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CARCINOID TUMORS

CARCINOID TUMORS. OF GIT. DEFINITION. “CARCINOID TUMORS ARE RARE ,SLOW GROWING NEURO ENDOCRINE TUMORS”. HISTORICAL BACKGROUND. 1 st described by LUBARSCH (1888) “ KARZINOIDE ” coined by OBERNDORFER (1907) Also known as ARGENTAFFINOMAS OCCURRENCE 13-34% OF ALL GIT TUMORS

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CARCINOID TUMORS

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  1. CARCINOID TUMORS OF GIT

  2. DEFINITION “CARCINOID TUMORS ARE RARE ,SLOW GROWING NEURO ENDOCRINE TUMORS”

  3. HISTORICAL BACKGROUND • 1st described by LUBARSCH(1888) • “KARZINOIDE” coined by OBERNDORFER(1907) • Also known as ARGENTAFFINOMAS • OCCURRENCE • 13-34% OF ALL GIT TUMORS • 17-46% OF ALL SMALL BOWEL MALIGNANCIES

  4. Classification • FOREGUT CARCINOIDS • MIDGUT CARCINOIDS • HINDGUT CARCINOIDS

  5. 1.FOREGUT • esophagus, stomach, duodenum, pancreas and bronchial tree • Atypical carcinoid syndrome • HORMONES SECRETED • Small amount of serotonin • More of 5 – HTP ( hydroxytryptophan) • Histopathology – Regular shaped, trabecular arrangement ,round granules, silver stained

  6. 2.MIDGUT • Jejunum up to the ascending colon including meckel’s diverticulum ; ovary and remnants of neural crest tissue • classical carcinoid syndrome • Hormones secreted • Serotonins, bradykinins, tachykinins, Prostaglandins , Substance P….. • Histopathology – pleomorphic, arranged in nests and capsulated

  7. 3.HINDGUT • Occurs from transverse colon to the rectum • Rarely secretes any hormones • Hence rare association with carcinoid syndrome • Histopathology- tabecular pattern,rounded granules, not silver stained

  8. G I SITES

  9. Extra gastro intestinal sites • Bronchial tree (commonest) • Thyroid • Thymus • Pancreas • Ovary • testes

  10. Secretory products • 5- HIAA 88% • 5- HTP NEUROPEPTIDE K 67% • 5- HT KALLIKREIN 40% • SUBSTANCE P 32% • PROSTAGLANDINS,HISTAMINE , DOPAMINE • ACTH • HCG – α,β • NEUROTENSIN 19 % • MOTILIN (14%)

  11. ETIOPATHOLOGY • Unknown • Risk factors • Family history of men 1 • Smoking • race – african americans • Not related to diet

  12. Benign or malignant? • Cytology does not show… • Malignant potential is related to location, size, depth of invasion and growth pattern • Location • Appendicular – 3% metastasize • Ileal – 35% metastasize • Size • <1cm – 2% metastasize • 1-2cm – 50% metastasize • >2cm – 80-90% metastasize

  13. APPENDICEAL CARCINOIDS • Most common malignancy of Appendix • 1 in every 200 to 300 appendectomies, • usually distal part is involved • TIP > BODY > BASE CARCINOID INVOLVING TIP

  14. pathology • Arise from kulchitzsky cells at the base of mucosal crypts • Macroscopy • Hard yellowish mass deep to mucosa • Microscopy • Silver staining ganules in cytoplasm • Involvement of muscularis mucosa and peritoneum

  15. HISTOLOGY SILVER STAINED GRANULES TUMOR IN A RESECTED APPENDIX

  16. Clinical features • Any age , Average age:42 years • Female predominance • Mostly asymptomatic and found incidentally • Metastasis is rare • Signs and symptoms of acute appendicitis • Pain, Nausea, Vomiting, Constipation/Diarrhoea, Low fever, Abdominal swelling.

  17. Diagnosis • Mostly Found incidentally on Appendectomy • Late presentation • ELEVATED HORMONES • CARCINOID SYNDROME

  18. INVESTIGATION • SERUM SEROTONIN ,5-HTP -(NORMAL-101-283 ng/ml ) • URINARY 5-HIAA (BEST INVESTIGATION) • TUMOR MARKERS • PLASMA CHROMOGRANIN A(80%) • PLASMA NEURON-SPECIFIC ENOLASE (17-47%) • ULTRASONOGRAPHY • HR CT SCAN • SIZE OF TUMOR • EVALUATION OF LIVER METASTASIS

  19. Contd… • RADIOACTIVE SCANS • I131-MIBG SCAN. • OCTREOSCAN -In111-LABELED DTPA-OCTREOTIDE SCINTIGRAPHY

  20. Urinary 5-HIAA assay (5-hydroxyindole-3-acetic acid ) TWO TYPES • FASTING PLASMA 5-HIAA ASSAY (PREFERED) • cut-off value of 118 nmol/L • sensitivity of 89%, a specificity of 97% • 24HR URINE ASSAY • NORMAL LEVEL -2 and 8 mg/day • 5-HIAA has 73% sensitivity and 100% specificity

  21. HORMONE SECRETION

  22. FALSE-POSITIVE ELEVATIONS • patients eating serotonin-rich foods, such as bananas, pineapple, walnuts, avocados, or hickory nuts, • medications (cough syrup containing guaifenesin, acetaminophen, salicylates, or L-dopa)

  23. Treatment • Tumor <1cm – Appendectomy • Tumor 1-2 cm • If resected edges are involved –right hemicolectomy • Non-involvement of resected edges – appendectomy is enough • Tumor >2cm/metastasis –right hemicolectomy + removal of ileocaecal LN

  24. DISTAL 15CM ILEUM • APPENDIX • ASCEDING COLON • RIGHT 1/3RD OF TRANSVERSE COLON RIGHT HEMICOLECTOMY

  25. Prognosis (5 year survival rate) • Localised leisions – 94 % • Regional invasion – 85 % • Distant mets – 34 %

  26. Small Intestinal Carcinoids • 70 to 80% in the ileum • 70% are within 60 cm of the ileocecal valve • Multi centric tumors in 20-30% • From enterochromaffin cells of the crypts of liberkuhn • CLINICAL FEATURES • bowel obstruction, abdominal pain, flushing, or diarrhea. • Additional investigations • BARIUM MEAL – MULTIPLE BOWEL KINKING • ANGIOGRAPHY

  27. HISTOLOGY MULTIPLE CARCINOIDS

  28. TREATMENT • <1CM- LOCAL RESECTION • >1CM - wide resection with en bloc resection of the adjacent lymph-bearing mesentery • Even 1cm tumors show metastasis

  29. Rectal carcinoid • Small asymptomatic seen incidentally on proctosygmoidoscopy • Larger lesions show ulceration and rectal bleed • Yellow gray submucosal nodules • TREATMENT • <1cm local resection is curative • 1-2cm -wide local full-thickness excision • >2cm -abdominoperineal resection or -low anterior resection

  30. RECTAL CARCINOID POLYP ENDOSCOPY AND RESECTION ULCERATING CARCINOID

  31. CARCINOID SYNDROME It is body’s reaction to various vasoactive amines like… • Serotonin, substance p, bradykinin and histamines • THORSON described carcinoid syndrome • 10 % carcinoid tumors only develop carcinoid syndrome • Mean age : 57 years

  32. PATHOLOGY • DUE TO HUMORAL FACTORS PRODUCED BY CARCINOID TUMORS • SEROTONIN, 5 – HTP • SUBSTANCE P • BRADYKININ • HISTAMINE • DOPAMINE • KALLIKREIN • PROSTAGLANDIN • NEUROPEPTIDE K

  33. Contd… • MAINLY IN PRESENCE OF LIVER METASTASIS • HORMONES NOT BEING METABOLISED BY LIVER • TUMORS BYPASSING LIVER • OVARIAN CARCINOID • RETROPERITONEAL CARCINOID

  34. Association • Midgut carcinoids(75–87%) • Foregut carcinoids(2–33%) • Hindgut carcinoids(1–8%) • Unknown (2–15%)

  35. Commonest symptoms/signs • Diarrhea (32–84%) • Flushing (63–75%) • Pain (10–34%) • Asthma (4–18%) • Heart disease (11–41%)

  36. Clinical features • VASOMOTOR • Flushing • DIFFUSE ERYTHEMATOUS • VIOLACEOUS • PROLONGED FLUSHES- 2-3 DAYS • BRIGHT RED PATCHY FLUSHNG-GASTRIC CARCINOID • pain • Asthma/Wheezing

  37. Contd… • CARDIAC • Tachycardia • PULMONARY STENOSIS(90%) • TRICUSPID INSUFFICENCY(47%) • TRICUSPID STENOSIS(42%) • GASTROINTESTINAL • Diarrhoea- episodic,after meals,watery • MALABSOPTION • pellagra –dementia ,dermatitis ,diarrhoea

  38. INVESTIGATIONS • Evaluaton of humoral factors • Similar to carcinoid tumors • PENTAGASTRIN PROVOCATION TEST • INVESTIGATION OF THE TUMOR • USG,ANGIOGRAPHY • BARIUM MEAL – MULTIPLE BOWEL KINKING • CT- EXTENT OF SPREAD, LIVER AND LN METS • RADIOACTIVE SCAN

  39. DIFFERENTIAL DIAGNOSIS • Flushing • Menopausal syndrome; pheochromocytoma; mastocytosis; benign cutaneous flushing; medullary carcinomas of the thyroid; ingestants (e.g., food, drugs) • Wheezing • Asthma; anaphylaxis; pulmonary edema; bronchial foreign bodyDiarrheaGastroenteritis; inflammatory bowel disease; infectious colitis; laxative abuse • Heart valve symptoms • Rheumatic heart disease; subacute bacterial endocarditis; dilated cardiomyopathy; ischemic heart disease with papillary muscle dysfunction • URINARY 5-HIAA NOT ELEVATED

  40. Treatment • Avoid precipitating conditions • - Stress ,anesthesia, chemotherapy • dietary suppl. with nicotinamide, • diuretics -heart failure • oral bronchodilators – wheezing • antidiarrheal agents – loperamide, diphenoxylate • somatostatin analogues • octreotide, lanreotide

  41. Contd.. • serotonin receptor antagonists(5HT1,5HT2) • methysergide, cyproheptadine, and ketanserin • 5HT3 receptor antagonists - ondansetron, tropisetron, alosetron • Interferon-a • Parachlorophenylanine , a-Methyldopa • SPECIFIC TREATMENT OF LIVER METASTASIS • Hepatic artery embolization • chemoembolization (5-fluorouracil, adriamycin, cisplatin, mitomycin) • radiofrequency ablation, cryosurgery, and alcohol injection

  42. ATYPICAL CARCINOID SYNDROME • FOREGUT CARCINOIDS • NORMAL OR SLIGHT ELEVATED URINARY 5- HIAA • ELEVATED URINARY 5HT AND 5- HTP • DOPA DECARBOXYLASE ABNORMALITY

  43. SUMMARY • NEUROENDOCRINE TUMORS • MIDGUT>HINDGUT>FOREGUT • COMMONEST IS APPENDICEAL • URINARY 5-HIAA • LOCAL RESECTION OF TUMOR • CARCINOID SYNDROME • VASOMOTOR,CARDIAC,GASTRO INTESTINAL

  44. Thank You..

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