Wilmsæxli á Íslandi : afturskyggn rannsókn tímabilið 1961-1995

Ingólfur Einarsson, Tómas Guðbjartsson, Guðmundur Vikar Einarsson, Jóhann Heiðar Jóhannsson, Guðmundur K. Jónmundsson, Guðmundur Bjarnason

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Abstract

Objective: Wilms' tumor is a malignant disease in the kidneys that usually affects young children. Information about the clinical behaviour of this tumor in Iceland has been scarce. The aim of this study was to find the incidence, clinical presentation, treatment and survival of patients with Wilms' tumor. Material and methods: Included in the study were all patients diagnosed with Wilms' tumor in Iceland from 1st of January 1961 to 31st of December 1995. Altogether, there were 17 patients, 15 children, mean age 33 months (standard deviation 19, range 5-77 months) and two adults (age 25 and 29), with M/F ratio 0.7. Information was gained from each patient's record and the cancer registry of the Icelandic Cancer Society. All the tumors were re-evaluated by a pathologist and staged according to the NWTS staging system. Results: Age adjusted incidence during the study period was 0.2/100,000 per year (1.0 for children under 15 years). Abdominal mass (65%) and abdominal pain (53%) were the most common symptoms. Histology was typical in all cases except one with anaplasia and another with sarcomatous growth. One patient was diagnosed in stage I (6%), six in stage II (35%) and seven in stage III (41 %). Two patients had pulmonary metastases (stage IV) and one had bilateral tumor (stage V). Nephrectomy was performed in all cases. The operative mortality was 12%. Of the 15 patients surviving surgery, 12 received radiotherapy, 12 chemotherapy and nine both treatments. Crude five-year-survival for the whole group was 42%, 25% for the patients diagnosed 1961-1976 and 61% for those diagnosed 1977-1995 (p=0.13). The patient with bilateral tumor was still alive 13 years after diagnosis. Conclusion: As in other Western countries, Wilms' tumor is rare in Iceland and has similar incidence and clinical presentation. Two thirds of the patients were diagnosed in stage II or III. Even patients with distant metastases can be cured with multimodal treatment: surgery, chemotherapy and radiotherapy. There was a trend toward better survival during the study period.
Tilgangur: Wilmsæxli er illkynja sjúkdómur í nýrum og greinist yfirleitt hjá ungum börnum. Lítið hefur verið ritað um sjúkdóminn hér á landi. Tilgangur þessarar afturskyggnu rannsóknar var að kanna faraldsfræði Wilmsæxla á Íslandi, sjúkdómseinkenni, meðferð og lífs-horfur sjúklinganna. Efniviður og aðferðir: Alls greindust 17 einstaklingar, 15 börn og tveir fullorðnir (25 og 29 ára) á tímabilinu 1. janúar 1961 til 31. desember 1995, samkvæmt krabbameinsskrá Krabbameinsfélags Íslands. Meðalaldur barnanna var 33 mánuðir (staðalfrávik 19, bil 5-77 mánuðir). Upplýsingar um einkenni, niðurstöður rannsókna og meðferð fengust úr sjúkraskrám. Öll vefjasýnin voru yfirfarin og æxlin stiguð (National Wilms' Tumor Study, NWTS flokkun). Niðurstöður: Nýgengi á rannsóknartímabilinu reyndist 0,2 á 100.000 íbúa á ári og 1,0 fyrir börn yngri en 15 ára. Helstu einkenni voru fyrirferð í kviðarholi (65%) og kviðverkir eða óværð (53%). Þvagfæramyndataka var gerð í 14 tilvikum og leiddi hún alltaf í ljós æxli í nýra. Við greiningu var einn sjúklingur á stigi I (6%), sex á stigi II (35%) og sjö á stigi III (41%). Tveir voru með meinvörp í lungum (stig IV) og einn með æxli í báðum nýrum (stig V) og er hann á lífi í dag. Nýrabrottnám var gert hjá öllum, en tveir létust í kjölfar aðgerðar (skurðdauði 12%). Af hinum 15 fengu 12 geislameðferð og 12 lyfjameðferð (níu fengu bæði geisla og lyf). Meingerð æxlanna var hefðbundin, en villivöxtur (anaplasia) sást í einu tilviki og sarkmeinsútlit í öðru. Fimm ára lifun þeirra átta sem greindust á árunum 1961-1976 var 25%, en lifun þeirra sem greindust 1977-1995 var 61% (p=0,13). í heild var fimm ára lifun 42%. Ályktun: Wilmsæxli eru sjaldgæf á Íslandi, en engu að síður er nýgengi í hærri kantinum sé miðað við nágrannalönd. Flestir greinast með langt genginn sjúkdóm (stig II og III) en þrátt fyrir það er lækning möguleg, jafnvel þó fjar-meinvörp séu einnig til staðar (stig IV). Á síðari hluta rannsóknartímabilsins var tilhneiging til bættra lífslíka, sennilega vegna breyttrar meðferðar.
Original languageIcelandic
JournalLæknablaðið
Publication statusPublished - 1 Feb 1998

Other keywords

  • Nýrnasjúkdómar
  • Wilmsæxli
  • Wilms Tumor
  • Kidney Neoplasms

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