Esophageal TumorsEsophageal Tumors

Cengiz PataCengiz Pata

Gastroenterology, Yeditepe Gastroenterology, Yeditepe University, IstanbulUniversity, Istanbul

Bening esophagial tumorsBening esophagial tumors LeiomyomaLeiomyoma HemangiomaHemangioma Granuller cell tumorGranuller cell tumor Congenital cell tumorCongenital cell tumor Fibrovasculer polypFibrovasculer polyp Bronchogenic cystBronchogenic cyst Eosinophilic granulomaEosinophilic granuloma LymphangiomaLymphangioma Squamose cell papillomaSquamose cell papilloma lipomalipoma

epidemiologyepidemiology

60-70 years60-70 years4/1 towards to men4/1 towards to men5-10/100.0005-10/100.0007. ca mortality in men7. ca mortality in menSquamose cell carcinoma(80%), Squamose cell carcinoma(80%),

adenocarsinoma, sarcomas, lymphoma, adenocarsinoma, sarcomas, lymphoma, malignt melanomemalignt melanome

etiologyetiologySquamose CaSquamose Ca Alchol, tabaccoAlchol, tabacco NitrosemanineNitrosemanine RadiationRadiation AchalasiaAchalasia TylosisTylosis Plummer Winson syndromePlummer Winson syndrome RadiationRadiation HPVHPVAdenocaAdenoca Baret’s (5% malignancy)Baret’s (5% malignancy) GERD (85% baret’s)GERD (85% baret’s) ObesityObesity SclerodermaScleroderma

Clinacal presentationClinacal presentation 1/3 upper esophagus 15%, middle 50%1/3 upper esophagus 15%, middle 50% Progressive dysphagia (firstly solid)Progressive dysphagia (firstly solid) OdynofagiaOdynofagia Back painBack pain AnorexiaAnorexia Weight lossWeight loss RegurgitaionRegurgitaion Voice changeVoice change Aspritaion/pnomoniaAspritaion/pnomonia hematemesishematemesis

prognosisprognosis

>5 cm involvement>5 cm involvementLymph node metastas (5 year survival Lymph node metastas (5 year survival

%20)%20)Small cell, malign melanomaSmall cell, malign melanoma

diagnosisdiagnosis

EndoscopyEndoscopyBarium graphyBarium graphyChest radiogramChest radiogramCTCTEUSGEUSG

treatmenttreatment

Surgery (only %40)Surgery (only %40) RadiationRadiation CheomoradiationCheomoradiation DilataionDilataion AblationAblation Photodynamic thrapyPhotodynamic thrapy EMR (<3cm)EMR (<3cm) StentsStents

Tumors of StomachTumors of Stomach

Cengiz Pata, M.DCengiz Pata, M.DGastroenterology Department, Yeditepe UniversityGastroenterology Department, Yeditepe University

IstanbulIstanbul

HISTOLOGYHISTOLOGY

95% 95% AdenocarcinomaAdenocarcinoma- papiller- papiller

- tubular- tubular

- mucinous- mucinous

4%4% Adeno-squamousAdeno-squamous

< 1%< 1% SquamousSquamous

< 1%< 1% undifferentiatedundifferentiated

Stomach CarcinomaStomach Carcinoma

EpidemiologyEpidemiologyChronic A-GastritisChronic A-GastritisChronic B-Gastritis (HP)Chronic B-Gastritis (HP)BI, BII resectionBI, BII resectionPolyposisPolyposisChr. Ulcer VentriculiChr. Ulcer VentriculiM, MenetrierM, MenetrierBlood Group ABlood Group AMen > WomenMen > Women

MacroscopicMacroscopic(Borrmann Classification)(Borrmann Classification)

I.I. PolypousformPolypousform

II.II. UlceratedUlcerated

III.III. Ulcerated-infiltrativeUlcerated-infiltrative

IV.IV. Diffuse-infiltrativeDiffuse-infiltrative

Early Cancer JapanEarly Cancer Japan

I.I. ProtrusionProtrusion

II. Surface (II. Surface (, , , , ))

III. ExcavatedIII. Excavated

TherapyTherapy

Radical Tumour ResectionRadical Tumour ResectionSt. OpSt. Op : : GastrectomyGastrectomy

LymphadenectomyLymphadenectomyOmentum major +Omentum major +

minorminorSplenectomySplenectomy

Antrum CaAntrum Ca : partial resection ? : partial resection ?Primary inoperable neoplasmPrimary inoperable neoplasm

chemotherapychemotherapyradiationradiation

PrognosisPrognosis

5 year rate5 year rate

Carcinoma in citu 100 %Carcinoma in citu 100 %

Early Ca : 90 %Early Ca : 90 %

Small intestine tumorsSmall intestine tumors

Cengiz Pata, M.DCengiz Pata, M.DGastroenterology Department, Yeditepe UniversityGastroenterology Department, Yeditepe University

IstanbulIstanbul

clasificationclasification Adenoca %35-50, proximalAdenoca %35-50, proximal Carcinoid TM %20-40 apendix, ileumCarcinoid TM %20-40 apendix, ileum Lymphoma %14Lymphoma %14-MALTOMA-MALTOMA-IPSID-IPSID-EATCL (after the gluten enteropathy %7-10)-EATCL (after the gluten enteropathy %7-10)-multiple Lymphoid Poliposis-multiple Lymphoid Poliposis-Periferal Nodal lenfoma-Periferal Nodal lenfoma FibrosarkomFibrosarkom AngiosarkomAngiosarkom LiposarkomLiposarkom LeiyomyosarkomLeiyomyosarkom

Bening (41%)Bening (41%)

AdenomaAdenomaGISTGISTHamartomaHamartomaAngiomaAngiomaPseudotumor (brunner gland hyperplasia, Pseudotumor (brunner gland hyperplasia,

pancreatic rest,endometrioma, pancreatic rest,endometrioma, hyperplastic polypshyperplastic polyps

Malign (51%)Malign (51%)

Carcinoids tumors (25-45%)Carcinoids tumors (25-45%)GIST (15-25%)GIST (15-25%)Adenocarcinoma (30-40%)Adenocarcinoma (30-40%)Lymphoma (4-10% )Lymphoma (4-10% )Metastatic carcinomaMetastatic carcinomaMalign melanomaMalign melanoma

NETNET

APUD (Amine precursor uptake and APUD (Amine precursor uptake and decarboxylation) celldecarboxylation) cell

IntroductionIntroduction

İntestine and pancreas İntestine and pancreas İncidance 1/ İncidance 1/ 100,000100,000Carcinoid %Carcinoid %5050

ClinicsClinics

tumor syndrome hormone localisation

Carcinoid Carcinoid syndrome Serotonin, taşikinin, bradikinin, histamin

Mmidintest ne

VIPoma Diorhea, achloirdi (WDHA)

Vazoaktif intestinal peptid (VIP)

pancreas alung

Glukagonoma Glucogonoma a

Glukagon Pankreas, duodenum

Gastrinoma Zollinger-Ellison sendromu

Gastrin Pankreas, duodenum

Insülinoma ghypoglycemia Insülin Pankreas

Zollinger Ellison SyndromeZollinger Ellison Syndrome

Severe peptic ulcer diathesis + gastric Severe peptic ulcer diathesis + gastric acid hypersecretion due to acid hypersecretion due to -cell -cell endocrine tumor endocrine tumor

0.1-1% of PUD patients 0.1-1% of PUD patients

Sporadic, or associated with MEN type I Sporadic, or associated with MEN type I (25%)(25%)

Zollinger Ellison SyndromeZollinger Ellison Syndrome

>80% Localized to gastrinoma triangle:>80% Localized to gastrinoma triangle:cystic & common bile ducts, duodenum, cystic & common bile ducts, duodenum, junction head and body of pancreas. junction head and body of pancreas.

60 % malignant, up to 50% with metastasis60 % malignant, up to 50% with metastasis

Clinical: PUD >90%Clinical: PUD >90% (recurrent, multiple, refractory, complicated) (recurrent, multiple, refractory, complicated)

Clinical features of Zollinger-Clinical features of Zollinger-Ellison syndromeEllison syndrome

MEN I MEN I Autosomal Dominant:Autosomal Dominant:

Parathyroid (~90%), Pancreas (40-80%)Parathyroid (~90%), Pancreas (40-80%)Pituitary (30-60%)Pituitary (30-60%)

Contributory effect of Contributory effect of hyperparathyroidism, hypercalcemia hyperparathyroidism, hypercalcemia hypergastrinemia hypergastrinemia acid secretion acid secretion

Higher incidence of carcinoids Higher incidence of carcinoids

Smaller and multiple duodenal Smaller and multiple duodenal gastrinomas gastrinomas

Diagnosis of Gastrinoma Diagnosis of Gastrinoma

Combination of clinical signs Combination of clinical signs Fasting gastrin levels (> 150 pg/ml) Fasting gastrin levels (> 150 pg/ml) Avoid confounding factorsAvoid confounding factors

(hypochlorhydria, PPIs, outlet obstruction, (hypochlorhydria, PPIs, outlet obstruction, renal failure) renal failure)

Assess acid secretion (if low- excludes)Assess acid secretion (if low- excludes)Provocative tests (calcium, secretin)Provocative tests (calcium, secretin)

Treatment Treatment

Localization (EUS, Oct scan, MRI, CT)Localization (EUS, Oct scan, MRI, CT)

Exclusion of metastasisExclusion of metastasis

If positive – symptomatic cureIf positive – symptomatic cure

If negative attempt surgical resection If negative attempt surgical resection ( less likely in MEN I ~ 6%) ( less likely in MEN I ~ 6%)

NETNET Diagnosis Diagnosis

ClinicsClinicsHistopaHistopathologythologyHormon levelHormon levelStimStimulating testulating testRadyologyRadyology

NET treatmentNET treatment

Image courtesy of Dr. L. Anthony.

SurgerySurgery(total excision difficult(total excision difficult))Stopping tumor growthStopping tumor growth AndAndBetter life Better life

SurgerySurgery(total excision difficult(total excision difficult))Stopping tumor growthStopping tumor growth AndAndBetter life Better life

NETNET, medical treatment, medical treatment

SomatostatinSomatostatinee

iinterferonnterferonee

CheomothreapyCheomothreapy

Carcinoid tumore Carcinoid tumore syndromesyndrome

Carcinoid tumorCarcinoid tumor

GIGIS system end respiratory systemS system end respiratory system İncidance İncidance 100,000100,000 1/2 1/2 ApeApenndectomdectomyy and diag and diagnnose !ose !

Carcinoid tumor Carcinoid tumor

Asymptomatic (up to liver metastase )Asymptomatic (up to liver metastase )– dyspepsiadyspepsia

– misdiagnosismisdiagnosis

Carcinoid syndromeCarcinoid syndrome ( (diseasedisease) )

%10%10LLarge, metastatic poor survivearge, metastatic poor survive

Carcinoid syndromeCarcinoid syndrome

serotonineserotonine

Carcinoid sendrom Carcinoid sendrom –– symptomssymptoms

Artriis(%7)Artriis(%7)

Dermatitis(%5)Dermatitis(%5)

Diarrhea(%68–84)Diarrhea(%68–84)

cyanose(%18)cyanose(%18)

Heart disease(%14–41)Heart disease(%14–41)

flushing(%63–94)flushing(%63–94)

Telanjektzy(%25)Telanjektzy(%25)

Bronkokonstriksiyon(%3–19)Bronkokonstriksiyon(%3–19)

Abdominal pain(%10–55)Abdominal pain(%10–55)

Attack of CarcinoidAttack of Carcinoid

4040

5-HIAA 5-HIAA Carcinoid tumor Carcinoid tumor

5-HIAA5-HIAA,, serotoninineserotoninineUrinary expression of Urinary expression of 5-HIAA5-HIAA

DiagnosisDiagnosis

Pathology(Pathology( arg argininooffil il andand argentaffin argentaffin ))Biochemical (Biochemical (5-HIAA, 5-HIAA, chchromogranin A)romogranin A) OctreoScanOctreoScan®*®*,,CT, MRCT, MRBBronronchoscopychoscopy, gastros, gastroscopy, copy,

colonoscopycolonoscopy

survivesurvive

Vinik A, et al. Dig Dis Sci. 1989; 34(suppl): 14S–27S.

treatmenttreatment

Carcinoid disease treatmentCarcinoid disease treatment

Carcinoid tumor follow upCarcinoid tumor follow up

Every year• 5-HIAA• Chromogranin A• Pre-operative marker• Abdominal CT (6-12

mounths)

Every year• 5-HIAA• Chromogranin A• Pre-operative marker• Abdominal CT (6-12

mounths)

Every 4 - 6 mounths• 5-HIAA• Chromogranin A• Follow up high marker

Every 6 - 12 muonths• Abdominal CT• Echocardiography

Every 4 - 6 mounths• 5-HIAA• Chromogranin A• Follow up high marker

Every 6 - 12 muonths• Abdominal CT• Echocardiography

• OctreoScan®• OctreoScan®

AsymtomaticAsymtomatic SymtpmaticSymtpmatic

Treatment, Treatment, Sandostatin Sandostatin

Long activation (every mounths)Long activation (every mounths)Binding Sst-2/sst-5 receptorBinding Sst-2/sst-5 receptor%70 and %80%70 and %80Activation of these receptorActivation of these receptor

SandostatinSandostatinSandostatinSandostatin

Adapted from Lancranjan I, et al. Metabolism. 1995;44(suppl 1):18–26.

• Theropatic level:7 - 11 day

• One mounths

• Theropatic level:7 - 11 day

• One mounths

Effect of 5 IHAA Effect of 5 IHAA

Supress to 50%Supress to 50%

*L

1. Rubin J, Ajani J, Schirmer W, et al. J Clin Oncol. 1999;17:600–6.

2. The Medical Network. Healthcarenews. http://www.themedicalnetwork.org/full_magazine/june2003-p28–40.pdf.

defecation flushingdefecation flushing

SCN = screening (tarama); BASE = baseline (başlangıç).Rubin J, et al. J Clin Oncol. 1999;17:600–6.

diarheadiarhea

diarheadiarhea

1. Rubin J, Ajani J, Schirmer W, et al. J Clin Oncol. 1999;17:600–6.

2. The Medical Network. Healthcarenews. http://www.themedicalnetwork.org/full_magazine/june2003-p28–40.pdf.

SurviveSurvive before afterbefore after

Anthony LB, et al. Digestion. 1996;57(suppl 1):50–3.

adenocarcinomaadenocarcinoma

High animal fat and protein, bile acid, High animal fat and protein, bile acid, polyposis syndrome, crohn disease, celiac polyposis syndrome, crohn disease, celiac diseasedisease

Crampy periumblical pain, bloating, nausea,Crampy periumblical pain, bloating, nausea,

Bleeding, obstruction, intussiception, Bleeding, obstruction, intussiception, volvulus, weight lessionsvolvulus, weight lessions

5 year survive %20-355 year survive %20-35

Cheomotherapy, surgeryCheomotherapy, surgery

lymphomalymphoma T cell lyphoma (celiac)T cell lyphoma (celiac) MALTomaMALToma Multiple lymphoid polyposisMultiple lymphoid polyposis Difuse large cellDifuse large cell Small noncleaved lymphomaSmall noncleaved lymphoma

(malabsorbtion: T cell, others: obstruction, pain, (malabsorbtion: T cell, others: obstruction, pain, mass unless bacterial overgrowth)mass unless bacterial overgrowth)

IPSIDIPSID

IPSIDIPSID

alfa chain disease (west type lymphoma )alfa chain disease (west type lymphoma ) beningn or low grade lymphomabeningn or low grade lymphoma IgAIgA 15-25 year15-25 year severe diarhea, abdominal pain, weight loss, feversevere diarhea, abdominal pain, weight loss, fever Double bloon endoscopy,band alfa2 or beta in Double bloon endoscopy,band alfa2 or beta in

electrophoresis, electrophoresis, Early stage antibiotics (tetracyline plus Early stage antibiotics (tetracyline plus

metranidazole 6-12 mounts)metranidazole 6-12 mounts) Malignant chance occurs: cheomathrapyMalignant chance occurs: cheomathrapy

GISTGIST Nonepithelial neoplasmsNonepithelial neoplasms Primitive mesencymal cellsPrimitive mesencymal cells Leiomyoma, schwanoma,leiyomyosarcoma Leiomyoma, schwanoma,leiyomyosarcoma

(70%), leiomyoblastoma, myofibroblastic tumor(70%), leiomyoblastoma, myofibroblastic tumor All segments of GIS, 60% stomach, 30% intestineAll segments of GIS, 60% stomach, 30% intestine Prognose: size>5cm, mitotic count 1-5 per 10 Prognose: size>5cm, mitotic count 1-5 per 10

high power, presence of c kit genehigh power, presence of c kit gene bleedingbleeding 5 year survive 25-30%5 year survive 25-30% Radiotion, cheomotherapy (tyrosinase kinaseRadiotion, cheomotherapy (tyrosinase kinaseİnhibitor –St1-571)`, surgeryİnhibitor –St1-571)`, surgery