased gastric acid output occurs due to parietal cell stimulation.

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8
Q

Fill in the blank: ZES is characterized by __________ due to unregulated gastrin release.

A

gastric acid hypersecretion

This is primarily due to gastrinomas.

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9
Q

What is the primary location of gastrinomas based on early studies?

A

The vast majority occurred within the pancreas

However, a significant number of these lesions are extrapancreatic.

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10
Q

What is the gastrinoma triangle?

A

Confluence of the cystic and common bile ducts superiorly, junction of the second and third portions of the duodenum inferiorly, and junction of the neck and body of the pancreas medially

This area is where 60 to 90% of gastrinomas are found.

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11
Q

Where are the most common nonpancreatic gastrinomas located?

A

Duodenum

Between 60 and 100% of gastrinomas are found here.

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12
Q

How do duodenal tumors compare to pancreatic lesions in terms of growth and metastasis?

A

Duodenal tumors are smaller, slower growing, and less likely to metastasize

This contrasts with pancreatic lesions.

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13
Q

List some less common extrapancreatic sites for gastrinomas.

A
  • Stomach
  • Bones
  • Ovaries
  • Heart
  • Liver
  • Lymph nodes
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14
Q

What percentage of gastrinomas are considered malignant?

A

More than 60%

Up to 30–50% of patients may have multiple lesions or metastatic disease at presentation.

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15
Q

What is the histological grade of gastrin-producing cells in gastrinomas?

A

Grade 1 or 2

These cells express markers typically found in endocrine neoplasms.

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16
Q

What markers do gastrin-producing cells express?

A
  • Chromogranin
  • Neuron-specific enolase
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17
Q

True or False: Histologic grade in pancreatic NETs is generally considered an important predictor of survival.

A

True

Although not clearly established in gastrinomas.

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18
Q

What is the most common clinical presentation for gastrinoma patients?

A

Abdominal pain in the presence of acid peptic disorders

Peptic ulcer is the most common clinical manifestation, occurring in >90% of gastrinoma patients.

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19
Q

What are clinical situations that should create suspicion of gastrinoma?

A

Ulcers in unusual locations, ulcers refractory to standard medical therapy, ulcer recurrence after acid-reducing surgery, ulcers presenting with complications, ulcers in absence of H. pylori or NSAID ingestion

Unusual locations include the second part of the duodenum and beyond.

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20
Q

What percentage of patients with ZES experience symptoms of esophageal origin?

A

Up to two-thirds

Symptoms can range from mild esophagitis to frank ulceration with stricture and Barrett’s mucosa.

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21
Q

What is the next most common clinical manifestation after abdominal pain in gastrinoma patients?

A

Diarrhea

Found in up to 70% of patients.

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22
Q

What factors contribute to the etiology of diarrhea in gastrinoma patients?

A

Marked volume overload to the small bowel, pancreatic enzyme inactivation by acid, damage to the intestinal epithelial surface by acid

This damage can lead to mild maldigestion and malabsorption.

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23
Q

What is the role of gastrin in relation to diarrhea in gastrinoma patients?

A

Gastrin has a direct stimulatory effect on enterocytes

Additional hormones from the tumor, such as vasoactive intestinal peptide, may also contribute.

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24
Q

What percentage of gastrinoma patients may develop gastrinomas in the presence of MEN 1 syndrome?

A

Approximately 25%

MEN 1 syndrome is an autosomal dominant disorder affecting parathyroid glands, pancreas, and pituitary gland.

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25
Q

What gene is associated with MEN 1 syndrome?

A

MEN1 tumor suppressor gene

Found on the long arm of chromosome 11q13.

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26
Q

What role does the MEN1 gene play in the body?

A

Encodes for menin, which is important in DNA replication and transcriptional regulation.

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27
Q

What determines a clinical diagnosis of MEN 1?

A

Tumors in two of the three endocrine organs or family history of MEN 1 with one endocrine organ tumor.

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28
Q

What effect does hyperparathyroidism and hypercalcemia have on ulcer disease in MEN 1 patients?

A

May have a direct effect on ulcer disease

Resolution of hypercalcemia by parathyroidectomy reduces gastrin and gastric acid output.

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29
Q

What distinguishing feature is observed in ZES patients with MEN 1 compared to sporadic gastrinoma patients?

A

Higher incidence of gastric carcinoid tumor development

ZES presents and is diagnosed earlier in MEN 1 patients.

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30
Q

What is the recommended approach for screening gastrinoma patients for MEN 1?

A

Detailed family history and obtaining serum markers including calcium, parathyroid, prolactin, and pancreatic polypeptide levels.

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31
Q

Why is establishing an early diagnosis of ZES important?

A

To minimize long-term sequelae of gastric acid hypersecretion, prevent metastatic disease, and counsel family members if MEN 1 is diagnosed

MEN 1 refers to Multiple Endocrine Neoplasia type 1, a genetic condition that can lead to gastrin-secreting tumors.

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32
Q

What role do biochemical measurements of gastrin and acid secretion play in ZES diagnosis?

A

They are critical in establishing the diagnosis of this rare condition.

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33
Q

What is the first step in evaluating a patient suspected of having ZES?

A

Obtain a fasting gastrin level.

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34
Q

What fasting gastrin level is usually considered normal?

A

<150 pg/mL.

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35
Q

What fasting gastrin level virtually excludes the diagnosis of ZES?

A

A normal fasting gastrin on two separate occasions, especially if the patient is on a PPI.

36
Q

What fasting gastrin level is indicative of gastrinoma?

A

> 150–200 pg/mL.

37
Q

What should be done if a patient is suspected of having ZES?

A

Repeat measurement of fasting gastrin to confirm clinical suspicion.

38
Q

What can lead to false-positive or false-negative fasting gastrin levels?

A

Variable specificity of the antibodies used in commercial biochemical assays.

39
Q

What are common causes of elevated fasting gastrin levels?

A

Gastric hypochlorhydria and achlorhydria, with or without pernicious anemia.

40
Q

What effect does gastric acid have on gastrin release?

A

Gastric acid induces feedback inhibition of gastrin release.

41
Q

List additional causes of elevated gastrin levels.

A
  • Retained gastric antrum
  • G-cell hyperplasia
  • Gastric outlet obstruction
  • Renal insufficiency
  • Massive small-bowel obstruction
  • Conditions like rheumatoid arthritis, vitiligo, diabetes mellitus, and pheochromocytoma.
42
Q

What fasting gastrin level is highly suggestive of ZES?

A

> 10 times normal.

43
Q

What should be done before testing gastrin levels after stopping a PPI?

A

Stop the PPI for a minimum of 7 days.

44
Q

What medication can be used during the PPI cessation period?

A

Histamine H2 antagonist, such as famotidine.

45
Q

What pH level in gastric fluid is suggestive of gastrinoma?

A

<3.

46
Q

What is considered pathognomonic of ZES regarding BAO?

A

A BAO >15 meq/h in the presence of hypergastrinemia.

47
Q

What ratio is highly suggestive of ZES?

A

BAO/MAO ratio >0.6.

48
Q

What is the most sensitive and specific gastrin provocative test?

A

The secretin stimulation test.

49
Q

What increase in gastrin after secretin injection indicates ZES?

A

An increase of ≥120 pg within 15 min.

50
Q

What must be done before conducting the secretin test?

A

Stop PPI for 1 week.

51
Q

What is the revised approach to diagnosing gastrinoma?

A

Based on elevated gastrin, low gastric pH, clinical setting, tumor localization tests, and positive histology by biopsy.

52
Q

What is the first step after confirming a biochemical diagnosis of gastrinoma?

A

The tumor must be located

53
Q

What imaging modality provides high-resolution imaging of the pancreas?

A

Endoscopic ultrasound (EUS)

54
Q

What is the sensitivity of EUS for finding duodenal lesions?

A

43%

55
Q

Why is EUS not routinely included in the preoperative evaluation for gastrinoma?

A

It is not very sensitive for finding duodenal lesions

56
Q

What subtype of somatostatin receptor is particularly relevant for endocrine tumors?

A

Subtype 2 (SSTR2)

57
Q

What is the sensitivity and specificity of OctreoScan for gastrinomas?

A

> 80%

58
Q

What imaging study is superior to OctreoScan for assessing tumor presence in well-differentiated NETs?

A

Positron emission tomography (PET)–computed tomography (CT) with 68 Ga-DOTATATE

59
Q

What are the sensitivity and specificity rates of 68 Ga-DOTATATE for gastrinomas?

A

> 90%

60
Q

What imaging method has been found useful in pancreatic NETs, including gastrinomas?

A

F-Fluordeoxyglucose (18 F-FDG) PET imaging

61
Q

What percentage of patients have metastatic disease at the time of gastrinoma diagnosis?

A

Up to 50%

62
Q

What initial imaging should a patient undergo to exclude metastatic disease after a biochemical diagnosis of gastrinoma?

A

Abdominal CT scan, MRI, or OctreoScan/PET-CT with 68 Ga-DOTATATE

63
Q

What may an experienced endocrine surgeon opt for after excluding metastatic disease?

A

Exploratory laparotomy with intraoperative ultrasound or transillumination

64
Q

What technique may be used for localizing tumors in a subset of gastrinoma patients?

A

Selective arterial secretin injection

65
Q

What is the primary focus of treatment for Zollinger-Ellison Syndrome?

A

Ameliorating signs and symptoms related to hormone overproduction, curative resection of the neoplasm, and attempts to control tumor growth in metastatic disease.

66
Q

What class of drugs is the treatment of choice for Zollinger-Ellison Syndrome?

A

PPIs (Proton Pump Inhibitors)

67
Q

What is the initial dose range for PPIs in Zollinger-Ellison Syndrome treatment?

A

60 mg in divided doses in a 24-h period.

68
Q

What BAO levels are targeted in surgery-naive patients undergoing treatment?

A

<10 meq/h at the drug trough.

69
Q

What BAO levels are targeted in patients who have previously undergone an acid-reducing operation?

A

<5 meq/h.

70
Q

What role do somatostatin analogues play in treatment?

A

They have inhibitory effects on gastrin release and gastric acid secretion.

71
Q

When might octreotide or lanreotide be considered in treatment?

A

As adjunctive therapy to the PPI in patients with tumors expressing somatostatin receptors.

72
Q

What is the ultimate goal of surgery in Zollinger-Ellison Syndrome?

A

To provide a definitive cure.

73
Q

What factors influence the decision to perform surgery on MEN 1 patients?

A

Presence of a clearly identifiable, nonmetastatic lesion.

74
Q

What are the challenges associated with laparoscopic surgical interventions for gastrinoma?

A

Significant percentage of tumors may be extrapancreatic and difficult to localize.

75
Q

What is the general prognosis for metastatic endocrine tumors?

A

Suboptimal, including gastrinomas.

76
Q

When is systemic tumor-targeted therapy recommended in metastatic gastrinoma?

A

Until evidence of tumor progression or refractory symptoms not controlled with PPIs.

77
Q

What are some medical approaches for treating metastatic gastrinomas?

A
  • Biologic therapy (IFN-α, long-acting somatostatin analogues, peptide receptor radionuclides)
  • Systemic chemotherapy (streptozotocin, 5-fluorouracil, doxorubicin)
  • Hepatic artery embolization
78
Q

What combination therapy has shown radiographic regression and progression-free survival in NETs?

A

Temozolomide with capecitabine.

79
Q

What is the overall 5-year survival rate for gastrinoma patients?

A

62–75%.

80
Q

What is the overall 10-year survival rate for gastrinoma patients?

A

47–53%.

81
Q

What survival rates do patients with completely resected tumors or negative laparotomy have?

A

> 90% for both 5- and 10-year survival.

82
Q

What is the survival rate for patients with incompletely resected tumors?

A

43% at 5 years and 25% at 10 years.

83
Q

What is the survival rate for patients with hepatic metastasis?

A

<20% at 5 years.

84
Q

What are some favorable prognostic indicators for gastrinoma patients?

A
  • Primary duodenal wall tumors
  • Isolated lymph node tumor
  • Presence of MEN 1
  • Undetectable tumor upon surgical exploration
85
Q

What factors are associated with poor outcomes in gastrinoma patients?

A
  • Shorter disease duration
  • Female sex
  • Older age at diagnosis
  • Higher gastrin levels (>10,000 pg/mL)
  • Poor histologic differentiation
  • High proliferative index
  • Large pancreatic primary tumors (>3 cm)
  • Metastatic disease to lymph nodes, liver, and bone
  • Cushing’s syndrome
86
Q

True or False: The clinical course of MEN 1 patients tends to be aggressive and leads to disease-related mortality.

A

False

Gastroenterology (14 decks)

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