Journal of

Craniomaxillofacial Research
Vol. 9, No. 2 Spring 2022

Conservative management of large unicystic ameloblastoma in

a young patient: A case report

Abbas Haghighat 1, Sayed Mohammad Razavi 2, Saeedeh Khalesi 3*

1. Craniofacial and Cleft Research Center, Department of Oral and Maxillofacial Surgery, School of Dentistry, Isfahan University of Medical Sciences, Isfahan,
Iran.

2. Dental Implant Research Center, Department of Oral and Maxillofacial Pathology, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran.

3. Dental Materials Research Center, Department of Oral and Maxillofacial Pathology, School of Dentistry, Isfahan University of Medical Sciences, Isfahan,
Iran.

ARTICLE INFO ABSTRACT

Article Type: Objectives: Ameloblastoma is a benign neoplasm with origin from odontogenic epithelium.
Case Report Unicystic ameloblastoma has clinical and radiographically features resemble to other odontogenic
cysts but it has a typical ameloblastomatous epithelium lining the cyst cavity.
Received: 8 Nov. 2021 Case: In this case report study, we presented a 9-year-old girl who was referred to Oral and
Revised: 5 Dec. 2021 Maxillofacial Surgery of Isfahan Dental School for the management of a large swelling on the right
Accepted: 19 Feb. 2022 posterior mandiblular region. The histopathologic examination of the specimen showed mural type
of unicystic ameloblastoma. In the first step, the patient was treated by decompression of the lesion.
*Corresponding author: Five month after it, shrinkage of the lesion was observed and in the second stage of surgery, curet-
Saeedeh Khalesi
tage of the remaining lesion and extraction of tooth buds in the areas of lesion was performed. Af-
ter two years, radiographic image showed new bone formation and complete healing of the lesion.
Dental Materials Research Center, Department of
Conclusion: Choosing the best treatment for children with unicystic ameloblastoma requires
Oral and Maxillofacial Pathology, School of Den-
more attention and all clinical and histopathological parameters should be considered. Conser-
tistry, Isfahan University of Medical Sciences, Isfa- vative treatment for ameloblastoma leads to reduce complications after treatment and affect the
han, Iran. patient’s quality of life.
Keywords: Ameloblastoma; Jaw; Pathology.

Tel: +98-913-1079487
Fax: +98-21-84902473
Email: [email protected]

Introduction

A meloblastoma is a benign neoplasm with origin

from odontogenic epithelium [1]. Unicystic am-
eloblastoma (UA) is a less encountered variant of
ameloblastoma that was described in 1977 for the first time
by Robinson and Martinez [2]. According to studies, uni-
cystic ameloblastoma may originate from reduced enamel
epithelium; or as a result of transformation of dentigerous
cyst; or cystic degeneration of solid ameloblastoma [3].
Unicystic ameloblastoma has clinical and radiographical
features resemble to other odontogenic cysts but it has a
Copyright © 2022 Tehran University of Medical Sciences.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International license (https://creativecommons.org/licenses/by-nc/4.0/). Non-com-
mercial uses of the work are permitted, provided the original work is properly cited.

J Craniomax Res 2022; 9(2) : 100-105

 Haghighat, et al. / 101

typical ameloblastomatous epithelium lining the cyst with plexiform pattern were observed. Finally, a defin-
cavity. This lesion has three different variants in his- itive diagnosis was made of mural unicystic ameloblas-
topathological feature with prognostic significance in- toma (Fig 2,A,B). Patient’s parents were informed re-
cluding luminal, intraluminal and mural [4,5]. Because garding the different treatment options and recurrence
the unicystic ameloblastoma is a less aggressive type of rate of lesion. After obtaining informed consent from
ameloblastoma, simple enucleation has been suggested the patient’s parents and taking into consideration the
to be adequate for the majority of these cases [6]. But, age of patient, conservative treatment plane was done
a treatment strategy of UA should be decided by his- under general anesthesia. In the first stage of treat-
topathological type of the lesion. Unicystic ameloblas- ment, decompression for privation of bone resection
toma in mandible bone is a very rare in under 10-year and subsequence of facial deformity was performed for
pediatric population [4]. We present a case of unicystic the patient.
ameloblastoma in a 9-year-old girl and report a conser-
vative approach of patient treatment. In the first stage of surgery, by cutting and remov-
ing the mandibular mucosal tissue in the affected area,
Case Report the retromandibular bone defect was enlarged with a
regenerator and washed with a large amount of normal
A 9-year-old girl referred to the Department of saline. Defective relationship was maintained with the
Oral and maxillofacial Surgery of Isfahan Dental oral cavity with a gauze impregnated with tetracycline
School, Iran, with the chief complaint of asymptomatic ointment which was left in the wound for 4 days. Anti-
swelling in the right posterior mandiblular region. She biotics and analgesics were prescribed. Seven days after
has not contributory medical history. The girl had no- the operation, the antibiotic gauze was removed and a
ticed the swelling approximately four months ago and silicon obturator fabricated on the cast and designed
the size of the lesion has increased significantly over to avoid occlusal interference was used to maintain
time. In extraoral examination, a diffuse large swelling the fenestration window. Marginal adaptation of the
on the right mandibular region with tenderness on pal- obturator was performed with a secondary silicon im-
pation was observed. In intraoral examination, there pression material. The obturator negated the require-
was bony expansion of the labial cortex from the lower ment for frequent gauze changes. The patient carefully
right retromolar area to the first deciduous molar (Fig washed the cavity regularly and maintain overall prop-
1,A). Teeth in the area responded to vitality tests. There er hygiene of the oral cavity through self-irrigation for
was no sign of facial nerve or inferior alveolar nerve five months with the help of her parents. Five months
involement. Panoramic x-ray revealed a well-defined after the first stage of surgery, shrinkage of the lesion,
unilocular radiolucent lesion involving the left side of repair and extensive improvement of the bone defect
ramus and angle of mandible that it has second perma- was observed and the radiolucent area was significant-
nent molar teeth bud and first molar teeth roots (Fig ly reduced (Fig 3,A). In the second stage of surgery,
1,B). scar tissue was removed from the bone surface of the
Based on the clinical and radiographical features, markedly diminished fenestration and curettage of the
differential diagnosis was considered as benign odon- remaining lesion was performed along with extraction
togenic cyst or tumor including dentigerous cyst, of the second and third molar tooth buds to reduce
odontogenic keratocyst and ameloblastoma. An inci- recurrence. Therefore, the patient was in a long-term
sional biopsy was performed under local anesthesia follow-up. After two years, panoramic x-ray showed
for the patient. In histopathological examination of that visible new bone formed and the buccal and lin-
the specimen observed a cystic lesion that was lined by gual cortex of mandible was found to be symmetry (Fig
ameloblastic epithelium. The lesion has typical features 3,B). At the last follow-up period, normal mandible
of ameloblastoma in some areas, including columnar bone contour without signs of recurrence was evident.
basal cells in palisading arrangement with vacuolat-
ed cytoplasm, hyperchromatic nuclei polarized away
from basement membrane that was resemble to pre-
ameloblasts. Suprabasal cells have loosely texture that
was resemble to stellate reticulum. Cystic odontogenic
epithelium with characteristic features lining fibrous
connective tissue wall and the ameloblastoma tumor
islands and bonds in the fibrous connective tissue wall
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Conservative management of large unicystic ameloblastoma in a young patient / 102

Figure 3. A: Reduce lesion size and repair the mandible

bone after first surgery in panoramic x-ray, B: Forming
visible new bone at the site of the lesion.
Figure 1. A: Intraoral view showing swelling on the Discussion
right mandibular region, B: Panoramic x-ray revealed
a well-defined unilocular radiolucent lesion involving Unicystic ameloblastoma is accounts for 10% to
the left side of mandible. 15% of all intra-osseous ameloblastoma. This lesion is
a tumor of young age group especially in the second
decade and has unilocular radiolucency in radiograph-
ic view [7]. However, it is often found with impacted
teeth, but this lesion can be seen anywhere on the jaw
without related to the tooth. The ratio of mandibular
to maxillary involvement in unicystic ameloblastoma
has been reported to be 13:1 [8]. The most clinical
manifestations include painless swelling, facial asym-
metry, tooth impaction, displacement and mobility of
tooth, root resorption or divergence, occlusal interfer-
ence, and extrusion of tooth [9]. The present case re-
port describes the UA of mandibular molar region in a
9-years-old girl. Kalaskar et al. reported this lesion in
9 and 12 years old patients [7]. Sasaki et al. described
a large UA in 20-years old patient [1]. UA associated
with impacted teeth occur more in males than females,
but the lesions without association the impacted teeth
were more common in females [4]. In this case being a
child with the unerupted permanent teeth.
Dentigerous cyst, odontogenic keratocyst, residual
cyst, adenomatoid odontogenic tumor and solid amelo-
blastoma are the differential diagnosis for UA. Odonto-
genic keratocyst usually has anterio-posteriorly growth
Figure 2. A: Cystic lesion lined by ameloblastomatous and large amount of keratin on aspiration. Residual
epithelium proliferating into connective tissue wall in a cysts are associated with older age and missing teeth
plexiform pattern, B: Preameloblasts and stellate retic- that have been extracted. Adenomatoid odontogenic
ulum in the surface of lesion. tumors have a predilection for anterior maxilla. Solid
ameloblastoma has multilocular radiolucency and is

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 Haghighat, et al. / 103

seen rare in patients less than 30 years old. Therefore, term follow-up [1]. Furthermore, in Meshram et alʼs
dentigerous cyst is the most important lesion which study showed that UA in younger population can be
should be differentiated from UA [9,10]. Based on successfully treated by a conservative treatment plan by
classification of Ackermann, UA are three groups on enucleation and bone curettage [15]. Sineedi et al. re-
histopathological feature including 1: cyst lined with a ported a UA of the mandible in a 9-year-old child. This
variable and often nondescript epithelium (luminal); 2: lesion was surgically managed by enucleation of the
cyst showing the intraluminal plexiform proliferation cyst with all the impacted teeth. There were no signs
of epithelium (intraluminal); 3: cyst with the invasion of recurrence and his latest radiographic examination
of epithelium into the cyst wall in either follicular or showed good bone formation [4]. Kim et al. described a
plexiform pattern (mural) [11]. According to most case of an 11-year-old girl with UA of the posterior re-
studies, there is a different behavior between patholog- gion of mandibular bone with impaction of the second
ical types of UA. The lesion with epithelial penetration and third molars. The lesion was marsupialized, and
in fibrous wall (mural) have the capacity to invade the 31 months after marsupialization, surgical enucleation
adjacent bone [12]. Therefore, histopathological type of was performed with extraction of the impacted third
UA is the most important factor in the treatment strat- molar. The second molar, which was preserved, sponta-
egy. The most studies reported that enucleation is the neously and completely erupted [13]. In the Ahmedʼs
best treatment plane for Group 1 and Group 2 lesions, study, 10 patients with UA was treated by enucleation
while Group 3 lesions should be treated with aggres- with bone curettage followed by application of carnoy’s
sive plan such as radical resection [1]. The treatment of solution. He showed that even mural types can be suc-
UA can be radical or conservative approaches. Radical cessfully treated with conservative approaches [18].
treatments can be achieved by segmental or margin- The patient in this study was a 9-year-old girl with a
al resectioning of the lesion, followed by insertion of large mural type of UA that was successfully treated
reconstructive plates. while, conservative approaches conservatively without recurrence and with favorable
comprise enucleation, enucleation followed by applica- bone formation after two years of follow-up. Then uni-
tion of Carnoy’s solution, or marsupialization followed cystic ameloblastomas are biologically less aggressive
by enucleation [13]. Based on studies, the recurrence and respond to conservative management such as enu-
rate of the lesion is 3.6% for resection, 30.5% for enu- cleation, curettage and marsupialization [16]. Aggres-
cleation, 16% for enucleation with the application of sive surgical treatment plane like segmental resection
Carnoy solution and 18% for marsupialization with should not be performed in children and used only in
or without other treatment in the second phase [14]. the patients with recurrent lesions [19]. Bone resection
However, overall health of patient, pathological and causes deformity that is requires reconstruction meth-
anatomical indicators, size, location, duration, periodic ods especially in young people [6]. In general, choos-
follow-up examinations should be taken when choos- ing the right treatment depends on several factors.
ing a treatment plan [15]. But, the presence of amelo- The continuous growth and facial bone physiology in
blastoma cells in the connective tissue of the cyst wall children with a higher percentage of cancellous bone,
was the most important predictor of the recurrence of bone turn-over and periosteal activity are the effec-
the lesion [16]. tive factors in choosing treatment in children with UA
[20]. Recent studies suggest that the radical treatment
In children, the treatment of UA is influenced by strategies have severe consequences for the patient and
three factors including [1] continuing facial growth, reduces their quality of life. Furthermore, conservative
different bone physiology (greater percentage of can- treatment methods for ameloblastoma are associated
cellous bone, increased bone turnover and reactive with a higher risk of recurrence but these methods
periosteum) and presence of unerupted teeth; [2] dif- have lower risks of other complications and fewer sur-
ficulty in initial diagnosis; and [3] type of UA. There- gical interventions for esthetic and functional rehabil-
fore, although invasive treatment has been suggested to itation. Consequently, conservative treatment methods
prevent recurrence, more conservative treatments can receive a better response from patients. Therefore, less
be used in children [15]. De Paulo et al. treated the invasive treatment plans for ameloblastoma associated
extensive unicystic ameloblastoma in a 7-year-old child with increasing the quality of life of patients and their
with marsupialization followed by enucleation [17]. greater satisfaction [21]. Marsupialization and decom-
Sasaki et al. reported the treatment with enucleation pression are different methods with the aim of reduc-
and deflation of a large UA with mural invasion in a ing the size of lesion by lowering the pressure of cystic
20-year-old patient without recurrence after a long- fluid and inducing bony apposition to the cystic wall.
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Conservative management of large unicystic ameloblastoma in a young patient / 104

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Please cite this paper as:

Haghighat A, Razavi M, Khalesi S. Conservative
management of large unicystic ameloblastoma in
a young patient: A case report. J Craniomax Res
2022; 9(2): 100-105

J Craniomax Res 2022; 9(2) : 100-105