endocrine tumors of the gastrointestinal tract dr n med. jacekpytel

Endocrine tumors of the gastrointestinal tract

Dr n med. JacekPytel


• 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


• 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


• These cells are characterized by the ability to capture and metabolize biogenic amines and their precursors, and synthesizing small molecule and peptide hormone secretion


• Properties:Amine precursor uptake and decarboxylation

APUD


• APUD system cells origin fromneuroectoderm and migrate to the mucosaof primitive gut and the buds of future organ in embryonic development


• This same origin as the cells of the nervous system, chemoreceptors cells, adrenal medulla cells


• Digestive tract there are a lot of active peptidesgastrin, secretin, cholecystokinin-pancreozymin (CCK-PZ) - classified as hormones - regulate physiological functions 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


• 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


• 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


• 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


• 80-90% of neuroendocrine tumors hassomatostatin receptorSomatostatin analogues (octreotide, lanreotide, RC-160)-for treatment,Diagnostics - marked octreotide - toscyntygraphy


• Endocrine tumors serotonin capture(precursor 5-hydroxytryptophan)

PET (positron emission tomography - PET)for locating small changesundetectable by scyntygraphy use octreotide


Diagnostics• Angiography of the celiac trunk • USG • Computed tomography CT• MRI Magnetic resonance tomography• Scyntygraphy (isotope indium-labeled

octreotide (In111) • Endoscopic Ultrasonography - EUS


Diagnostics:

- Arterial stimulation venossus sampling ASVS

- Transhepatic portal venosus sampling THPVS

- Intraoperative usltrasonography

- Intraoperative hormone measure


• 20-60% insulinomas fails to specify the position before the operation10-20% intraoperatively (experienced surgeon - 90% of the palpable sense)


• 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%


• Treatment: There is no uniform pattern of diagnostic and therapeutic- Removal of the tumor or tumors- Liver metastases - arterial embolization, liver transplant!


• treatment: - Radiation therapy - tumors generally resistant to radiotherapy - Isotope-labeled somatostatin analogue itrium (Y90)

Insulinoma

• 1869-Langerhans - described the pancreatic insulas

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

• Treatment of choice - surgery

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

Insulinoma

Ultrasonografia przedoperacyjna

Insulinoma

Ultrasonografia przedoperacyjna

Tomografia komputerowa

Insulinoma

Tomografia komputerowa – guz w głowie trzustki

InsulinomaUltrasonografia śródoperacyjna

Guz o średnicy 13 mm na pograniczu głowy i trzonu trzustki

InsulinomaUltrasonografia śródoperacyjna

Dobrze odgraniczony guz o średnicy 10 mm w okolicy wyrostka haczykowatego

Insulinoma

Złośliwy guz naciekający śledzionę, liczne przerzuty w wątrobie

Insulinoma

Mały guz ostro odgraniczony od miąższu trzustki

Insulinoma

Mały guz trzustki – 21 mm

Insulinoma

Ultrasonografia endoskopowa

Insulinoma

Laparoskopia

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

Gastrinoma

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

Vipoma

Liczne przerzuty w wątrobie

Vipoma

Tomografia komputerowa

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

Glukagonoma

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

Somatostatinoma

Carcinoid

• WHO classification term - carcinoid -replaced - neuroendocrine tumors

Carcinoid

• 0,5-2,1 / 100 000 persons per year - on alimentary tract

• 0.3 / 100 000 persons per year - bronchus

Carcinoid

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

Rakowiak jelita cienkiego

Rakowiak oskrzela

Rakowiak oskrzela – obraz endoskopowy

Rakowiak wyrostka robaczkowego

Rakowiak jelita

Rakowiak jelita

Przerzuty do węzłów chłonnych

endocrine tumors of the gastrointestinal tract dr n med. jacekpytel

Documents

gastrointestinal tract

gastrointestinal tract

inactive tumors

gastrointestinal tract

scyntygraphy slide

jacekpytel slide

uterus slide

malignant slide