1 / 45

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

kirby
Download Presentation

CARCINOID TUMORS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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..

More Related