Ameloblastoma is a true neoplasm of enamel organ type&nbsp;tissue. It is the most common odontogenic neoplasm with more&nbsp;frequency in the mandible. A 20 years old male patient presented&nbsp;with a swelling in the right side of the mandible of 10 months&nbsp;duration. Orthopantomograph revealed multilocular radiolucency&nbsp;extending from the region of 46 to the condyle. Incision biopsy&nbsp;revealed features of plexiform ameloblastoma. Numerous vascular&nbsp;spaces of varying size were seen throughout the stroma.&nbsp;Excision biopsy also revealed similar findings. Based on these&nbsp;findings, a diagnosis of hemangiomatous plexiform ameloblastoma&nbsp;was made. Hemangiomatous ameloblastoma (HA) is still&nbsp;a controversial entity, with some pathologists ruling it out as a&nbsp;&nbsp;separate lesion. This paper discusses the possibility that HA&nbsp;might be an aggressive variant of ameloblastoma and reviews&nbsp;relevant literature.&nbsp

Anuthama, K

Ilayaraja, V

Prasad, H

Rajmohan, M

Shanmugasundaram, N

Thangam, P Tamil

English

Atharva Scientific Publications (E-Jounals)

Journal of Orofacial Research, January-March 2014;4(1):63-66 63
JOFR
Hemangiomatous Ameloblastoma: A Rare Variant
AbstrAct
Ameloblastoma is a true neoplasm of enamel organ type 
tissue. It is the most common odontogenic neoplasm with more 
frequency in the mandible. A 20 years old male patient presented 
with a swelling in the right side of the mandible of 10 months 
duration. Orthopantomograph revealed multilocular radiolucency 
extending from the region of 46 to the condyle. Incision biopsy 
revealed features of plexiform ameloblastoma. Numerous vas-
cular spaces of varying size were seen throughout the stroma. 
Excision biopsy also revealed similar findings. Based on these 
findings, a diagnosis of hemangiomatous plexiform ameloblas-
toma was made. Hemangiomatous ameloblastoma  (HA) is still 
a controversial entity, with some pathologists ruling it out as a 
separate lesion. This paper discusses the possibility that HA 
might be an aggressive variant of ameloblastoma and reviews 
relevant literature.
Keywords: Hemangiomatous ameloblastoma, Unicystic amelo-
blastoma, Ameloblastoma.
How to cite this article: Rajmohan M, Prasad H, Shanmu-
gasundaram N, Thangam PT, Ilayaraja V, Anuthama K. Heman- 
giomatous Ameloblastoma: A Rare Variant. J Orofac Res 
2014;4(1):63-66.
Source of support: Nil
Conflict of interest: None declared
IntroductIon
Ameloblastoma is a true neoplasm of the enamel organ type 
tissue, being a benign odontogenic tumor that develops 
from epithelial rests of Malassez.1 It is usually unicentric, 
nonfunctional, intermittent in growth, anatomically benign, 
clinically persistent and exhibits aggressive behavior. It 
represents 1% of all oral tumors and 11% of odontogenic 
tumors. Its peak incidence is in the 3rd to 4th decades of life 
and is often associated with an unerupted third molar which 
can be detected during the course of routine radiography.2
The vast majority of ameloblastomas arise in the man-
dible, and the majority of these are found in the angle and 
ramus region and seldom in the maxilla. Furthermore, based 
on clinical and radiographic characteristics, histopathology 
and behavioral and prognostic aspects, four subtypes or 
variants of ameloblastoma can presently be distinguished:3,4
i Solid multicystic ameloblastoma (SMA)
ii Unicystic ameloblastoma (UA)
iii Peripheral ameloblastoma (PA)
iv Desmoplastic ameloblastoma (DA), including hybrid 
lesions.
Variations in the histomorphologic patterns of amelo-
blastoma do not appear to have a significant bearing on their 
biologic behavior or prognosis, with the possible exceptions 
of unicystic, desmoplastic and hemangiomatous ameloblas-
tomas (HA).5 The HA is a solid multicystic ameloblastoma 
in which part of the tumor contains spaces filled with blood 
or large endothelial lined capillaries.4,6,7 
cAse HIstory
A 20-year-old male patient was referred to the department 
of oral and maxillofacial pathology, for the evaluation of 
a gradually enlarging swelling in the right side of his face, 
present for almost a year. Patient had visited a private dentist 
10 months back for the complaint, and the mandibular right 
third molar was extracted. However, the swelling did not 
regress following the extraction. 
On examination, a single diffuse swelling of size 4 × 5 cm 
was noticed in the right lower half of the face (Fig. 1). 
The swelling was firm, nonfluctuant and painless. Intraoral 
Hemangiomatous Ameloblastoma: A rare Variant
1M rajmohan, 2H Prasad, 3n shanmugasundaram, 4P tamil thangam, 5V Ilayaraja, 6K Anuthama
Case RepORt
10.5005/jp-journals-10026-1130
1,2,5,6Reader, 3Professor, 4Postgraduate Student
1,2,4-6Department of Oral Pathology, KSR Institute of Dental 
Science and Research, Tiruchengode, Tamil Nadu, India
3Department of Periodontics, Vivekananda Dental College for 
Women, Tiruchengode, Tamil Nadu, India
Corresponding Author: M Rajmohan, Reader, Department 
of Oral and Maxillofacial Pathology, KSR Institute of Dental 
Science and Research, KSR Kalvi Nagar, Thokkavadi 
Tiruchengode-637215, Tamil Nadu, India, Phone: 98944 
90259, e-mail: mrajmohanmds@gmail.com
Fig. 1: Single diffuse swelling in the right lower half of the face
64
M Rajmohan et al
Fig. 2: Intraoral picture shows obliteration of buccal vestibule Fig. 3: Orthopantomograph reveals multilocular appearance with 
‘thinned out’ mandible
Fig. 4: Photomicrograph showing numerous anastomosing strands 
of odontogenic epithelium (arrows head) (H&E stain, ×100)
Fig. 5: Photomicrograph showing numerous vascular spaces 
(arrows) (H&E stain, ×200)
examination revealed a nontender swelling in the right 
lower quadrant, extending from distal aspect of 46 up to 
and beyond the retromolar region, with expansion of the 
cortical plate and obliteration of the buccal vestibule in the 
region. The swelling involved the entire depth of the lingual 
vestibule also, thereby obliterating it (Fig. 2). The right sub-
mandibular lymph node was palpable, nontender and firm.
IMAGInG eXAMInAtIon
Orthopantamograph revealed a well-defined multilocular 
radiolucency extending from distal aspect of 46 posteriorly 
up to the condyle. The lesion appeared predominantly 
radiolucent with intermingling radiopaque septa giving a 
soap bubble appearance. The margins of the lesion were 
scalloped with cortication of antero-inferior and posterior 
borders. The inferior border of the mandible appeared 
thinned out in some areas (Fig. 3). 
HIstoPAtHoLoGIcAL eXAMInAtIon
Sections from the incision biopsy specimen revealed nume-
rous anastamosing strands of epithelium, made up of peri-
pheral cuboidal cells and central cells resembling stellate 
reticulum. The connective tissue stroma showed bundles 
of collagen fibres and chronic inflammatory cells. Few 
blood vessels were also noticed (Figs 4 and 5). A diagnosis 
of ameloblastoma was made based on these features, and 
surgical excision of the lesion was advised.
Under general anesthesia, hemimandibulectomy was 
done (Fig. 6). Sections from the excisional biopsy specimen 
revealed similar histopathological features as the incision 
biopsy. In addition, numerous vascular spaces of varying 
size were also noticed throughout the connective tissue 
stroma. At places, the vessels were large enough to com-
press the epithelial strands to almost a single layer of cells. 
Most vascular spaces were engorged with RBCs, and areas 
Journal of Orofacial Research, January-March 2014;4(1):63-66 65
JOFR
Hemangiomatous Ameloblastoma: A Rare Variant
of hemorrhage were also seen. Based on these histopatho-
logical findings, a final diagnosis of hemangiomatous variant 
of ameloblastoma was made (Figs 7 and 8).
dIscussIon
Hemangiomatous ameloblastomas have been reported in 
lesser frequency than the other classic variants of ameloblas-
toma, probably because many pathologists do not consider 
it as a separate variant. Smith considered the HA as being 
histologically similar to one of the other recognized types of 
ameloblastomas and not as a distinct histologic entity.8 The 
origin of the vascular component of these tumors has always 
been a source of considerable debate. Various theories have 
been put forth to explain the pathogenesis of the vascular 
component in HA.
During amelogenesis, many capillaries are associated 
with the outer enamel epithelium, providing much needed 
nutrition to the developing enamel organ. It is probable that in 
the HA these blood vessels are abnormally induced to become 
part of the tumor.9 Excessive stimulation of angiogenesis 
during tumor development, by inductive influences such 
those that occur during odontogenesis or by other factors, 
may also result in the overgrowth of the vascular elements 
in the odontogenic ectomesenchyme or in the adjacent 
connective tissue.4 Trauma to the region resulting in tissue 
damage, such as due to extraction of a tooth, could also be 
a stimulus that results in proliferation of epithelial cell rests 
in the periodontal ligament and subsequent tumor develop-
ment. Such tissue damage is usually followed by repair and 
this involves the formation of the granulation tissue in which 
neoangiogenesis in the form of proliferating endothelial cells 
and new capillaries is prominent. A disturbance in the neo-
plastic odontogenic tissue may result in excessive granulation 
tissue formation or the development of an abnormal vascular 
component.9 It has also been suggested that HA represents a 
collision tumor. In this type, two separate tumors grow in the 
same area and collide, and the tumor elements intermingle.5 
However, whether the vascular component of HA is part of 
the neoplastic process, or whether it represents a separate 
neoplasm, or is a hamartomatous malformation, has not been 
satisfactorily resolved.10 In our case, it could be possible 
that the removal of impacted 48 could have triggered the 
proliferation of blood vessels in the lesion.
The biologic behavior of HA is thought to be similar to 
that of the conventional ameloblastoma. A second school 
of thought considers HA being one of the more aggressive 
variants of ameloblastoma. However, because only a few 
cases of HA have been reported till date, the pathogenesis 
and clinical features are not yet fully understood and biologic 
behavior cannot be predicted.10 Surgical complications that 
can arise as a result of the rich vascularity should be given 
due importance during treatment planning. It would, there-
fore, seem prudent to approach a case of HA with caution, 
especially during the surgical treatment. 
Fig. 8: Photomicrograph showing vascular spaces engorged with 
RBCs (arrows) (H&E stain, ×200)
Fig. 6: Excised hemimandibulectomy specimen Fig. 7: Photomicrograph showing areas of hemorrhage 
(H&E stain, ×100)
66
M Rajmohan et al
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