Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 182184

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Journal of Oral and Maxillofacial Surgery,

Medicine, and Pathology
journal homepage: www.elsevier.com/locate/jomsmp

Case Report

Hybrid variant of desmoplastic ameloblastoma, a rare

histomorphological entity A case report and review of literature
Sramana Mukhopadhyay a, , Cecil T. Thomas b , Kavitha Bali a , Santosh Koshy c ,
Pranay Gaikwad b
a
Department of General Pathology, Christian Medical College, Vellore, India
b
Department of General Surgery Unit 1 Head and Neck Oncosurgery, Christian Medical College, Vellore, India
c
Department of Dental and Oral Surgery, Christian Medical College, Vellore, India

a r t i c l e i n f o a b s t r a c t

Article history: Desmoplastic ameloblastoma is an uncommon variant of ameloblastoma with special anatomic distribu-
Received 2 April 2015 tion, histomorphology and radiographic features that differ from those of conventional ameloblastoma.
Received in revised form 3 May 2015 The Hybrid variant of desmoplastic ameloblastoma is a rare variant in which histologically, a follicular
Accepted 12 May 2015
or plexiform pattern of conventional ameloblastoma coexists with areas of desmoplastic ameloblastoma.
Available online 9 June 2015
We report an unusual case of such a hybrid lesion presenting as a left hemi-mandibular swelling in a 41-
year-old female patient. The purpose of this article is to add to the existing body of literature a rarely
Keywords:
reported condition.
Desmoplastic
Hybrid 2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.
Mandible
Plexiform

1. Introduction features of desmoplastic variant together with areas of classical fol-

Ameloblastoma is a benign neoplasm of odontogenic epithelial
origin. It arises from the remnants of the dental lamina, the enamel
organ of the developing tooth, the epithelial lining of odontogenic
cysts or the basal cells of the oral mucosa [1]. It is a slowly growing,
locally invasive benign tumor with a high propensity for local recur-
rence. Vickers and Gorlin in 1970 dened the histopathological
features of ameloblastoma. These features include hyperchroma-
sia, palisading and reverse polarization of the basal nuclei of the
epithelial cells with vacuolization of the cytoplasm [2]. Histolog-
ical types include: follicular, plexiform, acanthomatous and the
desmoplastic. Desmoplastic ameloblastoma (DA) often occurs in
the anterior region of jaws with unique radiographic appearance
resembling brosseous lesions and shows distinct histopathology
characterized by extensive stromal collagenization or desmoplasia
surrounding compressed islands of odontogenic epithelium. A tran-
sitional form of desmoplastic ameloblastoma, showing microscopic
Asian AOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian
Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathol-
ogy; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese
Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants.
Corresponding author at: Department of General Pathology, Christian Medical
College, Vellore, India. Tel.: +91 0416 228 2005.
E-mail address: drsramana@gmail.com (S. Mukhopadhyay).
licular/plexiform ameloblastoma, has been described as a hybrid
lesion [3,4].
We describe a unique case of hybrid variant of desmoplastic
ameloblastoma (HA) in a 41-year-old female with details of clini-
cal, radiographic and unusual histologic features. A detailed review
of literature revealed only a handful of reported cases since the
rst description of HA by Waldron and El-Mofty in 1987 [5] with
no recurrence of the lesion in any of the reported cases. Here,
we add an additional case of HA to the existing literature as the
synchronized existence of desmoplastic ameloblastoma with plex-
iform ameloblastoma is a rare event and this is the rst case to be
reported from our institution.
2. Case report
A 41-year-old female presented with swelling in the left hemi-
mandible for the past 10 years with gradual increase in size. There
was no associated pain, bleeding, discharge, or difculty in chewing
and swallowing. On examination, there was a 5 cm 5 cm non-
compressible, non-pulsatile, non-uctuant and immobile swelling
over the inferior aspect of the horizontal ramus of the left hemi-
mandible extending to the gingiva. It was globular in shape, with
a smooth surface, well dened margins and rm consistency. The
skin over the swelling was normal. There were no ulcers or satel-
lite lesions. A CT scan of the head and neck region showed a large

http://dx.doi.org/10.1016/j.ajoms.2015.05.003
2212-5558/ 2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.
 S. Mukhopadhyay et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 182184
exophytic, expansile, lytic lesion involving most of the mandibular
body on the left side extending into the vertical ramus with focal
areas of cortical breach (Fig. 1). The biopsy from the lesion was
reported as desmoplastic ameloblastoma. The patient was posted
for wide local excision. Intraoperatively, multiple swellings were
noted in the horizontal ramus with egg shell crackling for which
extended segmental mandibulectomy with free bular osteocuta-
neous ap reconstruction was done.
On gross pathological examination, the surgical specimen
showed multiple nodular swellings in the ramus of the mandible
measuring 7 cm 6 cm 5 cm in toto with cystic and solid cut sur-
face. The cystic area contained greenish-mucoid material while the
solid area appeared homogeneously white (Fig. 2A). Histopatholog-
ically, the lesion demonstrated solid and cystic components. The
solid areas of the tumor showed islands, nests and anastomosing
strands of ameloblastic epithelium with central stellate reticulum
and peripheral palisading of nuclei (Fig. 2B). Few of the islands
showed central squamoid differentiation and edema with forma-
tion of microcysts. Also, there were irregular shaped epithelial
islands, few displaying a swirled hypercellular center with spindle
shaped to squamoid epithelial cells, absent peripheral palisading
and surrounded by narrow zones of loosely structured connec-
tive tissue embedded in a desmoplastic stroma (Fig. 2C and D).
There was focal inltration of tumor into the cortical bone (Fig. 2E).
There was no necrosis or signicant mitotic activity. The cystic
component showed a brous cyst wall lined by ameloblastic epithe-
lium composed of columnar to cuboidal cells with hyperchromatic
nuclei displaying reverse polarity and basilar cytoplasmic vacuola-
tion (Fig. 2F). The overall features were suggestive of a hybrid form
of desmoplastic ameloblastoma as both typical desmoplastic areas
with plexiform areas were seen.
3. Discussion
Desmoplastic ameloblastoma was rst described by Tussole
et al. in 1984 and reviewed by Waldron and El-Mofty in 1987
[5]. In 1992, the World Health Organisation (WHO) regarded
Fig. 1. A large exophytic, expansile, lytic lesion involving most of the mandible on
the left side and extending into the ramus with focal areas of cortical breach and
thin irregular internal septations.
183
it as a variant of ameloblastoma [4]. The striking difference in
the anatomic location, mainly in the anterior premolar region of
the maxilla/mandible, unusual radiologic presentation of mixed
radiolucency-radioopacity with ill-dened borders and distinctive
histopathology of extensive stromal desmoplasia with scattered
odontogenic epithelium makes it a distinct clinicopathologic entity.
Hybrid lesion of desmoplastic ameloblastoma and conventional
ameloblastoma is an unusual variant of ameloblastoma, which was
rst described by Waldron and El-Mofty in 1987. Hybrid lesions
show typical microscopic features of DA characterized by pro-
nounced stromal desmoplasia together with areas of follicular,
plexiform and acanthomatous ameloblastoma [4,5].
In the literature, it is noticed that the DA represented the
least common odontogenic tumor. Among the 89 cases of amelo-
blastomas studied by Takata et al., 7.9% were diagnosed as DA and
only 1.1% as hybrid lesion [10]. In Japan, the DA was only 5.3% of
all the cases of intraosseous ameloblastomas diagnosed in 27 years
[6,7]. As per an Indian study, ameloblastoma accounted for 1.18% of
the total surgical specimens received over a period of 25 years with
DA accounting for only 2.25% of the various histological variants of
ameloblastoma. HA was not reported [8]. The present case is the
rst case of HA to be reported from our institution.
The HA has an almost equal sex predilection and occurs in a wide
age range of 1782 years. Strikingly, it has been reported predomi-
nantly in the Asians with a denite mandibular predilection [6]. Not
surprisingly, even the present case is a 41-year-old female patient
with the lesion in the anterior ramus of the left hemi-mandible.
The literature shows that most cases of HA present as mixed
radiolucent and radiopaque lesions with irregular borders simi-
lar to the common radiological pattern observed in DA or bro
osseous lesions. A few cases of HA also present as multilocular
radiolucencies similar to the common radiographic pattern of con-
ventional ameloblastoma [9]. Takata et al. also believed that the
mixed pattern expresses the inltrative nature of the tumor. As the
DA inltrates the bone marrow spaces, remnants of the original
non-metaplastic or non-neoplastic bone remain in the tumor tis-
sue. The ill-dened borders may also be attributed to the inltrative
behavior of the tumor [4,10]. The present case showed an expan-
sile, lytic lesion involving most of the mandibular body extending
to the adjoining ramus with focal areas of cortical breach which
was conrmed histologically. Microscopically, in the present case,
the tumor showed abundant desmoplastic stroma and islands of
ameloblastic epithelium together with plexiform areas display-
ing anastomosing strands of ameloblastic epithelium with central
stellate reticulum and peripheral palisading of nuclei, as exactly
described by Waldron and El-Mofty [5]. Histopathological inter-
pretation of the coexistence of DA with the conventional variant
in a Hybrid lesion remains challenging and is still unclear with
one of the three possibilities (a) the desmoplastic change occurs
secondarily in the stroma of a pre-existing solid or multicystic
ameloblastoma; (b) a part of primary desmoplastic ameloblastoma
alters into the conventional ameloblastoma; or (c) the hybrid lesion
being a type of collision tumor [4].
Mahadesh et al. reported the 5-year recurrence rate of 53%
after the surgical treatment of ameloblastomas [11]. Desmoplas-
tic ameloblastoma is more aggressive than its other variants. This
is suggested by its potential to reach large size, an early invasion
of adjacent structures, diffuse and ill-dened radiographic appear-
ance and the histological presence of bone invasion [12]. However,
the prognosis of hybrid lesions has not been clearly established. It
is generally described that most cases of DA have ill-dened mar-
gins and may have a propensity to recur, at least as frequently
as the conventional ameloblastomas. As the biological behavior,
radiographic and histological features of the hybrid lesion are still
not fully understood, the lesion still needs more extensive tumor
analysis, follow-up and reporting into the medical literature.
184 S. Mukhopadhyay et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 182184

Fig. 2. (A) Solid component of the tumor involving the left ramus of the mandible. (B) Plexiform areas of the tumor with islands and anastomosing strands of ameloblastic
epithelium with central stellate reticulum and peripheral palisading of nuclei. (C and D) Irregular epithelial islands, few displaying a swirled hypercellular center, absent
peripheral palisading and embedded in a desmoplastic stroma. (E) Tumor inltrating bone. (F) Cystic component of tumor lined by ameloblastic epithelium. (For interpretation
of the references to color in the text, the reader is referred to the web version of the article.)

Conict of interest
None.
References
[1] Belardo E, Velasco I, Guerra A, Rosa E. Mandibular ameloblastoma in a 10-
year-old child: case report and review of the literature. Int J Odontostomatol
2012;6:3316.
[2] Shaikhi K, Neiders M, Chen F, Aguirre A. Morphological variants of ameloblas-
toma and their mimickers. N Am J Med Sci 2012;5:208.
[3] Gupta A, Jindal C. Hybrid ameloblastoma: report of a rare case and review of
literature. Int J Oral Maxillofac Pathol 2011;2:6872.
[4] Vardhan BGH, Ingle E, Dornadula K, Gopal S. Hybrid lesion of desmoplastic and
conventional ameloblastoma a case report. JIOH 2011;3.
[5] Waldron CA, El-Mofty SK. A histopathologic study of 116 ameloblastomas with
special reference to the desmoplastic variant. Oral Surg Oral Med Oral Pathol
1987;63:44151.
[6] Angadi PV, Kale A, Hallikerimath S, Kotrashetti V, Mane D, Bhatt P,
et al. Hybrid desmoplastic ameloblastoma: an unusual case report with
immunohistochemical investigation for TGF- and review of literature. East
J Med 2011;16:917.
[7] dos Santos JN, DeSouza VF, Azevdo RA, Sarmento VA, Souza LB. Hybrid
lesion of desmoplastic and conventional ameloblastoma: immunohistochem-
ical aspects. Rev Bras Otorrinolaringol 2006;72:70913.
[8] Chawla R, Ramalingam K, Sarkar A, Muddiah S. Ninety-one cases of ameloblas-
toma in an Indian population: a comprehensive review. J Nat Sci Biol Med
2013;4:3105.
[9] Efom OA, James O, Akeju OT, Salami AS, Odukoya O. Hybrid ameloblas-
toma in a Nigerian: report of a case and review of literature. Open J Stomatol
2013;3:34753.
[10] Takata T, Miyauchi M, Ito H, Ogawa I, Kudo Y, Zhao M, et al. Clinical and
histopathological analyses of desmoplastic ameloblastoma. Pathol Res Pract
1999;195:66975.
[11] Mahadesh J, Rayapati DK, Maligi PM, Ramachandra P. Unicystic ameloblas-
toma with diverse mural proliferation a hybrid lesion. Imaging Sci Dent
2011;4:2933.
[12] Rastogi R, Jain H, Mohan C, Rastogi V, Rastogi K. Hybrid ameloblastoma an
unusual combination of desmoplastic and conventional ameloblastoma. JIMSA
2010;23.