Endocrine tumors of the gastrointestinal tract
• In 1902 secretin was discovered by Bayliss and Starling
• Among the cells covering the gastrointestinaltract are single cells with characteristic typical endocrine cellsThey create a kind of "diffuse"endocrine organ - scattered intraepithelial endocrine glands
Endocrine tumors of the gastrointestinal tract
• Thanks to cytochemical- imunohistochemistry- and fluorescent reactions presence of these glands in the epithelium were found ingastrointestinal tract and in the islands of Langerhans of pancreas, adrenal medulla,bronchial epithelium, fallopian tubes, vagina and uterus
Endocrine tumors of the gastrointestinal tract
• These cells are characterized by the ability to capture and metabolize biogenic amines and their precursors, and synthesizing small molecule and peptide hormone secretion
Endocrine tumors of the gastrointestinal tract
• Properties:Amine precursor uptake and decarboxylation
APUD
Endocrine tumors of the gastrointestinal tract
• APUD system cells origin fromneuroectoderm and migrate to the mucosaof primitive gut and the buds of future organ in embryonic development
Endocrine tumors of the gastrointestinal tract
• This same origin as the cells of the nervous system, chemoreceptors cells, adrenal medulla cells
Endocrine tumors of the gastrointestinal tract
• Digestive tract there are a lot of active peptidesgastrin, secretin, cholecystokinin-pancreozymin (CCK-PZ) - classified as hormones - regulate physiological functions of the gastrointestinal tract
Endocrine tumors of the gastrointestinal tract
• The epithelial cells of gastrointestinal tract and pancreatic endocrine function displaying can develop cancer of a APUDomaThey can be „silent” or produce characteristic symptoms of hormone secretion
Endocrine tumors of the gastrointestinal tract
• The incidence 5-20 cases per million population per year
• In about 70% of patients have the syndrome of multiple endocrine tumors type I(Multiple Endocrine Neoplasia - MEN)
• Hormonally inactive tumors - significantlymore
Endocrine tumors of the gastrointestinal tract
• 3% of pancreatic cancers - neuroendocrine tumors (ca. 76% - hormonally inactive)
• Resective in approximately 76% (pancreatic cancer 19%)
• 30-50% located outside the pancreas
• About 50% - malignant
Endocrine tumors of the gastrointestinal tract
• Treatment aimed at inhibiting grow tumor and reduce the excessive production of hormones
• Somatostatin analogs effectively control the secretion of hormones
• The therapeutic target for tumor treatment
Endocrine tumors of the gastrointestinal tract
• 80-90% of neuroendocrine tumors hassomatostatin receptorSomatostatin analogues (octreotide, lanreotide, RC-160)-for treatment,Diagnostics - marked octreotide - toscyntygraphy
Endocrine tumors of the gastrointestinal tract
• Endocrine tumors serotonin capture(precursor 5-hydroxytryptophan)
PET (positron emission tomography - PET)for locating small changesundetectable by scyntygraphy use octreotide
Endocrine tumors of the gastrointestinal tract
Diagnostics• Angiography of the celiac trunk • USG • Computed tomography CT• MRI Magnetic resonance tomography• Scyntygraphy (isotope indium-labeled
octreotide (In111) • Endoscopic Ultrasonography - EUS
Endocrine tumors of the gastrointestinal tract
Diagnostics:
- Arterial stimulation venossus sampling ASVS
- Transhepatic portal venosus sampling THPVS
- Intraoperative usltrasonography
- Intraoperative hormone measure
Endocrine tumors of the gastrointestinal tract
• 20-60% insulinomas fails to specify the position before the operation10-20% intraoperatively (experienced surgeon - 90% of the palpable sense)
Endocrine tumors of the gastrointestinal tract
• Treatment:chemotherapy: streptozotocin and 5 fluorouracil, doxorubicin, cispaltyna (partial remission for about two years 40-60%)Interferon alpha - 50% biochemical response, tumor reduction 20%Somatostatin analogues (octreotide), symptomatic and biochemical response 35-75%
Endocrine tumors of the gastrointestinal tract
• Treatment: There is no uniform pattern of diagnostic and therapeutic- Removal of the tumor or tumors- Liver metastases - arterial embolization, liver transplant!
Endocrine tumors of the gastrointestinal tract
• treatment: - Radiation therapy - tumors generally resistant to radiotherapy - Isotope-labeled somatostatin analogue itrium (Y90)
Insulinoma
• 1902 - Nicholls described a pancreatic endocrine tumor cells
• The incidence of approx. 0.5-5 / 1 million persons / year
• The most common tumor causing hypoglycemia
• Other tumors - beyond the pancreas - insulin-secreting growth factors (IGF-insulin-like growth factors)
Insulinoma
• 60% of all pancreatic insulomasTumor diameter 5-60 mm aprx. 20 mm 90% of cases does not exceed 20 mm10% of multiple endocrine syndrome (MEN 1)10-15% of malignant
Insulinoma
Clinical symptoms:
Whipple’ triad- occurrence of spontaneous hypoglycemia- low blood glucose levels below 50 mg%- regression of hypoglycemia after oral or
intravenous administration of glucose
Insulinoma
• Approximately 70% of patients after surgery recover
• Perioperative mortality rate is 10%
• In the case of malignant changes in the average survival time is about 2 years
InsulinomaUltrasonografia śródoperacyjna
Dobrze odgraniczony guz o średnicy 10 mm w okolicy wyrostka haczykowatego
Gastrinoma
• Gastrinoma - a tumor, which causes gastrin releasing hypergastrinaemia and increased gastric acid secretionTumors: ovarian cancer, bronchial carcinoid may secrete gastrin, but in such quantity that do not cause hypergastrinaemia
Gastrinoma
• In1955 Zollinger and Ellison described a syndrome:
• - Persistent, recurrent ulcers stomach or duodenum, even after partial gastric resection and vagotomy
• - Excessive basal secretion of gastric acid • - The presence of pancreatic cancer island
cells derived from D
Gastrinoma
•Frequency of gastrinoma 0.5-10 / 1mln/per year•About 0.1% of patients with peptic ulcer disease
Gastrinoma
• Excessive production of gastrin by tumors derived from not beta-cell (G cells) pancreatic islet
• 70% malignat tumors• In 20% of single tumor • Often develop slowly and multifocuses • Metastasis in to lymph nodes and liver but rarely
to the lung.• In about 20% occur in MEN I syndrome
Gastrinoma
Zolliner Ellison Syndrom:
-The presence of multiple peptic ulcers in the esophagus, stomach or duodenum
-Hipersecretion gastrc acid
-The presence of pancreatic tumor secreting gastrin
Gastrinoma
Symptoms:- Abdominal pain associated with active
peptic ulceration or perforation in the course of morbus ulcerosus
- Diarrhea caused by excessive production of gastric juice
- Tarry stools due to bleeding from the upper gastrointestinal tract
Gastrinoma
The symptoms and laboratory results gives rise to a suspicion of ZES:
-Recurrent peptic ulcer disease resistant to treatment -Basal secretion of gastric juice above 15 mmol HCl /h-Ratio of primary to a maximum of gastric acid secretion
(BAO: MAO) does not exceed 0.6-Gastrin concentration in serum above 500 pg / ml (normal up
to 200 pg / ml)- In tests with intravenous administration calcium, glucagon,
or secretin in patients with ZES showed an increase in gastrin
GastrinomaTreatment
The basic goal
by surgery
Inhibition of gastric acid secretion with high acidity
Tumor resection
Not always possible
Ectopic placement metastases
Multifocus changes
Total gastric resection
GastrinomaPrognosis
In the case of ZES good provided hypersecretion and eradicate gastric hyperacidity
Survival can be long
Malignant neoplasms in many cases - may grow slowly
Vernera-Morrisona Syndrom (1958), WDHA – watery diarrhea hypokalemia achlorhydria
Clinical symptoms
•persistent watery diarrhea (more than 3l / d)
•symptoms of hypokalaemia
•asthenieachlorhydria
•Tetany (as a result of hypomagnesaemia)
WDHA syndrom diagnosed very rare o
Pure prognosis
Vipoma
Tumor derived from pancreatic islet beta produced vasoactive intestinal hormon, often malignant, usually single, 80% of
pancreatic APUDoma
Glukagonoma
The tumors derived from pancreatic islet alpha cells secrete glucagon, about 70% is malignant, approximately 100 cases yet documented in
the world
Clinical symptoms
•hiperglikemia
•skin lesion - erythema migrans with necrosis places
•stomatitis
•hemolytic anemia
•elevated glucagon levels above 500 pg / ml
diagnosis - Clinical signs + pancreatic tumor
Somatostatinoma
The tumors associated with tumor cell proliferation delta pancreatic islet
Diagnosis and treatment is the same as in other pancreatic tumors derived from the APUD system cells
•are extremely rare
•large virulence
•previously documented in the world only a few dozen cases
Carcinoid
• 0,5-2,1 / 100 000 persons per year - on alimentary tract
• 0.3 / 100 000 persons per year - bronchus
Carcinoid
85% alimentary tract
- appendix 44%
- rectum 15%
- ileum 11%
10% lungs
30% non malignat tumors
Carcinoid
The incidence of -appendix- rectum, ileum
tumor below in diameter
1 cm 2 cmmetastase
2 % 80 %
Slow growth - 5-year survival in more than 70% of operated patients
Carcinoid
• Carcinoid - of I part of the primitive gut (respiratory system, thymus, stomach, duodenum) - Usually secrete corticotropin-CRH, ACTH, somatolibertin (growth-releasing hormone-GH-RH), serotonin, gastrin, glucagon
Carcinoid
• Carcinoid tumors of the second part primary gut (ileum, colon ascendens) usually secrete serotonin, tachykinins, bradykinin, chromogranin AThe final part of the primary gut (colon, rectum) are often hormonal inactive, can produce chromogranin A, human chorionic gonadotropin
Carcinoid
Carcinoid syndrome is caused by the action of vasoactive substances - serotonin
Clinical symptoms
•flush
•diarrhoea
•bronchospasmus - rare
•damage to the right side of the heart valves
Carcinoid
• Carcinoid crisis) in patients with metastatses, usually to the liver. Usually after induction (biopsy, surgery) - a significant drop in pressure, feeling beat and palpitations, massive watery diarrhea, shortness of breath, abdominal cramps, asthma attacks, flushing of the face, neck, upper part, hyperthermia
Carcinoid
• Carcinoid crisis- a life-threatening condition.Requires intensive treatment.- Octreotide 100 micro grams- Histamine receptor blockers
Carcinoid
• Cardiac symptoms (carcinoid heart disease) - subendocardial deposition of collagen – thickening, distortion and narrowing of the tricuspid valve and pulmonary trunk
Carcinoid
Extensive excision of the primary tumor and regional lymph nodes
Treatment
In the case of liver metastases
Adjuvant treatment - cytostatics
Symptomatic treatment - serotonin inhibitors (cyproheptydyna), somatostatin analogues
hepatic artery embolization
Rakowiaki
krezka jelita cienkiego
1
zagięcie śledzionowe
1
okrężnicy
jelito cienkie
1
wyrostek robaczkowy
4
Klinika Chirurgii Endokrynologicznej i Ogólnej UM w Łodzi
1974 - 2005
Apendektomie
3371
Hemikolektomie
512
guzy o śr. od 5 do 15 mm
guz śr. 120 mm
guz śr. 35 mm był przyczyną martwicy jelita cienkiego
guz śr. 30 mm