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Unicystic ameloblastoma of maxilla

Article in Journal of Cranio-Maxillary Diseases · October 2012

DOI: 10.4103/2278-9588.102507

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Case Report

Unicystic ameloblastoma of maxilla

Yadavalli Guruprasad, Dinesh Singh Chauhan, Ramesh Babu
Department of Oral and Maxillofacial Surgery, AME’S Dental College Hospital and Research Centre, Raichur, Karnataka,
India

ABSTRACT
Unicystic ameloblastoma (UA) refers to those cystic lesions that show clinical and radiologic characteristics of
an odontogenic cyst but in histologic examination show a typical ameloblastomatous epithelium lining part of
the cyst cavity, with or without luminal and/or mural tumor proliferation. These tumors characteristically expand
within the jaw and displace the bone, the teeth and their roots. Occasionally, infiltrating tumors may erode
through the bone and extend into the soft tissue. The tumor is most commonly seen in the posterior mandible,
but may also arise in the maxilla and the anterior aspect of the jaws. The unicystic type of ameloblastoma is one
of the least encountered variant of the ameloblastoma. We report a case of UA of maxilla in a 32-year-old male
patient.
Keywords: Maxilla, odontogenic, unicystic ameloblastoma, unilocular

INTRODUCTION Men are affected slightly more often than women.

Ameloblastoma is the most common neoplasm
arising from the primary odontogenic or tooth-
forming tissue. Ameloblastomas are benign tumors
whose importance lies in their potential to grow to
enormous sizes, with resulting bone deformity.[1]
According to the World Health Organization (WHO)
1992 definition, ameloblastoma is a benign but
locally invasive polymorphic neoplasm consisting of
proliferating odontogenic epithelium, which usually
has a follicular or plexiform pattern, lying in a fibrous
stroma.[1,2] Usually, 80% of ameloblastomas occur
in the mandible and 20% of the ameloblastomas
occur in the maxilla. In the maxilla, they are localized
most often in the canine and antral regions, whereas
in the mandible 70% are located in the area of
the molars or the ascending ramus, 20% in the
premolar region and 10% in the anterior region.
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DOI:
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Ameloblastoma appears most commonly in the
third to fifth decades, but the lesion can be found
in any age group, including in children.[3] There are
six histologic subtypes of ameloblastoma: Follicular,
plexiform, acanthomatous, granular, basal cell
and desmoplastic. They can be found combined
or isolated, and are not related to prognosis of the
tumor. There are also three different macroscopic
subtypes: Solid or multicystic, unicystic and peripheral.
Radiographically, ameloblastomas may either
have unilocular or multilocular radiolucencies with
scalloped or sclerotic margins.[3,4] The teeth adjacent to
the tumor may show root displacement or resorption.
Unicystic ameloblastoma (UA) is considered a variant
of the solid or multicystic ameloblastoma, accounting
for 6–15% of all intraosseous ameloblastomas.
The term unicystic is derived from the macro- and
microscopic appearance, the lesion being essentially
a well-defined, often large, monocystic cavity with
a lining, focally but rarely entirely composed of
odontogenic (ameloblastomatous) epithelium. The
relative frequency of occurrence of UA has been
reported to be between 5% and 22% of all types
of ameloblastomas. The mean age at the time of

Correspondence to:
Dr. Yadavalli Guruprasad, Department of Oral and Maxillofacial Surgery, AME’S Dental College Hospital and Research Centre, Raichur-583104,
Karnataka, India. E-mail: guru_omfs@yahoo.com

44 Journal of Cranio-Maxillary Diseases / Vol 1 / Issue 1 / January 2012

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diagnosis differs considerably according to the UA
variants. This lesion occurs in a younger age group,
with slightly more than 50% of the cases occurring
in patients in the second decade of life.[4] Treatment
for ameloblastomas ranges from en-bloc resection to
much more conservative procedures like peripheral
osteotomy and enucleation, followed by aggressive
curettage. We report a case of UA of maxilla in a
32-year-old male patient.
CASE REPORT
A 32-year-old male patient was referred to the
Department of Oral and Maxillofacial Surgery for
evaluation of a right facial swelling [Figure 1]. The
lesion had been slowly increasing in size since it
was first noticed. There was no history of trauma,
pain, paresis, paresthesia or lymphadenopathy.
There was significant facial asymmetry caused by
an approximately 4 cm×3.5 cm mass involving the
right maxilla. The mass was firm and nontender to
palpation and not adherent to the overlying skin.
No bruits or pulsations were detected. Intraoral
examination showed expansion of the right buccal
cortex and palate thus obliterating the buccal
vestibule [Figure 2]. A panoramic radiograph
showed a well-defined unilocular radiolucency
involving the right maxillary sinus. There was
evidence of tooth displacement in relation to 14, 15
and 16 and root resorption in relation to 14, 15 and
16 [Figure 3]. A computed tomography (CT) scan
of the right maxilla showed unilocular radiolucency
Figure 1: Frontal view photograph showing right maxillary
swelling obliterating the nasolabial fold and displacing the
ala of the nose
Journal of Cranio-Maxillary Diseases / Vol 1 / Issue 1 / January 2012
Guruprasad, et al: Unicystic ameloblastoma of maxilla
with medial and lateral cortical expansion with
obliteration of the right maxillary sinus [Figures 4
and 5]. Laboratory values were within normal
limits and incisional biopsy was subsequently
performed. Based on the clinical, radiological and
histopathological findings, diagnosis of unicystic
plexiform ameloblastoma of maxilla was made. The
lesion was excised under general anesthesia using
the Caldwell-Luc approach. Partial maxillectomy
was then performed along with extraction of the
involved teeth [Figure 6]. Grossly, the specimen
measured approximately 3.5 cm×2.5 cm×2 cm
and had a smooth, lobulated outer surface
[Figure 7]. Histopathological examination revealed
plexiform ameloblastoma predominantly composed
of epithelium arranged as a tangled network of
anastomosing strands enclosing cysts of various sizes
suggestive of plexiform ameloblastoma [Figure 8].
The patient was discharged from the hospital on
the second postoperative day and sutures were
removed after 1 week; removable prosthesis was
given after 6 months [Figure 9] and followed-up for
1 year.
DISCUSSION
UA was first described as a distinct variant of
ameloblastoma in 1997 by Robinson and Martinez.
Thereafter, Ackermann et al. reclassified UA
into three types with prognostic and therapeutic
implications.[4] In type 1, the tumor is confined
to the luminal surface of the cyst with a lining
Figure 2: Intraoral photograph showing swelling on the
right side of the maxilla thus obliterating the vestibular
sulcus
45
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Guruprasad, et al: Unicystic ameloblastoma of maxilla
Figure 3: Orthopantomograph (OPG) showing unilocular
radiolucency in the right maxilla displacing and causing
resorption of the involved teeth
Figure 5: Coronal computed tomography scan view showing
unilocular radiolucency in the right maxilla causing thinning
of the anterior surface and palatal surface of the maxilla thus
obliterating the maxillary sinus
Figure 7: Excised specimen showing the smooth lobular
surface along with the extracted teeth
of ameloblastomatous epithelium. Type 2 is
characterized by epithelial nodules arising from the
46 Journal of Cranio-Maxillary Diseases / Vol 1 / Issue 1 / January 2012
Figure 4: Axial computed tomography scan view showing
unilocular radiolucency in the right maxilla causing thinning
of the anterior surface of the maxilla
Figure 6: Intraoperative photograph showing the lesion
attached to the floor of the maxillary sinus
cystic lining and projecting into the cyst lumen. There
is no evidence of infiltration of the fibrous cystic wall
in either type of lesion. In type 3, the fibrous wall
of the cyst is infiltrated by a trabecular pattern that
resembles the plexiform pattern seen in conventional
ameloblastoma. In addition, it is characterized by a
basal layer of columnar cells with hyperchromatic
nuclei. These cells are loosely cohesive and resemble
stellate reticulum epithelium.[4,5] The radiographic
features of UA are typically unilocular and there is
a round area of radiolucency. Therefore, this lesion
is often misdiagnosed as an odontogenic keratocyst
or a dentigerous cyst.[5]
UAs account for 10–15% of all extraosseous
ameloblastomas. The occurrence of this lesion may
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Figure 8: Photomicrograph showing histopathological
findings revealing plexiform ameloblastoma predominantly
composed of epithelium arranged as a tangled network
of anastomosing strands enclosing cysts of various sizes
(hemotoxylin and eosin stain, 40 × magnification)
be de novo, and whether it is a result of neoplastic
transformation of a noncystic epithelium or not has
been long debated. More than 90% of such lesions
occur in the posterior region of the mandible,
and are asymptomatic. A large lesion may cause
painless swelling of the jaws.[6,7] Late recurrence
following treatment is commonly seen, the average
interval for recurrence being 7 years. Recurrence
is also related to the histological subtypes of UA,
with those invading the fibrous wall having a rate
of 35.7%, but others having a rate of only 6.7%.
Recurrence rates are also related to the type of
initial treatment.[7] The recurrence rates are 3.6%
for resection, 30.5% for enucleation alone, 16%
for enucleation followed by Carnoy’s solution
application and 18% by marsupialization followed
by enucleation (where the lesion reduced in size).[8]
The age of the patient is another influencing
factor that is related to the choice of treatment. As
UA tends to affect young adolescent patients, the
concern to minimize surgical trauma and permit
jaw function should be one of the important aspects
in tumor management. While conservative surgery
seems to have been justified with preference over
mutilating radical surgery for this young patient,
the choice of treatment has to be considered in
conjunction with other clinical and pathological
factors such as the size, location and growth pattern
of the tumor.[8,9] Whatever surgical approach
Journal of Cranio-Maxillary Diseases / Vol 1 / Issue 1 / January 2012
Guruprasad, et al: Unicystic ameloblastoma of maxilla
Figure 9: Postoperative photograph after 3 months
the surgeon decides to take, long-term follow-
up is mandatory, as the recurrence of UA may be
long delayed. Prognosis is more dependent on
the method of surgical treatment rather than the
histology of tumor. The treatment of ameloblastoma
itself is considered an important prognostic factor,
as suggested in several studies. [9] Recurrence of
this tumor reflects the inadequacy or failure of the
primary surgical procedure. A recent systematic
review (considered the best level of evidence)
showed that enucleation of UA resulted in a highest
recurrence rate and the lowest recurrence rate was
associated with resection of tumor.[10] Therefore,
proper preoperative diagnosis of these kinds of
lesions and long-term follow-up is a must because
recurrence may appear years after removal.
REFERENCES
1. Isacsson G, Andersson L, Forsslund H, Bodin I, Thomsson M.
Diagnosis and treatment planning of unicystic ameloblastoma.
Int J Oral Maxillfac Surg 1986;15:759-64.
2. Williams TP. Management of ameloblastoma: A changing
perspective. J Oral Maxillofac Surg 1993:51:1064-70.
3. Philipsen HP, Reichart PA. Unicystic ameloblastoma. A review
of 193 cases from the literature. Oral Oncol 1998;34:317-25.
4. Li TJ, Wu YT, Yu SF, Yu GY. Unicystic ameloblastoma:
A clinicopathologic study of 33 Chinese patients. Am J Surg
Pathol 2000;24:1385-92.
5. Lee PK, Samman N, Ng IO. Unicystic ameloblastoma--Use of
Carnoy’s solution after enucleation. Int J Oral Maxillofac Surg
2004;33:263-7.
6. Navarro CM, Principi SM, Massucato EM, Sposto MR.
Maxillary unicystic ameloblastoma. Dentomaxillofac Radiol
2004;331:60-2.
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Guruprasad, et al: Unicystic ameloblastoma of maxilla
7. Lau SL, Samman N. Recurrence related to treatment modalities
of unicystic ameloblastoma: A systematic review. Int J Oral
Maxillofac Surg 2006;35:681-90.
8. Kalaskar R, Unawane AS, Kalaskar AR, Pandilwar P. Conservative
management of unicystic ameloblastoma in a young child:
Report of two cases. Contemp Clin Dent 2011;2:359-63.
9. Pogrel MA, Montes DM. Is there a role for enucleation in the
management of ameloblastoma? Int J Oral Maxillofac Surg
Commentary
In “Unicystic ameloblastoma of the maxilla”,“
Guruprasad et al.[1] present a clinically well-
documented case report about a rare subtype
of ameloblastoma. Although the incidence of
ameloblastoma is only 0.6/1 million,[2] it is a
clinically relevant entity as it is the most frequent
odotogenic tumor[2] and implies some diagnostic
and therapeutic pitfalls.[3] Guruprasad et al. rightly
point out that choosing the correct surgical strategy
is essential for minimizing relapses, but long-term
follow-up is nonetheless mandatory as relapses in
all subtypes of ameloblastoma occur usually not
before years.
It must not be forgotten that ameloblastomas are
not only intraosseous lesions but can also be found
in an extraosseous localization, e. g. in the maxillary
sinus. In the case of unicystic ameloblastoma,
a radiologist will usually (but understandably)
misinterpret the finding as a dentigerous cyst or
mucocele.[4] For the pathologist, it is difficult to
diagnose unicystic ameloblastomas as the histological
presentation in the mainly cystic parts of the lesion
is usually not suggestive for ameloblastoma, and in
extraosseous ameloblastomas, this diagnosis might
not be considered due to the missing reference to
bone.[4] Therefore, the head and neck surgeon is the
relevant “gateway” and should keep the differential
diagnosis of “unicystic ameloblastoma” in mind in
any cystic lesions, especially when displaced teeth
are involved.
48 Journal of Cranio-Maxillary Diseases / Vol 1 / Issue 1 / January 2012
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2009;38:807-12.
10. Zhang J, Gu Z, Jiang L, Zhao J, Tian M, Zhou J, et al.
Ameloblastoma in children and adolescents. Br J Oral
Maxillofac Surg. 2010 Oct;48:549-54.
How to cite this article: Guruprasad Y, Chauhan DS, Babu R. Unicystic
ameloblastoma of maxilla. J Cranio Max Dis 2012;1:44-8.
Source of Support: Nil. Conflict of Interest: None declared.
Submission: May 18, 2012, Acceptance: July 29, 2012
Thomas Braun
Department of Otorhinolaryngology, Head and Neck
Surgery, Ludwig Maximilian University
Correspondence to:
Dr. Thomas Braun,
Department of Otorhinolaryngology, Head and Neck Surgery,
Ludwig Maximilian University, Marchioninistraße 15,
D-81377 Munich, Germany.
E-mail: thomas.braun@med.uni-muenchen.de
REFERENCES
1. G u r u p r a s a d Y, C h a u h a n D S , B a b u R. U n i c y s t i c
ameloblastoma of maxilla. J Cranio Max Dis 2012;1:
44-8.
2. Larson A, Almeren H. Ameloblastoma of the jaw. An analysis
of a consecutive series of all cases reported to the Swedish
Cancer Registry during 1958-1971. Acta Pathol Microbiol
Scand A 1978:86:337-49.
3. Braun T, Leunig A. Ameloblastoma of the paranasal
sinuses. forum Hals-Nasen- Ohrenheilkunde 2011;3:
76-8.
4. Braun T, Ihrler S, Kisser U, Leunig A. Cystic lesion with a
displaced tooth in the maxillary sinus. HNO 2011;59:700-4.
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Submission: July 24, 2012 Acceptance: July 29, 2012