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

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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
Department of General Pathology, Christian Medical College, Vellore, India
Department of General Surgery Unit 1 Head and Neck Oncosurgery, Christian Medical College, Vellore, India
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
reported condition.
Hybrid 2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.

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

licular/plexiform ameloblastoma, has been described as a hybrid
Ameloblastoma is a benign neoplasm of odontogenic epithelial lesion [3,4].
origin. It arises from the remnants of the dental lamina, the enamel We describe a unique case of hybrid variant of desmoplastic
organ of the developing tooth, the epithelial lining of odontogenic ameloblastoma (HA) in a 41-year-old female with details of clini-
cysts or the basal cells of the oral mucosa [1]. It is a slowly growing, cal, radiographic and unusual histologic features. A detailed review
locally invasive benign tumor with a high propensity for local recur- of literature revealed only a handful of reported cases since the
rence. Vickers and Gorlin in 1970 dened the histopathological rst description of HA by Waldron and El-Mofty in 1987 [5] with
features of ameloblastoma. These features include hyperchroma- no recurrence of the lesion in any of the reported cases. Here,
sia, palisading and reverse polarization of the basal nuclei of the we add an additional case of HA to the existing literature as the
epithelial cells with vacuolization of the cytoplasm [2]. Histolog- synchronized existence of desmoplastic ameloblastoma with plex-
ical types include: follicular, plexiform, acanthomatous and the iform ameloblastoma is a rare event and this is the rst case to be
desmoplastic. Desmoplastic ameloblastoma (DA) often occurs in reported from our institution.
the anterior region of jaws with unique radiographic appearance
resembling brosseous lesions and shows distinct histopathology 2. Case report
characterized by extensive stromal collagenization or desmoplasia
surrounding compressed islands of odontogenic epithelium. A tran- A 41-year-old female presented with swelling in the left hemi-
sitional form of desmoplastic ameloblastoma, showing microscopic 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-
Asian AOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian compressible, non-pulsatile, non-uctuant and immobile swelling
Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathol- over the inferior aspect of the horizontal ramus of the left hemi-
ogy; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese mandible extending to the gingiva. It was globular in shape, with
Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants.
Corresponding author at: Department of General Pathology, Christian Medical a smooth surface, well dened margins and rm consistency. The
College, Vellore, India. Tel.: +91 0416 228 2005. skin over the swelling was normal. There were no ulcers or satel-
E-mail address: (S. Mukhopadhyay). lite lesions. A CT scan of the head and neck region showed a large
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 183

exophytic, expansile, lytic lesion involving most of the mandibular it as a variant of ameloblastoma [4]. The striking difference in
body on the left side extending into the vertical ramus with focal the anatomic location, mainly in the anterior premolar region of
areas of cortical breach (Fig. 1). The biopsy from the lesion was the maxilla/mandible, unusual radiologic presentation of mixed
reported as desmoplastic ameloblastoma. The patient was posted radiolucency-radioopacity with ill-dened borders and distinctive
for wide local excision. Intraoperatively, multiple swellings were histopathology of extensive stromal desmoplasia with scattered
noted in the horizontal ramus with egg shell crackling for which odontogenic epithelium makes it a distinct clinicopathologic entity.
extended segmental mandibulectomy with free bular osteocuta- Hybrid lesion of desmoplastic ameloblastoma and conventional
neous ap reconstruction was done. ameloblastoma is an unusual variant of ameloblastoma, which was
On gross pathological examination, the surgical specimen rst described by Waldron and El-Mofty in 1987. Hybrid lesions
showed multiple nodular swellings in the ramus of the mandible show typical microscopic features of DA characterized by pro-
measuring 7 cm 6 cm 5 cm in toto with cystic and solid cut sur- nounced stromal desmoplasia together with areas of follicular,
face. The cystic area contained greenish-mucoid material while the plexiform and acanthomatous ameloblastoma [4,5].
solid area appeared homogeneously white (Fig. 2A). Histopatholog- In the literature, it is noticed that the DA represented the
ically, the lesion demonstrated solid and cystic components. The least common odontogenic tumor. Among the 89 cases of amelo-
solid areas of the tumor showed islands, nests and anastomosing blastomas studied by Takata et al., 7.9% were diagnosed as DA and
strands of ameloblastic epithelium with central stellate reticulum only 1.1% as hybrid lesion [10]. In Japan, the DA was only 5.3% of
and peripheral palisading of nuclei (Fig. 2B). Few of the islands all the cases of intraosseous ameloblastomas diagnosed in 27 years
showed central squamoid differentiation and edema with forma- [6,7]. As per an Indian study, ameloblastoma accounted for 1.18% of
tion of microcysts. Also, there were irregular shaped epithelial the total surgical specimens received over a period of 25 years with
islands, few displaying a swirled hypercellular center with spindle DA accounting for only 2.25% of the various histological variants of
shaped to squamoid epithelial cells, absent peripheral palisading ameloblastoma. HA was not reported [8]. The present case is the
and surrounded by narrow zones of loosely structured connec- rst case of HA to be reported from our institution.
tive tissue embedded in a desmoplastic stroma (Fig. 2C and D). The HA has an almost equal sex predilection and occurs in a wide
There was focal inltration of tumor into the cortical bone (Fig. 2E). age range of 1782 years. Strikingly, it has been reported predomi-
There was no necrosis or signicant mitotic activity. The cystic nantly in the Asians with a denite mandibular predilection [6]. Not
component showed a brous cyst wall lined by ameloblastic epithe- surprisingly, even the present case is a 41-year-old female patient
lium composed of columnar to cuboidal cells with hyperchromatic with the lesion in the anterior ramus of the left hemi-mandible.
nuclei displaying reverse polarity and basilar cytoplasmic vacuola- The literature shows that most cases of HA present as mixed
tion (Fig. 2F). The overall features were suggestive of a hybrid form radiolucent and radiopaque lesions with irregular borders simi-
of desmoplastic ameloblastoma as both typical desmoplastic areas lar to the common radiological pattern observed in DA or bro
with plexiform areas were seen. osseous lesions. A few cases of HA also present as multilocular
radiolucencies similar to the common radiographic pattern of con-
3. Discussion ventional ameloblastoma [9]. Takata et al. also believed that the
mixed pattern expresses the inltrative nature of the tumor. As the
Desmoplastic ameloblastoma was rst described by Tussole DA inltrates the bone marrow spaces, remnants of the original
et al. in 1984 and reviewed by Waldron and El-Mofty in 1987 non-metaplastic or non-neoplastic bone remain in the tumor tis-
[5]. In 1992, the World Health Organisation (WHO) regarded 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
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 not fully understood, the lesion still needs more extensive tumor
thin irregular internal septations. 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 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
None. lesion of desmoplastic and conventional ameloblastoma: immunohistochem-
ical aspects. Rev Bras Otorrinolaringol 2006;72:70913.
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