Læknablaðið - 15.02.1998, Page 30

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LÆKNABLAÐIÐ 1998; 84
Wilmsæxli á íslandi
Afturskyggn rannsókn tímabilið 1961-1995
Ingólfur Einarsson”, Tómas Guðbjartsson2’, Guðmundur Vikar Einarsson2-31,
Jóhann Heiðar Jóhannsson41, Guðmundur K. Jónmundsson", Guðmundur Bjarnason"
Einarsson I, Guðbjartsson T, Einarsson GV, Jó-
hannsson JH, Jónmundsson GK, Hjarnason G
Wilms’ tumor in Iceland 1961-1995. A retrospect-
ive study
Læknablaðið 1998; 84: 118-24
Objective: Wilms’ tumor is a malignant disease in
the kidneys that usually affects young children. In-
formation 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 lstof January 1961 to 31 st 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 abdom-
inal 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 bilater-
al tumor (stage V). Nephrectomy was performed in
Frá "Barnaspítala Hringsins, "handlækninga- og þvag-
færaskurðdeild Landspítalans, 3llæknadeild Háskóla ís-
lands, ‘"Rannsóknastofu Háskóla íslands í meinafræði.
Fyrirspurnir, bréfaskipti: Ingólfur Einarsson, Holtsgötu 17,
101 Reykjavík.
Lykilorð: Wilmsæxli, nýgengi, lifun.
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 di-
agnosis.
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 dis-
tant metastases can be cured with multimodal treat-
ment: surgery, chemotherapy and radiotherapy.
There was a trend toward better survival during the
study period.
From ''Dpt. of Pediatrics, 2|Dpt. of Surgery, 3|University of
lceland, Faculty of Medicine, 4lDpt. of Pathology. Land-
spítalinn, University Hospital, 101 Reykjavík, lceland.
Key words: Wilms’ tumor, incidence, survival.
Ágrip
Tilgangur: Wilmsæxli er illkynja sjúkdóm-
ur í nýrum og greinist yfirleitt hjá ungum börn-
um. Lítið hefur verið ritað um sjúkdóminn hér
á landi. Tilgangur þessarar afturskyggnu rann-
sóknar var að kanna faraldsfræði Wilmsæxla á
Islandi, 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. des-
ember 1995, samkvæmt krabbameinsskrá
Krabbameinsfélags Islands. Meðalaldur barn-
anna var 33 mánuðir (staðalfrávik 19, bil 5-77
mánuðir). Upplýsingar um einkenni, niðurstöð-
ur rannsókna og meðferð fengust úr sjúkra-
skrám. Öll vefjasýnin voru yfirfarin og æxlin
stiguð (National Wilms’ Tumor Study, NWTS
flokkun).