Læknablaðið - 15.01.2002, Qupperneq 21

FRÆÐIGREINAR / MIÐTAUGAKERFISÆXLI í ÆSKU
Greining, árangur meðferðar og síðkomnar
aukaverkanir æxla í miðtaugakerfi í æsku
Hilma Hólm',
Ólafur Gísli
Jónsson2,
Árni V. Þórsson12,
Bjarni Hannesson3,
Guðmundur Kr.
Jónmundsson4,
Jón R. Kristinsson4,
Ásgeir Haraldsson14
'Háskóli íslands, læknadeild,
:Barnadeild Landspítala
Fossvogi, 3Heila- og
taugaskurðdeild, Landspítala
Fossvogi, 4Barnaspítali
Hringsins, Landspítala
Hringbraut. Fyrirspurnir,
bréfaskipti: Ásgeir
Haraldsson prófessor,
Barnaspítala Hringsins,
Landspítala Hringbraut, 101
Reykjavík. Sími: 5601000;
netfang: asgeir@landspitali.is
Lykílorð: börn, miðtaugakerfi,
œxli, síðkomnar aukaverkanir.
Ágrip
Inngangur: Æxli í miðtaugakerfi er annar algengasti
illkynja sjúkdómurinn í börnum, næst á eftir hvít-
blæði. Árangur meðferðar hefur farið stöðugt batn-
andi og því eykst mikilvægi þess að greina möguleg
langtímaáhrif og síðkomna fylgikvilla meðferðar. Til-
gangur rannsóknarinnar var að finna heildarfjölda
sjúklinga á Islandi, greiningu þeirra og árangur með-
ferðar og kanna síðkomna fylgikvilla og langtíma-
áhrif meðferðar á eftirlifandi einstaklinga.
Aðferðir: Sjúklingar voru fundnir með leit í
sjúkraskrám á Sjúkrahúsi Reykjavíkur og Landspít-
alanum auk þess sem farið var yfir aðgerðarbækur á
SHR. Upplýsingar um sjúkdóminn, greiningu og
meðferð var safnað. Allir þátttakendur í rannsókn-
inni komu í viðtal og skoðun, heyrnarmælingu, blóð-
og þvagrannsóknir. Spurningalisti varðandi félags-
lega aðlögun, skólagöngu, minni, einbeitingu og líð-
an var einnig lagður fyrir þátttakendur.
Niðurstööur: Á árunum 1970-1995 greindust 57
börn á Islandi yngri en 16 ára með æxli í miðtauga-
kerfi, 30 stelpur og 27 strákar. Tveir sjúklingar með
meinvörp í heila voru útilokaðir frá frekari upp-
vinnslu. Nú eru 38 þessara einstaklinga á lífi, 19 kon-
ur og 19 karlar. Sautján sjúklingar höfðu astrocytoma
af gráðu I eða 2 en sjö sjúklingar astrocytoma af
gráðu 3 eða 4. Sjö sjúklingar höfðu greinst með
meduHoblastoma, aðrar tegundir voru sjaldgæfari.
Fjórir sjúklingar með góðkynja æxli í mænu voru úti-
lokaðir frá rannsókn. Þrír búa erlendis og þrír neit-
uðu þátttöku. Því tóku 28 einstaklingar þátt í rann-
sókninni, 15 karlmenn og 13 konur. Aldur við grein-
ingu var að meðaltali sjö ár og átta mánuðir (7:8)
EIVGLISH SUMMARY
Hólm H, Jónsson ÓG, Þórsson ÁV, Hannesson B,
Jónmundsson GK, Kristinsson JR, Haraldsson Á
Central nervous system tumours in lcelandic
children; diagnoses, treatment results and late
effects
Læknablaöiö 2002; 88: 21-7
Objective: Tumours in the central nervous system are the
second most common malignant diseases in children. With
improved treatment, the number of survivors is increasing.
Therefore, better knowledge of the long-term effects of the
disease and the therapy is needed. The aim of the current
study was to find the incidence of central nervous system
tumours in lceland, evaluate the treatment results and
study the long-term effects on the individuals.
Material and methods: Data on diagnosis and treatment
as well as demographic data were gathered from hospital
records from the Reykjavik City Hospital and The
University Hospital and operating lists at the Department
of Neurosurgery were reviewed. On survivors, physical
examination was carried out, blood tests and urine-
analysis were done and hearing was tested. Social
adaptation, school performance, memory, concentration
and general well being were studied by a questionnaire.
Results: In the years 1970-1995, 57 children, aged 16 and
younger, were diagnosed in lceland with central nervous
system tumours, 30 girls and 27 boys. Two children with
brain metastases were excluded. Of the 55 individuals, 38
are alive today, 19 girls and 19 boys. Seventeen children
had astrocytoma, grade 1 or 2 and seven had astrocytoma
of grade 3 or 4. Seven children had medulloblastoma,
other tumours were less common. Four patients with
benign tumours in the spine were excluded from the study;
three are living abroad and three refused participation.
Therefore, 28 patients were included in the further study,
15 males and 13 females. The mean age at diagnoses was
7 years and 8 months (7:8) (median 6:7 years, span 0:0-
15:11), the mean age at study was 21:4 years (median 20:2
years, span 7:6-39:9) and the mean time from diagnosis
until study was 12:8 years (median 11:5 years, span 2:5-
26:3). The mean standard deviation score for height (SDS)
was -0.63 at the time of study, five of the patients had SDS
below two. Five individuals need hormone replacement
therapy and one patient has scoliosis. Three patients have
disabilities; two of those are incapable of activities of daily
life. Three patients have hearing impairment; one of them
is also blind. Of five patients who had seizures when
diagnosed, two still have convulsions. Of 28 patients,
twelve (43%) had learning difficulties in school and ten
(36%) needed remedial teaching.
Conclusions: The incidence of central nervous tumours in
lcelandic children is comparable to what has been
reported in other countries. The results of the treatment are
similar to what has been found in the other Nordic
countries which maybe better than in other countries. The
most prominent long-term effects among the survivors are
endocrine dysfunctions and specific learning disabilities.
Other, severe long-term complications are rare but have
considerable effect on the individuals. We emphazise that
organised, long-term follow-up is essential for these
individuals, paying special attention to learning difficulties
and endocrine dysfunction.
Key words: children, central nervous system, tumour, late
effects.
Correspondence: Ásgeir Haraldsson: asgeir@landspitali.is
Læknablaðið 2002/88 21