Carcinoid Tumors - Mount Sinai Surgery Residency Page

Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Carcinoid Tumors,10/6/04,Umut Sarpel,Case presentation,47M,asymptomatic,PMHx,:,hypertriglyceridemia,PSHx,:R ACL repair,T&A,1996:Incidental liver lesions seen on U/S,Case presentation,MRI,:multiple liver lesions,largest 8cm,no other masses seen,Fine needle,Bx,:carcinoid tumor,OctreoScan,:activity in liver lesions,no other areas of activity,Mild,Sx,:bloating,diarrhea,Case presentation,Unknown primary,1998:Small bowel series,EGD,colonoscopy-no neoplasm,Carcinoid crises:flushing,headaches,itching,diarrhea,hypo/hypertension,Sandostatin-short-acting and depot,Systemic chemotherapy-,Streptozotocin,Chemoembolization,x 3,Case presentation,Increasingly frequent and severe crises,2001:Capsule,endoscopy,to search for primary tumor:lesion in jejunum,CT:new mesenteric mass-tumor and,bulky nodes,Referred for,cytoreductive,surgery,Case presentation,Operative findings:,2.5cm white,hard lesion in jejunum,grossly invading through wall,Similar lesion on adjacent mesentery with bulky nodes,Liver riddled w/metastases,Case presentation,Small bowel resection including extensive resection of adjacent mesentery,total resection of 75cm small bowel,Radiofrequency ablation x 2,Liver wedge biopsy,Prophylactic,cholecystectomy,Pathology,Multiple carcinoid tumors in small bowel,Extensive,serosal,involvement/retraction,Angiolymphatic,invasion,Margins negative for tumor,5/11 lymph nodes positive for carcinoid,Gall bladder with focus of carcinoid on,subserosal,aspect,Liver,Bx,:focal carcinoid tumor,Introduction,First described in 1888,Carcinoid=“Cancer-like,thought to be more indolent,Most often diagnosed in 5th/6th decade,55%female,Introduction,Cell of origin:,Kulchitsky,cell,a,neuroendocrine,cell found along,the primitive GI tract,Highly biologically active tumors,Secretion of several hormonal mediators,Introduction,Incidence:reported as 3.8 per 100,000,Much higher rate at autopsy:,20-yr study at Mayo Clinic,small bowel only,found 97/14,852(0.65%),12-yr study in Sweden found 199/16,294(1.2%),Maggard MA et al.Ann Surg 2004;240:117-122.,Distribution,11,427 cases of carcinoid examined,Largest ever study of distribution,GI tract:54.5%,Lung/Bronchus:30.1%,Pancreas:2.3%,Ovarian:1.2%,Biliary,:1.1%,Maggard MA et al.Ann Surg 2004;240:117-122.,Distribution,Within GI tract,Small bowel:44.7%,Rectum:19.6%,Appendix:16.7%,Colon:10.6%,Stomach:7.2%,Maggard MA et al.Ann Surg 2004;240:117-122.,Etiology,Genetics have not been fully elucidated,Reports of mutations in:,p53,bax,bcl,-2,n-,myc,and c-,jun,Overall increased malignant risk:,28%with multiple carcinoid primaries,Synchronous non-carcinoid cancer 17-53%,Schnirer II et al.Acta Oncologica 2003;42(7):672-92,Etiology,MEN 1-5-10%have carcinoid tumor,Hyperparathyroidism,Pituitary tumors,Pancreatic/duodenal tumors,More often in thymus,bronchus,Tend to be more aggressive tumors,Etiology,Gastric carcinoids:,75%a/w chronic atrophic gastritis,5-10%a/w,gastrinoma,15-25%sporadic cases,High,gastrin,seen w/first two groups,Gastrin,appears to be,mitogenic,Symptoms,Symptom,Frequency,Characteristics,Flushing,85-90%,minutes to days,Diarrhea,70%,secretory,Abdominal pain,35%,obstruction,ischemia,hepatomegaly,R heart disease,30%,if metastatic,Teleangiectasia,25%,facial,Bronchospasm,15%,-,Pellegra,In carcinoid tumors,up to 60%of Tryptophan,can be shunted to 5-HT,causing pellegra,Triad:dermatitis,diarrhea,dementia,Mediators,Symptom,Mediator,Flushing,5-HT,substance P,histamine,Kallikrein,Diarrhea,Gastrin,5-HT,prostaglandins,VIP,Bronchospasm,5-HT,histamine,Heart disease,5-HT,substance P,Diagnosis,24hr Urinary 5-,HIAA,88%specificity,Chromogranin,A,100%sensitive,but very non-specific,Also released by hormonally,inactive,tumors,Other tests include:substance P,neurotensin,hCG,neuropeptide,K,neuropeptide,PP,but none as useful,Imaging,CT/,MRI,OctreoScan,:80-90%sensitive,PET scan:high false-negatives/positives,MIBG,:55-70%sensitivity,95%specific,may have a role in patients on long-acting,octreotide,analogues,Endoscopic U/S:can detect 2-3mm lesions,Prognosis,Local tumors have excellent prognosis regardless of site of origin,Stomach,colon,and rectal tumors carry a relatively worse prognosis if advanced,Poor prognostic indicators,Male gender,Carcinoid syndrome,Histological grade,Maggard MA et al.Ann Surg 2004;240:117-122.,5-year survival by location,Site,All stages,Local,Distant,Stomach,75.1/55.3%,90.2/69.6%,18.0/9.0%,Small bowel,76.1/54.6%,94.5/70.4%,51.2/32.4%,Appendix,76.3/65.0%,95.6/87.8%,37.5/26.8%,Colon,69.5/41.8%,94.1/77.1%,27.8/4.1%,Rectum,87.5/77.8%,94.9/86.2%,14.6/13.1%,*Cancer-specific survival/relative overall survival,Maggard MA et al.Ann Surg 2004;240:117-122.,Medicinal Treatment,Somatostatin,:inhibits release of,gastrin,secretin,growth hormone,insulin,glucagon,Directly,cytostatic,to tumor cells,Somatostatin,analogue:,octreotide,relieves symptoms,appears to slow tumor growth-?,suvival,benefit,Formulations,Octre