Pediatric Surgical Oncology

189 Pages·1989·6.346 MB·English

by  D. Pellerin, H. Martelli, X. Latouche, G. Couly, F. Gasnier (auth.), Professor Lewis Spitz PhD, FRCS, Primarius Professor Dr. Peter Wurnig, Priv.-Doz. Dr. Thomas A. Angerpointner (eds.)

Progress in Pediatric Surgery
Volume 22
Executive Editors
M.W.L.Gauderer . W.Ch.Hecker
J.Prevot . L.Spitz
U.G.Stauffer . P.Wurnig
SeniorEditor
P.P.Rickham
Editors
A.H.Bill, Seattle/USA J.A.Haller, Baltimore
J.Boix-Ochoa, Barcelona M.Kasai, Sendai
C.C.Ferguson, Winnipeg O.Knutrud, Oslo
S.L.Gans, Los Angeles J.Lister, Liverpool
R.K.Ghandi, Bombay N.A.Myers, Melbourne
Assistant Editor
T.A.Angerpointner, Munich
Pediatric
Surgical Oncology
Volume Editors
L.Spitz, London
P.,Wurnig, Vienna
Th.A. Angerpointner, Munich
With 78Figures and 44Tables
Springer-Verlag
Berlin Heidelberg NewYork
London Paris Tokyo
ProfessorLEWISSPITZ, PhD,FRCS
Nuffield ProfessorofPediatricSurgery
InstituteofChildHealth
30 GuilfordStreet
London WCIN lEH, England
PrimariusProfessorDr. PETERWURNIG
ChirurgischeAbteilungdes MautnerMarkhof'schen
KinderspitalsderStadtWien
Baumgasse75,1030Vienna, Austria
Priv.-Doz. Dr. THOMAS A.ANGERPOINTNER
Kinderchirurgische Klinik
imDr. von HaunerschenKinderspital
LindwurmstraBe4,8000Munich2,
FederalRepublicofGermany
Volumes 1-17ofthis serieswere published
byUrban&Schwarzenberg, Baltimore-Munich
ISBN-13:978-3-642-72645-3 e-1SBN-13:978-3-642-72643-9
DOl: 10.1007/978-3-642-72643-9
LibraryofCongressCataloging-in-PublicationData
Pediatricsurgicaloncology1volumeeditors,L.Spitz,P.Wurnig,Th.A.Angerpointner.
p. cm.- (Progressinpediatricsurgery;v.22)
Includesbibliographiesandindex.
1.Cancer- Surgery.2.Tumorsinchildren- Surgery.1.Spitz,Lewis.II.Wurnig,Peter.
III.Angerpointner,Thomas.IV.Series.
[DNLM:1.Neopiasms- ininfancy& childhood.2.Neoplasms- surgery. WIPR677KA
v.22/QZ268P371] RD137.A1P7vol.22 [RD651] 617'.98s- dc19 [618.92'994059]
DNLMIDLC 88-24765
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©Springer-VerlagBerlinHeidelberg1989
Softcoverreprintofthehardcover1stedition1989
Theuseofregisterednames,trademarks,etc.inthispublicationdoesnotimply,evenintheab
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Contents
Congenital Soft Tissue Dysplasia: A New Malformation Entity
and Concept. D.PELLERIN, H.MARTELLI, X.LATOUCHE, G.COULV,
andF.GASNIER. With34Figures 1
Monoclonal Antibodies as Targeting Agents for Cytotoxic Com
pounds In Vivo: A Current Assessment. J.T. KEMSHEAD, D.H.
JONES, L.LASHFORD, J.PRITCHARD, 1.GORDON, F.BREATNACH, and
H.B.CoAKHAM. With3Figures 30
BulkDisease as the MajorProblemin the CureofPaediatricSar-
comas. P.N.PLOWMAN. With3Figures .. . . . . . . . . . . .. 45
TheCare ofthe ChildFacingDeath. R LANSDOWN 64
HepatobiliaryTumoursofChildhood: InvestigationandManage-
ment. A.D.JovCEand E.RHoWARD. With10Figures 69
Wilms'Tumour: Trials andTribulation. D.C.S.GOUGH 94
The Place ofSurgery in the Management ofGerm CellTumours
inChildhood. A.BARRET. With1Figure . . . . . . . 106
SecondPrimaryTumoursin Children. J.E.KINGSTON 113
FibrousTissueTumours. YHAVASHI,L.SPI1Z,E.KIELV,J.PRITCHARD,
andJ.R PINCOTT. With9Figures . . . . . . . . . . . . . . . ., 121
Vascular Access. S.J.K.HoLMES, E.M.KrnLV, and L.SPITZ. With
1Figure 133
Surgeryfor Neuroblastoma. E.M.KIELV 140
Implantation of a Silastic Balloon for Reduction of Radiation
Injuries of the Bowel in Two Children with Neuroblastoma.
R.HAWLICZEK, W.PUMBERGER, W.GEISSLER, and P.WURNIG. With
7Figures 146
VI Contents
Surgical Aspects in the Treatment of Soft Tissue Sarcomas: A
PreliminaryNote. D.BuRGERandJ.TREUNER. With2Figures .. 155
NewAspectsintheTreatmentofChildhoodRhabdomyosarcoma:
Results of the German Cooperative Soft-Tissue Sarcoma Study
(CWS-81). J.TREUNER, J.KOHL, J.BECK, J.RrrrER, H.J.SPAAR,
H.JORGENS,M.KEIM,P.WEINEL,W.BRANDEIS,A.REITER,D.BORGER,
and D.NIETHAMMER. With8Figures 162
SubjectIndex . . . . . . . . . . . . 177
List of Editors and Contributors
Editors
Angerpointner, T.A., Priv.-Doz. Dr., Kinderchirurgische Klinik im
Dr. von Haunerschen Kinderspital der UniversitatMunchen, Lind
wurmstraBe4, D-8000Munchen2
Gauderer, Michael, W.L., M.D., University Pediatric Surgical Asso
ciates, 2101 Adelbert Road, Cleveland, OH44106, USA
Hecker, W.Ch., Prof. Dr., Kinderchirurgische Klinik im Dr. von
Haunerschen Kinderspital der UniversiHit Munchen, Lindwurm
straBe4, D-8000Munchen2
Prevot, J., Prof., Clinique Chirurgical Pediatrique, Hopital d'Enfants
deNancy, F-54511 Vandrevre Cedex
Rickham, P.P., Prof. Dr. M.D., M.S., F.R.C.S., F.R.C.S.I.,
F.R.A.C.S., D.C.H., F.A.A.P., Universitatskinderklinik, Chirur
gische Abteilung, SteinwiesstraBe75, CH-8032Zurich
Spitz, L., Prof., PhD. FRCS, Nuffield Professor ofPediatric Surgery,
Institute of Child Health, University ofLondon, Hospital for Sick
Children, Great Ormond Street, 30 Guilford Street, GB-London
WC1N lEH
Stauffer, U.G., Prof. Dr., Universitatskinderklinik, Kinderchirur
g~scheAbteilung, SteinwiesstraBe75, CH-8032Zurich
Wurnig, P., Prof. Dr., Kinderchirurgische Abteilung des Mautner
Markhof'schenKinderspitals, Baumgasse75, A-1030Wien
VIII ListofEditorsandContributors
Contributors
Youwillfindtheaddressesatthebeginningoftherespectivecontribution
Barret, A. 106 Kiely, E.M. 121,133,140
Beck, J. 162 Kingston, J.E. 113
Brandeis, W. 162 Kuhl, J. 162
Breatnach, F. 30 Lansdown, R. 64
Burger, D. 155, 162 Lashford, L. 30
Coakham, H.B. 30 Latouche, X. 1
Couly, G. 1 Martelli, H. 1
Gasnier, F. 1 Niethammer, D. 162
Geissler, W. 146 Pellerin, D. 1
Gordon, I. 30 Pincott, J.R. 121
Gough, D.C.S. 94 Plowman, P.N. 45
Hawliczek, R. 146 Pritchard,J. 30, 121
Hayashi, Y. 121 Pumberger, W. 146
Holmes, S.J.K. 133 Reiter, A. 162
Howard, E.R. 69 Ritter, J. 162
Jones, D.H. 30 Spaar, H.J. 162
Joyce, A.D. 69 Spitz,L. 121, 133
Jurgens, H. 162 Treuner,J. 155,162
Keirn, M. 162 Weinel, P. 162
Kemshead, J.T. 30 Wumig, P. 146
Congenital Soft Tissue Dysplasia:
A New Malformation Entity and Concept
D.Pellerin, H.Martelli, X.Latouche, G.Couly, andF.Gasnier
Until recently, managementofpathologicalconditionsofsofttissuewasscattered
among subspecialities according to the clinical presentation: dermatologists, vas
cularsurgeons,orthopaedists,plasticsurgeons,neurologists,neurosurgeons,and,
of course, paediatric surgeons all took their turn. The pathological conditions
were termed giant hamartomas, segmentary gigantism, hypertrophic limbs,
phacomatosrs, angiodysplasia; or were grouped in syndromes such as Parkes
Weber or Klippel-Trenaunay. Hardly anything was known about their embryo
logical or physiopathological development, and consequently complete cure was
impossible.
In the light of some privileged observations, and because of a better under
standing of the embryological mechanisms in the development of ectoderm and
mesoderm, we propose to regroup these syndromes into a new entity, congenital
soft-tissue dysplasia (CSTD). This entity is based on a histological characteristic
commonto allthe aboveconditions, namelyovergrowthofnormaldifferentiated
cells. Alldiseasesincludedintheentitysharethesametypeofnaturalhistoryand
have someimportanttherapeuticaspectsincommon.
Before going into the historical review, we will show features in a 4-year-old
boy(Fig.1)thatillustratethisentity,withitswidevarietyofclinicallesionslinked
byacommonhistologicalaspect. Allhislesionsaresecondaryto abnormaldevelop
ment of ectodermal and mesodermal tissues. There is an overgrowth of both
thighs andthe leftleg, partialhypertrophy withgigantismofthefeet, andasmal
ler area of overgrown soft tissue on the'left side of the thorax. Such anomalies
may be diffuse or localised. On the back, the hamartomatous hypertrophy may
give rise to the typical bison-like appearance (Fig.2). Dysplasiacan belimitedto
a unger or atoe. Reflecting the ectodermal anomalies, the covering skinis often
abnormalwithsuperficialangiomatosis,pigmentednaevus,or,occasionally,pilose
naevi. Dermo-epidermal lymphangiectasis may result in chronic lymphorroea,
with high risk ofinfection. In the case ofbony hypertrophy, the bone's structure
is radiologically normal. Whatever the clinical appearance, the pathology is al
waysthatofnormaldifferentiatedcells. Softtissuesarealwaysconstitutedofnor
mal mesodermal or ectodermal components. The abnormality lies in either the
overgrowth or the abnormal localisation of these normal components. Here we
canreallytalkof"polymorphichamartoma",withcombinationsoflipomatoustis
sues, haemangiomas orlymphangiomas, oftenwithlarge fibrous stromal compo
nents (Figs.3-5).
Dept.ofPaediatricSurgery,H6pitaldesEnfantsMalades,149ruedeSevres,75015Paris,France
ProgressinPediatricSurgery,Vol.22
Ed.byL.Spitzetal.
©Springer-VerlagBerlinHeidelberg1989
2 D.Pellerinetal.
2
Fig.I. Four-year-old boy with diffuse overgrowth of the thighs and the left leg, partial distal
gigantism,andhamartomaoftheleftsideofthetrunk
Fig.2.Bison-likehamartomaofthebackwithovergrownrightupperlimb
Historical Review
The clinical aspects ofCSTD have been known for a long time. Early on, some
caseswereshownorpublished ascuriositiesormonstrosities. Twocaseswerede
scribed preciselyby Chassaignac in 1858to the Societe des Chirurgiens de Paris,
in these words: 'The limbs onthe leftside are those ofaperson ofnormal build;
those on the right seem to belong to a giant' [7] (Fig.6). Ten years later, Trelat
and Monad [47] published in the Archives Generales de Medecine a clinical de
scription of 'l'hypertrophie unilaterale partielle au totale du corps' (partial or
total unilateral hypertrophy of the body): 'Sometimes of limited extent, con
genitalhypertrophymayaffectthewholesystemofanorganoralarge portionof