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General Surgery
Endocrine Tumors of the Pancreas
Built from Maingot's Abdominal Operations
What’s inside
11 sections · 145 slides
Overview
- What this topic covers
- Terms used in this topic
- Definition of a pancreatic endocrine tumor
- Functioning and nonfunctioning tumors
- How the diagnosis is established
- Role of surgical resection
Islet structure, cell types and pathology
Where these tumours come from, and how the pathologist recognises them
- Structure of the pancreatic islet
- Islet cell types and the tumors they give rise to
- Where each cell type sits inside the islet
- Cell components of a pancreatic islet
- Distribution of endocrine cells across the gland
- Islet cell types, hormones, syndromes and their distribution
- Islet cell types, hormones, syndromes and their distribution (continued)
- Histologic appearance of pancreatic endocrine tumors
- Normal pancreatic islet and pancreatic endocrine tumor on H and E staining
- Chromogranin and gastrin staining of a pancreatic endocrine tumor
- Defining malignancy and functional status
Historical descriptions and relative frequency
How each syndrome was recognised, and how common each tumour is
- Landmarks in the recognition of islet cell tumors
- First descriptions of islet cell tumors
- Recognition of the glucagonoma and gastrinoma syndromes
- Recognition of the watery diarrhea syndrome
- Radioimmunoassay and the study of these syndromes
- Relative frequency of the tumor types
- Relative incidence of pancreatic endocrine tumors
Genetics and multiple endocrine neoplasia type 1
Why these tumours are not driven by the usual cancer genes, and which families are at risk
- Genetic changes in pancreatic endocrine tumors
- Chromosome losses and what they predict
- Multiple endocrine neoplasia type 1
- Tumors and features of multiple endocrine neoplasia type 1
- Behaviour of MEN1-associated tumors
- Screening and treatment order in MEN1
Insulinoma
The commonest of these tumours, and the one most often cured by a simple operation
- Insulinoma: incidence and patient profile
- 60%
- Location and gross appearance of insulinomas
- Biological behaviour of insulinomas
- How an insulinoma produces hypoglycemia
- Symptoms of hypoglycemia in insulinoma
- Timing of attacks and the eating pattern
- Consequences of chronic hypoglycemia
- Whipple's triad
- Whipple's triad and the fast that precipitates it
- The monitored 72-hour fast
- Results of the fast
- Laboratory thresholds that support insulinoma
- Glucose and insulin during a fast in insulinoma
- Glucose levels before and after removal of an insulinoma
- Conditions that mimic insulinoma
- Provocative testing for insulinoma
Gastrinoma and Zollinger-Ellison syndrome
Ulcers that will not heal, and the hormone behind them
- Gastrinoma: incidence and patient profile
- Gastrin and the loss of normal control
- How a gastrinoma causes ulcers and diarrhea
- Malignant behaviour and spread of gastrinomas
- The gastrinoma triangle
- MEN1 in Zollinger-Ellison syndrome
- Ulcer disease in Zollinger-Ellison syndrome
- Other symptoms of Zollinger-Ellison syndrome
- Endoscopic findings and when to suspect the syndrome
- Measuring gastrin and documenting acid output
- Causes of hypergastrinemia
- Preparing the patient for gastrin testing
- Gastrin levels and the secretin stimulation test
VIPoma and the Verner-Morrison syndrome
Litres of watery diarrhoea, and the potassium loss that can kill
- Vasoactive intestinal peptide in health
- VIPoma: frequency, site and behaviour
- Age distribution and associations of VIPoma
- How VIP produces watery diarrhea
- Verner-Morrison syndrome
- Features that separate VIPoma diarrhea from other causes
- Stool findings in VIPoma
- Differential diagnosis of chronic diarrhea
- Differential diagnosis of chronic diarrhea (continued)
- Metabolic consequences of VIPoma
- Diagnosis of VIPoma
Glucagonoma, somatostatinoma and nonfunctional tumors
The rare syndromes, and the silent tumours that are found only when they grow large
- Glucagonoma: frequency, size and site
- The four Ds of the glucagonoma syndrome
- Metabolic effect of excess glucagon
- Blood findings in glucagonoma
- Necrolytic migratory erythema
- Course of the rash in glucagonoma
- Necrolytic migratory erythema in glucagonoma
- Diagnosis of glucagonoma
- Somatostatinoma: frequency and site
- Associations of somatostatinoma
- Effects of excess somatostatin
- Diagnosis of somatostatinoma
- Nonfunctional pancreatic endocrine tumors
- Behaviour and presentation of nonfunctional tumors
- Rarer hormone-producing tumors
Imaging and localization
Finding a tumour that the blood test has already proved must exist
- Why localization is the central problem
- Computed tomography technique and yield
- Insulinoma on arterial and venous phase CT
- Magnetic resonance imaging
- Somatostatin receptor scintigraphy
- Yield and limits of receptor scintigraphy
- Somatostatin receptor scintigram in metastatic gastrinoma
- Endoscopic ultrasound
- Insulinoma on transgastric endoscopic ultrasound
- Fine-needle aspiration during endoscopic ultrasound
- Endoscopic ultrasound-guided needle biopsy of a pancreatic endocrine tumor
- Angiography and venous sampling
- Insulinoma on angiography and on triphasic CT
- Transhepatic selective venous sampling for insulin
- Intraoperative localization
- Stepwise localization of insulinoma
- Localization pathway for insulinoma
- Stepwise localization of gastrinoma
- Localization pathway for gastrinoma
- Routine duodenotomy in gastrinoma surgery
- Localizing the larger endocrine tumors
- Nonfunctional pancreatic endocrine tumor in the head of the pancreas
Surgical management
Preparing the patient, exposing the gland, and choosing between shelling out and resecting
- Preoperative control of hypoglycemia in insulinoma
- Exposure of the pancreas at operation
- Solitary insulinoma in the tail of the pancreas at operation
- Matching the operative finding to the preoperative scan
- Enucleation of an insulinoma
- When formal resection is needed
- Laparoscopic resection
- When the tumor cannot be found at operation
- Results after insulinoma resection
- Treatment of metastatic insulinoma
- Indications and goals of gastrinoma surgery
- Operative steps for gastrinoma
- Choice of resection for gastrinoma
- When the gastrinoma is not found at operation
- Medical control of acid in Zollinger-Ellison syndrome
- Remaining indications for total gastrectomy
- Survival predictors in gastrinoma
- Outcome after resection in gastrinoma
- Survival in gastrinoma according to liver metastases
- Treating metastatic gastrinoma
- Preparing a patient with VIPoma for surgery
- Preparing a patient with glucagonoma for surgery
- Resection of VIPoma, glucagonoma and nonfunctional tumors
- Outcomes for VIPoma
- Outcomes for glucagonoma
- Surgery for somatostatinoma and nonfunctional tumors
Conclusions
What to carry away from this topic
- Key points on diagnosis and localization
- Key points on surgical treatment
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- Maingot's Abdominal Operations, 12th Edition