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J Oral Maxillofac Surg

60:762-770, 2002
Ameloblastoma in Children
R.A. Ord, DDS, MD, FRCS, MS,* R.H. Blanchaert, Jr, DDS, MD,
N.G. Nikitakis, DDS, and J.J. Sauk, DDS, MS
Purpose: The purpose of this report was 1) to report the experience of the University of Maryland,
Department of Oral and Maxillofacial Surgery (OMS Department) in the treatment of ameloblastoma in
childrenand2)toreviewtheworldliteratureonthetreatmentofameloblastomainchildrenfrom1970
to 2001.
Methods and Materials: This study rst reviews the experience of the OMS Department of the
UniversityofMarylandwithameloblastomasinchildrenandthenreviewstheliteratureonthissubject.
Therstpartofthestudywasundertakenbyaretrospectivechartreviewofallpatientswithadiagnosis
of ameloblastoma in the OMS Department between May 1991 and December 1999. The literature on
ameloblastoma in Western societies and Africa was separately reviewed from 1970 through 2001.
Reportsearlierthan1970werenotreviewed,asthehistologicdiagnosisofameloblastomawasnotwell
dened before that period.
Results: In the Maryland series, 11 patients under the age of 20 years with ameloblastoma were treated.
Eightpatientswereseenprimarily,and3presentedwithrecurrentlesions.Theaverageagewas15.5years;
5of11patientswereblack,and9of11tumorswereunicysticameloblastomas.Theliteraturereviewshowed
85childrenintheWesternreportsand77reportedfromAfrica.Theaverageageswere14.3and14.7years,
respectively,butunicysticameloblastomasaccountedfor76.5%oftheWesternandonly19.5%oftheAfrican
children,withanincreasedfrequencyofoccurrenceinthemandibularsymphisisinAfrican(44.2%)versus
Western(5.8%)patients.Analysisofrecurrenceafterenucleationofunicysticameloblastomasin20children
followedatleast5yearsoruntilrecurrenceshowedarecurrenceof40%.
Conclusions: Ameloblastomas in children differ from adults, with a higher percentage of unicystic
tumors. African children appear to resemble the adult pattern. Although enucleation has been claimed
to give acceptable recurrence rates in unicystic ameloblastoma, there are no large series with long
follow-up in children. The histologic pattern that exhibits mural invasion in unicystic ameloblastoma
suggests that more aggressive surgery is necessary.
2002 American Association of Oral and Maxillofacial Surgeons
J Oral Maxillofac Surg 60:762-770, 2002
The management of pathologic lesions of the jaws is gery.Abroadspectrumofbiologicbehaviorisrepre-
central to the specialty of oral and maxillofacial sur- sented in the numerous types of lesions that are en-
countered. Difculty exists in determining the most
appropriateformoftherapyforbenigntumorsofthe
*Professor, Oral and Maxillofacial Surgery, Baltimore College of
jaws.Theexistingliteraturefailstoprovidedocumen-
Dental Surgery, University of Maryland Medical Systems, Greene-
tationofbenigntumorsofthejawsinthesamewayas
baum Cancer Center, Baltimore, MD.
malignant tumors are reported. The primary reasons
Assistant Professor, Oral and Maxillofacial Surgery, Baltimore
for this are the lack of standardization in data collec-
CollegeofDentalSurgery,GreenebaumCancerCenter,University
tion, including excision terminology, the need for
of Maryland Hospital, Baltimore, MD.
lengthy follow-up, and the absence of a nationwide
Graduate Research Assistant, Resident, Oral Pathology, Balti-
mandatory database for benign tumors as exists for
more College of Dental Surgery, Baltimore, MD.
malignant disease.
Professor,OralPathology,BaltimoreCollegeofDentalSurgery,
Ameloblastomaisthemostcommonaggressivebe-
Baltimore, MD.
nigntumorofthejaws.Signicantdecienciesinthe
Address correspondence and reprint requests to Dr Ord: Uni-
versity of Maryland Dental School, Oral and Maxillofacial Surgery
Department, 666 W Baltimore St, Room 3-G-21, Baltimore, MD
21201; e-mail: rord@umm.edu
published literature exist with regard to incidence,
management, and recurrence. No controlled studies
have been reported. This report documents the clin-
2002 American Association of Oral and Maxillofacial Surgeons
0278-2391/02/6007-0007$35.00/0
doi:10.1053/joms.2002.33242
icalexperienceoftheDepartmentofOralandMaxil-
lofacial Surgery of the University of Maryland in re-
gard to ameloblastoma in children. This report adds
762
ORD ET AL 763
Table 1. PEDIATRIC AMELOBLASTOMAS
Age Morphologic Follow-
Case (yr) Gender Race Site Type Comments Treatment Up Recurrence
1 16 M W Mand Sym Unicystic Involves Marginal resect 7 yr No
lower border
and soft tissue
2 16 F B Mand Sym Unicystic Marginal resect 6 yr No
3 12 M W Angle/Vert Unicystic Enuc 5 mo LFU
Ram
4 14 F B Angle Multicystic Involves Resect primary 5 mo LFU
lower border BG
and soft tissue
5 17 M W Angle Solid Enuc 1 yr 6 mo Yes,
Radical
Resect and
bular ap
6 13 F W Angle Unicystic Enuc 1 yr 6 mo No
7 15 F B Maxillary Sinus Unicystic Gorlins Synd Enuc 6 mo No
arose in OKC
8 18 F B Mand Sym Unicystic Involves Resect and 6 mo No
lower border DCIA ap
and soft tissue
Abbreviations: LFU, lost to follow-up; Mand, mandible; Sym, symphysis; Enuc, enucleation; Resect, resection; Synd, syndrome; BG, bone
graft;Vert,vertical;Ram,ramus;Radicalresect,resectionofmandibleplusmasticatormusclestoskullbase;OKC,odontogenickeratocyst;
DCIA, deep circumex iliac artery microvascular free ap.
11 new cases of ameloblastoma in a pediatric popu-
lation to the literature and reviews the clinicopatho-
logicdifferencesbetweenthistumorinchildrenand
adults and between the Western and African experi-
ence with this tumor.
Methods and Materials
All cases of ameloblastoma (38 cases) seen in the
Department of Oral and Maxillofacial Surgery be-
tweenMay1991andDecember1999werereviewed.
Elevenpediatricpatientswereseen,ofwhom8were
treated primarily (Table 1) and 3 represented recur-
rentlesionsinadultswhoseameloblastomawasorig-
inally treated in childhood (Table 2). Data extracted
includedage,gender,race,site,histologictype,treat-
ments, and outcome. One patient, a 19-year-old
woman with a keratoameloblastoma of the maxilla,
was excluded as having a varient tumor.
In addition, a literature search for all patients 20
years old or younger with ameloblastoma reported
since 1970 was undertaken. Western and African se-
ries were analyzed separately.
Results
Of 38 patients, 11 (28.9%) were younger than 20
years when they were originally treated. Although
Table 2. RECURRENT AMELOBLASTOMAS INITIALLY TREATED IN CHILDHOOD
Primary
Treatment
Age Morphologic Secondary Follow-
Case (yr) Gender Race Site Type Comments Treatment Up (yr) Recurrence
9 14 319 M B Body Unicystic Involves soft Enuc3Seg. 1 No
Mand tissue Resect
10 18325 F W Body Multicystic Reccur at Marg 3Seg. 2 No
Mand lower Resect Resect
border
11 19333 F W Angle/Vert Unicystic Soft tissue Enuc 3Rad. 3 No
Ramus skull base Resect
Mand
Abbreviations:Age:14319,initialtreatmentat14yearsandrecurredatage19years;Mand,mandible;Vert,Vertical;Seg,segmental;Enuc,
enucleation; Marg, marginal; Rad, radical; Resect, resection of mandible matricator muscles to skull base.
Treatment abbreviations: Enuc3Seg. Resection, enucleation (initial therapy)3segmental resection (treatment of recurrence).
764
thisgureseemshigh,thisismostprobablyaharvest-
ing effect from the nature of tertiary referral to a
university center. In support of this, the series in-
cluded 10 maxillary lesions (26.3%) and 8 recurrent
lesions (21%), which indicates referral of the more
challenging cases. The mean age of the 38 patients
was44.2years(range,12to93years),andthemean
age of the children at presentation was 15.5 years
(range, 12 to 19 years). Eighteen of the 38 patients
were black (47.5%), and 5 of the 11 children were
black (45.5%).
In the pediatric group, there were 4 boys and 7
girls, and 10 of 11 tumors were found in the mandi-
ble.Theangle/ramuswasthemostcommonsite(5of
11 [45.5%]), followed by the symphysis (3 of 11
[27.3%]),body(2of11[18.2%]),andmaxilla(1of11
[9%]).Eightof11ofthelesions(72.7%)wereunicys-
tic,and3(27.3%)wererecurrenttumors.Therewere
a total of 10 unicystic tumors in the 38 patients, but
only 2 of 27 adult patients (7.8%) had unicystic tu-
mors.Theremainderoftheclinicalndingsaresum-
marized in Tables 1 and 2. The 8 patients who pre-
sentedprimarilytoourunitaspediatricpatientswere
analyzed separately from the 3 patients who pre-
sented as recurrences (2 adults and 1 child).
All8patients(Table1)presentedwithfacialswell-
ing; 6 (cases 1 and 3 to 7) had associated unerupted
teethandwerediagnosedasdentigerouscysts(Figs1,
2). Three of the 8 unicystic amloblastomas were
treated with marginal resection (retaining the lower
border of the mandible) or segmental resection be-
causeoftheirsizeorinvolvementoflowerborderor
softtissues(Fig3).In1patientwhohadenucleation,
mural involvement was found in histologic examina-
tionoftheentirespecimen,andfurtherresectionwas
suggestedbutrefused.Incase5,aradiologicallyuni-
cystic lesion was ennucleated elsewhere with a clin-
icaldiagnosisofdentigenerouscyst.Thenaldiagno-
siswasameloblastomasolidtype.Thepatientrefused
further surgery; 18 months later, a recurrent lesion
extendingtothecondyleandinvolvingthepterygoid
muscles was widely resected. Follow-up has proved
difcult; only cases 1 and 2 have been followed for
more than 5 years, cases 3 and 4 were lost before 6
months, and the other patients have only a short
follow-up.Ourpediatricameloblastomapatientswho
had recurrance as in adults (Table 2) have a longer
follow-up.Allweretreatedwithsegmentalresection;
1 patient who had recurrence at the skull base re-
quired radical resection with soft tissue (Fig 4).
Review of the Literature
Ameloblastomaisuncommoninchildren.Themost
commonlyquotedarticleregardingameloblastomais
areviewof1,036ameloblastomasinwhichtheaver-
AMELOBLASTOMA IN CHILDREN
FIGURE 1. A, A 12-year-old boy with marked swelling of the right
face.B,Panographshowinglargecysticlesionofverticalramusand
angle with unerupted molar tooth.
age patient age is 38.9 years, with only 2.2% (19 of
858)under10yearsoldand8.7%(75of858)between
10and19yearsold.
1
Thisreport,however,waspub-
lished in 1955, when adenoameloblastomas and
ameloblastic bromas were included as ameloblasto-
mas and before the histologic characteristics for
ameloblastomas in cysts had been delineated. We
thereforehavenotreviewedarticlespublishedbefore
1970, to eliminate histologic confusion. Kessler and
Dominquez
2
described 8 of 92 patients (8.7%) less
than16yearsofage,whereasKhan
3
found38of311
ameloblastomas (12.2%) in patients younger than 19
years.Mehlischetal
4
found16of126patients(12.6%)
less than 20 years old but only 1 patient under 10
yearsofage,andSandleretal
5
found15%oftheir87
patientstobeunder20yearsoldand3%tobeunder
10 years old.
Large series of Asian patients show a higher per-
centage of children with ameloblastoma to be
younger than 20 years: 19 of 104 Japanese patients
(18.2%)
6
and 29 of 147 Thai patients (19.7%).
7
765 ORD ET AL
InAfrica,ameloblastomaismoreprevalent.Reports
from Nigeria show an increased frequency of amelo-
blastomasinchildren;30of206casesunder18years
(14.6%),
8
16of70casesunder18years(22.8%),
9
and
19of76casesunder20yearsofage(25%).
10
Similar
high rates are reported from Zimbabwe, with 20 of
117patients(17.1%)under18yearsofage.
11
Whether
this increase represents the younger population in
developing countries or is a true racial difference in
tumor incidence is not known.
The literature review showed little differences in
agebetweentheWesternandAfricanpatients.How-
ever,thesiteofpredilectionforAfricanpatientswas
the mandibular symphysis in 44.2%, as previously
reportedinadultseriesfromAfrica.Moststrikingwas
thedifference inmorphologictype (74.3%wereuni-
cystic in Western children and 19.5% were unicystic
in African children) (Table 3). It appears that the
Africanchildrenmorecloselyresembletheadultpat-
terns of ameloblastoma in their countries.
FIGURE2.A,A14-year-oldgirl
with swelling in the left angle of
the jaw. B, Panograph showing
dentigerouscystwithunerupted
second and third molars. C,
High-power view showing mural
ameloblastomawithislandsoftu-
mor in the brous capsule.
Discussion
years,
It is impossible to estimate the true incidence of
childhood ameloblastoma because different authors
havedenedthepediatricpopulationupperagelimit
at differing time periods as less than 20 years,
10
18
8,9,11
16years,
2
and15years.
12
Also,manyofthe
earlier publications contain data that would now be
regarded as histologically inaccurate. It is also likely
thatonlyasmallsamplingoftheactualincidencehas
been reported. An article that supposedly reports 7
casesofameloblastomaundertheageof9years
13
was
published in 1962, but 2 of these cases were amelo-
blastic bromas and 1 was an odontoameloblastoma.
Gurneyscaseofa2-month-oldinfantwithamaxillary
ameloblastoma
14
is also included despite the lack of
histology and an original histologic diagnosis of mel-
anoma, which indicates this lesion probably repre-
sented a pigmented neuroectodermal tumor of in-
fancy. It was not until 1970, when Vickers and
766
FIGURE3.A,Panographof16-year-oldboywithlargecysticlesion
of anterior mandible with loosening and loss of incisor teeth. B,
Low-powerviewofunicysticlesionshowingtransitionfromcysticlining
to plexiform ameloblastoma involving lining and lumen only.
Gorlin
15
denotedthe4histologicfeaturesthatdene
ameloblastoma,thatanyconsensusontheearlydiag-
nosisofthistumorinrelationtocystsappears.There-
fore, we have chosen to discard all cases reported
before 1970 in this literature review (Table 3). This
reviewshowsanaverageageof14.3years(Western)
and 14.7 years (African) and conrms that less than
10% of childhood cases occur under the age of 10
years.
3-7
In adults, the reported gender ratio for ameloblas-
tomais1:1.InWesternchildrenandinthisreport,it
is1:1.2formale/female.InAfricanchildren,thereisa
reported male predominance of 1.4:1. The mandible
is the site of 85% of adult ameloblastoma, with the
thirdmolarregionbeingthemostcommonsite.How-
ever,inlargeseriesfromNigeria,
16-18
themandibular
symphysis is the predominant site. This pattern is
mirrored in children with 72% angle and 5.8% sym-
physisinvolvementinWesternchildren(Table3)and
with 27.3% angle and 44.2% symphysis involvement
inAfricanchildren.Although15%ofadultcasesoccur
in the maxilla, Regezi and Sciubba
19
and others have
reported incidences of 5%
6
to 12%.
16,20
In children,
approximately7%ofcasesaremaxillary:intheWest-
ernseries,only6casessince1970(1ina14-year-old
AMELOBLASTOMA IN CHILDREN
girl,
21
our case 7; a 12-year-old boy,
22
and 3 other
casesreportedbyTsaknisandNelson,
23
whofound3
of 24 [13%] maxillary ameloblastoma in children).
Ackerman et al
24
reviewed 57 cases of unicystic
ameloblastoma, approximately half of which oc-
curredaftertheageof20years,andonly3cases(6%)
were found in the maxilla. However, Shteyer et al
25
reviewed mural ameloblastoma, which they dene
asanameloblastomaarisinginthewallofdentigerous
cyst,andfound84cases,ofwhich11(14%)occurred
in the maxilla.
Theunicysticameloblastomaisthemostcommonly
seenanatomicforminWesternchildren(76.5%)(Ta-
ble3),althoughthefrequencyofunicysticameloblas-
tomaisonly5%inlargeseriesofadults
25
and15%of
allameloblastomas.
24
Inaseriesof33unicysticamelo-
blastoma, 42% occurred in the second decade,
26
and
in another report of 14 unicystic ameloblastoma, 10
patients (70%) were younger than 20 years.
27
It is
interesting to speculate whether this frequency in
children represents a continuum in development
fromodontogeniccysttocystwithameloblastomain
the lining only (a preinvasive phase of tumor devel-
opment)tounicysticameloblastomawithmuralinva-
sion and eventual bone invasion as a multicystic or
FIGURE4.Magneticresonanceimageof33-year-oldwomanshow-
ingcysticrecurrenceofameloblastomainvolvingthemedialpterygoid,
lateralpterygoid,andskullbase.Theoriginallesionwasenucleatedat
age 19 years.
11-20
ORD ET AL 767
Table 3. LITERATURE REVIEW FROM 1970 TO THE
PRESENT
Western Reports African Reports
Age (yr)
10 8 3
76 (range, 4-20) 33 (range, 5-18)
Unknown 1 (average, 14.3) 41 (average, 14.7)
Gender
Male 39 45
Female 43 32
Unknown 3
Race
Black 26 77
White 37
Asian 1
Unreported 21
Type
Solid/multilocular 16 46
Unicystic 64(74.3%) 15(19.5%)
Unreported 5 16
Site
Symphysis 5(5.8%) 34(44.2%)
Body 13 16
Angle 60(72%) 21(27.3%)
Maxilla 6 2
Unreported 1 4
NOTE. Data from References 2-4, 8-12, 16-18, 21-27, 35, 36, 40,
46-51, and present series.
solid ameloblastoma as the patient ages. The slow
growthofthistumorcouldindicatethatmanyadults
diagnosed in their mid-30s had tumors since child-
hood.
30
It is certainly true that multicystic/solid
ameloblastomaisrareinchildren,withonereviewof
pediatric jaw tumors nding 1 case in 10 years.
28
In
African children, the tumor is more like the adult
variety, with most pediatric ameloblastoma being
solid despite their cystic radiologic appearance.
29
However, this may be due to late diagnosis, with
larger tumors showing increasing bone involve-
ment.
11
Theimportanceoftheunicysticameloblastomalies
inthebetterprognosisthatthelesionissaidtohave
anditsimplicationsfortreatment.Gold
30
hasempha-
sizedtheimportanceofthehistologiccriteriaofVick-
ers and Gorlin
15
and Gardner
31
in the diagnosis of
unicysticameloblastoma.Unfortunately,somereports
classifyunicysticameloblastomasolelyonthebasisof
radiologic appearance, but septation or a multicystic
lesionmaybeseenonplainlms,notjustaunilocular
radiolucency in unicystic ameloblastoma. In Khans
article,
3
10 unicystic ameloblastomas of 36 (27.8%)
were multilocular on radiography. The diagnosis of
unicystic ameloblastoma usually relies on retrospec-
tive examination of both histologic examination and
radiographs.
33
The radiologic appearance is fre-
quentlythatofadentigerouscyst,butthepresenceof
root resorption should alert the clinician to the pos-
sibility of ameloblastoma. In the 85 cases reviewed
(Table 3), only 14 cases could be assessed for this
radiologic sign and 7 (50%) showed root resorption.
The resorption of roots is said to be more frequent
with ameloblastoma than odontogenic keratocysts.
34
Treatment of ameloblastoma in children is compli-
cated by 3 factors: 1) the continued facial growth,
differentbonephysiology(greaterpercentageofcan-
cellous bone, increased bone turnover, and reactive
periosteum), and presence of unerupted teeth; 2)
difcultyininitialdiagnosis;and3)predominanceof
the unicystic type of ameloblastoma.
In the treatment of children, some advocate only
enucleation
35
or minimal treatment,
36
while Fung
37
statesthatintheyoungerpatient,therewillbemore
cancellousbonewiththelesiongrowingmorerapidly
withextensivedestruction,makingsurgerymoredif-
cult and demanding.
dren,
Diagnosisiscomplicatedbythefactthatthemajor-
ity of lesions present radiologically as dentigerous
cysts.
31,38
InRobinsonandMartinezsstudy,
27
70%of
unicystic ameloblastomas were associated with an
impacted tooth, and another series showed 83%,
25
although 1 series showed only 19% related to un-
erupted teeth.
24
In our series, 6 of 8 children (75%)
were initially diagnosed as having dentigerous cysts.
This can lead to initial treatment with marsupuliza-
tion, curretage, or enucleation. Only when the com-
plete lining is examined is the denitive diagnosis
made, and then decisions regarding whether further
therapyisindicatedhavetobemade.Akermanetal
24
advocated enucleation for diagnosis because inci-
sional biopsy is subject to sampling error and may
miss an area of ameloblastic change. Gardner and
Corio
39
excludedonecasefromtheirseriesthatwas
marsupulized and diagnosed as a dentigerous cyst,
whichrecurredasaplexiformameloblastoma4years
later. Although marsupulization has been advocated
intheliteratureasaconservativeprocedureinchil-
40,41
these cases usually show multiple recur-
rences with removal of more bone and teeth.
40
In-
deed,2of4childrentreatedwithmarsupulizationor
marsupulization and currettage primarily eventually
underwentresection.
41
Thisisnotsurprisingasother
authors have described tumor cells remaining in the
cancellousboneaftermarsupulization,promotingre-
currence.
42,43
Themajorityofameloblastomainchildrenareuni-
cystic (Table 3; 74.3%). The literature has suggested
these tumors have much lower rates of recurrence
with enucleation than the multicystic or solid types.
Rates of 10%,
25
18%,
26
and 25%
27
recurrence after
initial enucleation have been reported for unicystic
ameloblastomas,whichfulllVickersandGorlinscri-
teria,
15
andareviewoftheunicysticplexiformtypeas
768 AMELOBLASTOMA IN CHILDREN
Table 4. RECURRENCE AFTER ENUCLEATION OF UNICYSTIC AMELOBLASTOMA IN WESTERN CHILDREN
Follow-Up (yr) Average Follow-Up (yr) Total No. of Cases No. of Recurrences Time to Recurrence (yr)
2 5.9 32 8(25%) Average, 4.5
4 6.7 26 8(30.7%) Range, 1 to 14
5 7.2 20 8(40%)
NOTE. Data from References 2-4, 8-12, 16-18, 21-27, 35, 36, 40, 46-51, and present series.
described by Gardner and Corio
39
showed 10.7% re-
currencerates.However,thereareseveralcaveatsto
be examined in interpreting these data. First, these
series included children, young adults, and adults.
Second,theadequatetimeforfollow-uphasnotbeen
determined. Kessler and Dominquez
2
showed 3 of 7
children who had unicystic ameloblastomas enucle-
ated had recurrence after 1 year. In Robinson and
Martinezsstudy,
27
3of11patentsfollowedformore
than 5 years had recurrence and 3 of 14 patients
followed by Leider et al
26
for 2 to 5 years had recur-
rence.Thelatterauthors
26
alsoreportedarecurrence
at13yearssimilartoourcase11,whohadrecurrence
at14years.Itisverydifculttondalargeseriesof
childrenfollowedforasuitablelengthoftime.Fourof
our8primarycaseswerealreadylosttofollow-upby
6months.Inourliteraturereviewofunicysticamelo-
blastomas in children who were treated by enucle-
ation(Table4),therewere8recurrences(25%)inthe
32childrenfollowedforaminimumof2yearsoruntil
thetumorrecurred.Whenthetimetorecurrencewas
analyzed, the average time was 4.5 years. However,
only 20 children were followed for a minimum of 5
years or until recurrence, and in this group, the 8
recurrencesrepresentarecurrencerateof40%.Other
authors have also reported higher recurrence rates;
Sampson and Pogrel
43
reported 100% (4 of 4 cases)
recurrence in unicystic ameloblastoma after curet-
tage,whichtheyusesynonymouslywithenucleation.
tion,
Third,istheproblemthatthetermunicysticamelo-
blastoma encompasses several histologic growth
forms or patterns. Ackerman et al
24
classify unicystic
ameloblastoma into 4 types. Type I and II show in-
traluminal proliferation or epithelial involvement
with no proliferation beyond the basement mem-
braneintotheconnectivetissuewall.TypeIIIshows
involvement of the connective tissue wall, mural
ameloblastoma,byinvadingepitheliumorbyislands
ofepithelium(typeIV).Severalauthorshavethought
these lesions with mural invasion should be treated
aggressively with marginal or segmental resec-
24,38
whereasKhan
3
thoughtwidermarginswith
uninvolved bone should be achieved when there
weresignicantmuralnestsorameloblastomaprolif-
erating through the cyst wall. It would seem logical
that unicystic ameloblastomas with involvement of
theconnectivetissuecapsulearemorelikelytohave
residualtumorislandsremainingafterenucleation.It
can be theorized that the gures for recurrence rep-
resentonlythosetumorswithintramuralinvolvement
and that unicystic ameloblastoma type I and II are
100% cured by enucleation.
Gardner
38
states that only a small percentage of
unicysticameloblastomasextendoutsidethecapsule,
whichwouldcorrelatewiththelowrecurrencerate.
However, Leider et al,
26
noted that 81% of unicystic
ameloblastoma exhibited ameloblastic proliferation
into the cyst wall, whereas Ackerman et al
24
found
that28of57cases(49%)showedmuralinvolvement.
It also is important to realize that unicystic amelo-
blastomaisvirtuallyseenonlyinthemandiblein92%
to 100% of cases.
24,39
There are very few reports of
unicystic ameloblastoma in the maxilla. Tsaknis and
Nelson
23
found 1 of 24 maxillary ameloblastomas to
beintramural,andtherstplexiformunicysticamelo-
blastomaofthemaxillawasnotreporteduntil1987.
22
Maxillary ameloblastoma has a well-earned sinister
reputation, and in 1 recent publication, 2 of 13 pa-
tients (15%) died of disease, with 1 other patient
(7.5%)alivewithdisease.
44
Thereareinsufcientdata
intheliteraturetodeterminewhetheraconservative
approach (enucleation) for unicystic ameloblastoma
inthemaxillaiseverjustied.Thereis1casereport
ofarecurrentunicysticameloblastomaofthemaxilla
thatoninitialenucleationhadshownislandsofodon-
togenic epithelium in the cyst wall (intramural in-
volvement).
45
The tumor recurred 5 years later, and
theauthorsrecommendthatenucleationbefollowed
by further excision if mural invasion is seen.
45
InAfricanchildren,Arotiba
12
treatedonly2ofhis6
unicystic ameloblastoma cases (33%) with enucle-
ation, both of which recurred as extensive multiloc-
ularlesionsrequiringresection.OlaitanandAdekeye
8
treated6unicysticlesionswithenucleationfollowed
by peripheral ostectomy with an acrylic bur. They
had no recurrence with a median follow-up of 5.3
years.
In our series of 8 primary ameloblastoma in chil-
dren,6representedunicysticlesionsofthemandible.
Case3wasenucleatedandshowedonlyintraluminal
involvement. Cases 1 and 2 underwent marginal re-
sectionduetomuralinvolvement,breachofthecor-
ORD ET AL
tices with soft tissue involvement, and total loss of
bonearoundalloftheanteriorteeth,whereascase8
underwent segmental resection as the lower border
was also invaded. Ackerman et al
24
reported that
unicystic ameloblastoma with no mural invasion or
islandscanbesolargeandcausesomuchdestruction
thatenucleationwastechnicallyimpossible.Thishas
beenourexperienceinsomechildren(Fig3).Incase
6, the tumor was enucleated elsewhere and showed
capsularinvolvementwithextensionstothemargins.
Advantagesanddisadvantagesoffurthersurgerywere
discussedwiththepatientandparents,andalthough
further excision was recommended, the families de-
cided on a watch and wait policy.
Our 1 case of maxillary lesion, case 7, occurred in
agirlwithGorlinssyndromeand3separateodonto-
genickeratocystsinhermaxilla.Oneoftheseshowed
afocalareaofameloblasticchangeintheliningonly
with no proliferation but meeting the histologic cri-
teriaofVickersandGorlin.
15
Althoughfurthersurgery
was not planned, the patient is currently lost to fol-
low-up.
The management of unicystic ameloblastoma re-
mains controversial, but we believe that multicystic/
solid ameloblastoma or recurrent lesions should be
treated with resection in children, in the same way
that we would treat adults. Our practice is usually
segmentalresectionwitha1-cmmarginofcancellous
boneandresectionofoverlyingsofttissuesifcortical
perforationispresent.Ourcase4wastreatedinthis
manner, with immediate reconstruction with recon-
structionplateandfreebonegraft.Inrarecaseswhen
the lesion is diagnosed early, there may be sufcient
bone to resect the tumor with an adequate margin
and maintain continuity of the lower border. These
casesrequirecarefulassessment,ascase10illustrates.
Thiswomanhadamultilocularameloblastomatreated
at age 18 years with marginal resection and bone
graft.Asubsequentrecurrenceatage25yearswasin
the lower border inferior to her previous resection.
Althoughameloblastomahasbeensaidtonotinvade
the cortical bone of the lower border, this has not
been our experience.
The authors practice is to undertake enucleation
only for unicystic ameloblastoma with intraluminal
proliferation or ameloblastic changes of the epithe-
lium. When epithelial proliferation or odontogenic
islands occur in the connective tissue wall of the
lesion, we believe that further bone removal, either
keeping mandibular continuity or segmental resec-
tion(dependingonthesizeandextentofthetumor),
is advisable. Unfortunately, the diagnosis of connec-
tivetissuewallinvasioncanbemadereliablyonlyon
examination of the entire specimen histologically.
Frozen sections are subject to sampling error. This
769
means that enucleation is always regarded as a diag-
nostic or possible staging procedure.
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