Kirtilkrabbamein í botnlanga á Íslandi 1990-2009 - lýðgrunduð rannsókn

Abstract

Adenocarcinoma of the appendix is less than 0.5% of all gastrointestinal cancers. The aim of this study was to analyse the incidence, symptoms, pathology and treatment of appendiceal adenocarcinoma in a well defined cohort as well as the prognosis of the patients.

This is a retrospective study on all patients diagnosed with adenocarcinoma of the appendix in Iceland from 1990-2009. Information on epidemiological factors, survival and treatment was collected. All histological material was reviewed. Overall survival was estimated with median follow up of 15 months (range, 0-158).

A total of 22 patients were diagnosed with appendiceal adenocarinoma in the study period (median age 63 yrs, range: 30-88, 50% males). Age-standardized incidence was 0.4/100,000/year. The most common symptom was abdominal pain (n=10). Eight patients had clinical signs of appendicitis. Most patients were diagnosed at operation or at pathological examination but one patient was diagnosed at autopsy. Five patients had an appendectomy and 11 a right hemicolectomy. One patient was not operated on and in three patients only a biopsy was taken. Twelve patients had chemotherapy and seven of them for metastatic disease. Eight patients had adenocarcinoma, seven mucinous adenocarcinoma, three signet ring adenocarcinoma, one mixed goblet cell carcinoid and mucinous adenocarcinoma,one mixed adenocarcinoma and signet ring adenocarcinoma and two a mucinous tumour of unknown malignant potential. In eight cases the tumor originated in adenoma. Most of the patients had a stage IV disease (n=13), three stage III, three stage II and three stage I. Operative mortality was 4.8% (n=1). Disease specific five year survival was 54% but overall five year survival was 44% respectively.

Adenocarcinoma of the appendix is a rare disease. No patients were diagnosed pre-operatively. Over half of the patients presented with stage IV disease.

Tilgangur: Kirtilkrabbamein í botnlanga er innan við 0,5% krabbameina í meltingarvegi. Tilgangur rannsóknarinnar var að skoða nýgengi, einkenni, meinafræði, meðferð og horfur sjúklinga hér á landi. Efniviður og aðferðir: Rannsóknin var afturskyggn og náði til allra sjúklinga sem greindust með kirtilkrabbamein í botnlanga á Íslandi 1990-2009. Skoðaðir voru faraldsfræðilegir þættir, meðferð og lifun. Öll vefjasýni voru skoðuð og meinafræði endurmetin. Meðaleftirfylgni lifandi greindra var 15 mánuðir (bil: 0-158). Niðurstöður: Alls greindust 22 sjúklingar með kirtilkrabbamein í botnlanga (miðaldur 63 ár, bil: 30-88, 50% karlar). Aldursstaðlað nýgengi var 0,4/100.000 á ári. Algengasta einkennið var kviðverkur (n=10) en átta sjúklingar höfðu klínísk einkenni botnlangabólgu. Flestir sjúklingar greindust í aðgerð eða við vefjagreiningu en einn við krufningu. Fimm sjúklingar fóru í botnlangatöku og 11 í brottnám á hægri hluta ristils. Einn sjúklingur fór ekki í skurðaðgerð og hjá þremur var eingöngu tekið vefjasýni. Tólf sjúklingar fengu krabbameinslyfjameðferð, þar af sjö við dreifðum sjúkdómi. Átta sjúklingar höfðu kirtilkrabbamein, sjö slímkrabbamein, þrír sigðfrumukrabbamein, einn blandað krabbalíkisæxli af slímfrumugerð og slímkrabbameini, einn kirtilkrabbamein með sigðfrumukrabbameini og tveir höfðu slímæxli af óvissri illkynja hegðan. Í átta tilvikum var æxlið upprunnið í kirtilsepaæxli. Flestir höfðu sjúkdóm á stigi IV (n=13), 3 á stigi III, 3 á stigi II og 3 á stigi I. Skurðdauði var 4,8% (n=1). Sjúkdómssértæk fimm ára lifun var 54% en heildar fimm ára lifun 44%. Ályktun: Kirtilkrabbamein í botnlanga eru sjaldgæf. Allir sjúklingar greindust fyrir tilviljun. Rúmlega helmingur sjúklinga var með dreifðan sjúkdóm við greiningu.