Case Report

Hemangiomatous Ameloblastoma with Dentinoid Formation:

A Sui Generis Case Report
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Neha Gupta, Pramod Kumar1, Megha Malu2, Manjula Marandi3

Senior Resident, Ex Senior Resident, Department of Oral Pathology, Microbiology and Forensic Odontology, Dental College, RIMS, Ranchi, Jharkhand, 1Reader,
3

Department of Oral Pathology and Microbiology, CDCRI, Sundra, 2Reader, Department of Oral Pathology and Microbiology, Maitri Dental College and Research
Institute, Anjora, Chhattisgarh, India
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Abstract
Hemangiomatous ameloblastoma has long been explicated as a divergent of solid multicystic ameloblastoma (SMA). Described first by Kuhn
in 1932, it is histologically distinct from a conventional ameloblastoma by the presence of spaces filled with blood or large endothelial lined
capillaries. Less than 12 cases have been reported till date and mostly in the middle‑aged population with a predilection for the mandibular
posterior region. The unlikeness of the case being reported here is the fact that it was manifested in a 16‑year‑old teenage girl. Being an
extraordinary version of SMA, very little is known about its behavior and prognosis. In the following case report, we try to annotate the
clinical, radiological, and the histological features that lead us to the diagnosis of hemangiomatous ameloblastoma with dentinoid formation.

Keywords: Blood filled spaces, dentinoid, hemangiomatous ameloblastoma, solid multicystic ameloblstoma

Introduction been postulated to serve as either an alternative pathogenetic

The solid multicystic ameloblstoma (SMA) is traditionally
considered to be a benign epithelial neoplasm which is slow
growing but locally destructive somewhat mimicking a
low‑grade malignant tumor. However, it lacks the potential
to metastasize. Many authors in their various epidemiological
studies have unanimously agreed with the calculations made
by Gardner regarding the mean age of occurrence which was
39 years for the diagnosis of the classical SMA, 22 years for the
unicystic and 51 years for the peripheral type.[1] A definite male
predilection (1:1.1) is ascertained with most number of cases
manifested in the mandibular molar region in both sexes (1:5.8)
with an associated impacted third molar.[1] The discovery of
recurrent activating mutations in fibroblast growth factor
receptor 2 (FGFR‑2), gene encoding protein serine/thereonine
protein kinase B-Raf (BRAF), and rat sarcoma (RAS) in a large
majority of ameloblastomas has implicated dysregulation of
mitogen activated protein kinase (MAPK) pathway signaling as
a critical step in the pathogenesis of this tumor. Some degree of
controversy exists regarding the role of mutations affecting the
sonic hedgehog pathway, specifically Smoothened, which have
mechanism or secondary mutations.[2‑5]
Based on clinical, radiological and histopathological attributes
of ameloblastoma four subtypes of it can be recognized. These
include the classical SMA, the unicystic type which has a
better prognosis, the hybrid type which is the desmoplastic
ameloblastoma and lastly its peripheral variant. Histological
variations of ameloblastoma include the follicular and the
plexiform patterns which are the predominant ones. Other
variants include the acanthomatous, granular, desmoplastic,
basal cell, clear cell, keratoameloblastoma, mucous cell
differentiation, and hemangiomatous. Although variations in the
histomorphological patterns do not primarily affect the behavior
and prognosis except in cases of unicystic, desmoplastic,
and hemangiomatous types.[1] The hemangiomatous type is
essentially characterized by the blood filled spaces or large
endothelial lined vascular channels.[1] Several theories have
Address for correspondence: Dr. Neha Gupta,
Department of Oral Pathology, Microbiology and Forensic Odontology,
Dental College, RIMS, Ranchi, Jharkhand, India.
E‑mail: doc.gneha@gmail.com

Received: 02‑01‑2023 Accepted: 20‑01‑2023 Published: 30-06-2023

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DOI: How to cite this article: Gupta N, Kumar P, Malu M, Marandi M.

10.4103/ijmo.ijmo_1_23 Hemangiomatous ameloblastoma with dentinoid formation: A sui generis
case report. Int J Med Oral Res 2023;8:35-9.

© 2023 International Journal of Medical and Oral Research | Published by Wolters Kluwer - Medknow 35
 Gupta, et al.: Haemangiomatous ameloblastoma with dentinoid formation

been postulated regarding the origin of the vascular component.
However, whether this represents a separate neoplasm, a
hamartomatous malformation or a part of the neoplastic process
per se is yet to be proved.[1] Ameloblastomas are typically seen
with no hard tissue formation. This can be explained due to the
presence of a mature connective tissue stroma which essentially
lacks the inductive ability. We, on the contrary came across
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arranged in the form of islands and interconnecting strands
with minimum intervening connective tissue stroma. Multiple
cystic spaces were noted within the follicles representing
cystic degeneration [Figures 3 and 4]. When examined under
higher magnification (×400), the lining epithelium revealed
the presence of tall columnar cells with polarized nuclei
resembling ameloblast‑like cells and central star‑shaped

dentinoid like material during histopathological evaluation
which was confirmed by Van Gieson Stain. The presence of
numerous vascular channels along with dentinoid like material
indispensably makes our case sui generis.
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cells appearing to be stellate reticulum like [Figure 5]. The
intervening connective tissue stroma was minimal and mature.
The most prominent feature that was noted was the presence of
large blood‑filled spaces along with numerous endothelial lined

capillaries [Figure 6]. Besides, this is a large eosinophilic area

Case Report was noted which resembled dentinoid like material [Figure 7].
To confirm the presence of dentinoid like material, the tissue
A 16‑year‑old female patient reported to the department of section was stained with Van Gieson Stain which stained the
oral pathology and microbiology for evaluation of a swelling speculated area red thus confirming the findings [Figure 8].
in the left lower back region of jaw since 6–8 months. The Overall, a predominant plexiform pattern with multiple
patient was apparently alright 8 months back when she cystic degeneration along with numerous blood‑filled spaces
noticed a small, painless swelling in the left posterior region and endothelial lined capillaries with dentinoid formation
of the mandible. The swelling gradually progressed causing was the hallmark of the lesion microscopically. These
difficulty in mastication along with esthethic disfigurement. findings navigated us to a final diagnosis of hemangiomatous
The patient gives no history of any associated symptoms ameloblastoma with dentinoid formation.
such as trauma, pain, or discharge of any kind. The patient
denied any dysphagia, odynophagia, difficulty in breathing,
or swallowing. The medical history and review of symptoms Discussion
were noncontributory. There was no history of similar The HA was first described by Kuhn in 1932 as a SMA in
lesions in the patient or members of her family. Extraoral which part of the tumor contained spaces filled with blood or
examination revealed a bilaterally asymmetrical face with a large endothelial‑lined capillaries.[1]
noticeable extraoral swelling present on the left mandibular
Later Van Rensburg et al. reported a similar case in a
aspect extending anteriosuperiorly from tragus of the left
26‑year‑old woman in the posterior aspect of left mandible.
ear to the midline and superioinferiorly from the left angle
Radiographs and computed tomography revealed a lesion
of mouth to the inferior border of the mandible [Figure 1].
with mixed radiolucent radioopaque appearance along with
Her lips were competent and lymph nodes were not palpable.
cortical bone expansion and mild root resorptions. Magnetic
Intraoral examination revealed a firm, diffuse swelling in the
resonance imaging revealed an intensity suggestive of bundles
left posterior mandibular region. The overlying mucosa was
of vascular structures or blood vessels in various stages of
of normal color and ulceration was not noted. On palpation,
thrombosis or slow flow. Histologically it revealed a plexiform
the swelling was bony hard with few areas exhibiting egg
ameloblastoma with a prominent vascular component thus
shell crackling. No mobility of the associated teeth was
pointing towards HA.[1] Similar findings were reported in our
noted. The patient was advised to go for extraoral radiographs
and the subsequent orthopantomogram (OPG) revealed a
multilocular radiolucent lesion with cortical thinning and
resorption of roots of 36, 37 [Figure 2]. Based on clinical and
radiological evidence a provisional clinical diagnosis of SMA
was made. Later, the patient was sent for incisional biopsy.
The preliminary histological findings of the incised tissue
suggested a diagnosis of plexiform ameloblastoma. The patient
was admitted for surgical intervention and the lesion was
excised under general anesthesia. A hemimandibulectomy with
marginal clearance was performed. Intraoperatively, the lesion
was found to be vascular. The excess bleeding was controlled
by ligating the feeder vessel and concomitant use of bone wax.
After achieving complete hemostasis, the wound was closed
in layers. The excised tissue was sent to the department of oral
pathology for histopathological evaluation.
Microscopic examination of H‑ and E‑stained sections Figure 1: Extraoral photograph showing diffuse swelling on the left
revealed the presence of odontogenic epithelium which were mandibular aspect

36 International Journal of Medical and Oral Research ¦ Volume 8 ¦ Issue 1 ¦ January-June 2023
 Gupta, et al.: Haemangiomatous ameloblastoma with dentinoid formation

case. Very few cases of HA have been reported [Table 1] thus believed that the unusual vascularity is not due to a neoplastic
making it a relatively rarer variant of SMA. process. According to him, there is an absence of vasoformative
process in toto. During stromal cyst formation in the ordinary
Several theories have been proposed explaining the vascular
type of plexiform ameloblastoma, the subsequent persistence
component of the lesion. One of them could be an excessive
stimulation of angiogenesis during tumor development
and trauma postextraction procedures. Some believe HA
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to be a collision tumor formed by two separate entities,

i.e. hemangioma and ameloblastoma. Lucas [1] however
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Figure 3: Photomicrograph showing odontogenic epithelium arranged

in a plexiform pattern
Figure 2: Orthopantomogram

Figure 5: Photomicrograph showing ameloblast-like cells with central

Figure 4: Photomicrograph showing odontogenic epithelium stellate reticulum like cells

Table 1: Year wise literature review of haemangiomatous ameloblastoma[6]

Author Year Age/sex Site Treatment Healing and prognosis
Aisenberg MS[6] 1950 48/female Right posterior mandible Enucleation Uneventful
Lucas RB[1] 1957 43/female Right posterior mandible Resection Uneventful
van Rensburg[1] 2001 26/female Left posterior mandible Partial hemimandibulectomy Patient refused any treatment
Ide F[6] 2001 56/male Maxillary anterior region Enucleation and curettage Good
Tamgadge et al.[7] 2010 31/male Mandibular left premolar‑molar region Enucleation and curettage Good
Sharma et al.[8] 2012 15/male Maxillary right posterior region Enucleation Good
Harshvardhan SJ[6] 2012 42/male Mandibular right posterior region Hemimandibulectomy Uneventful
Sarode GS[6] 2013 18/male Mandibular right posterior region Curettage Unknown
Rajmohan M[6] 2014 20/male Mandibular right posterior region Hemimandibulectomy Unknown
Kasangari et al.[6] 2015 35/female Mandibular left posterior region Enucleation Unknown
Chatterjee et al.[9] 2016 35/male Mandibular left posterior region Enucleation Unknown
Our case* 2017 16/female Mandibular left posterior region Hemimandibulectomy Unknown
*Also revealed dentinoid like material which was not a feature in other cases

International Journal of Medical and Oral Research ¦ Volume 8 ¦ Issue 1 ¦ January-June 2023 37
 Gupta, et al.: Haemangiomatous ameloblastoma with dentinoid formation
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Figure 6: Photomicrograph showing numerous endothelial lined vascular
channels
Figure 7: Photomicrograph showing dentinoid
Figure 8: Photomicrograph showing dentinoid under van gieson stain (VGS)
38
and dilatation of blood vessels lead to this secondary change.
So far, many authors have come up with their theories
explaining the vascularity of the tumor, but a definite answer
is still not vested. However, another feature that was noted in
our case was the appearance of dentinoid‑like material which
was confirmed by van gieson stain (VGS). The formation of
dentinoid‑like material is still a matter of great controversy.
In the cases reported by Slabbert et al. and Orlowski et al.[10]
and in the present report, hard tissue formation was found
with tumor diagnosed as ameloblastoma. Because of the
presence of odontogenic ectomesenchyme in a few isolated
cases, inductive changes take place leading to the formation
of dentin and enamel in parts of the tumor. Papagerakis
et al.[11,12] demonstrated that ameloblastic epithelial cells
in mixed odontogenic tumors expressed gene products
normally present in ectomesenchymal cells and resulted in
conversion and co‑expression of mesenchymal phenotype.
Dentinoameloblastoma shows dentinoid induction without
concomitant enamel formation while odontoameloblastoma
shows enamel and dentinoid formation in ameloblastoma.
Thus, it is probable that neoplastic epithelial cells committed
to ameloblastic differentiation could produce the dentinoid
which exists in some tumors. It has been seen that induction in
ameloblastomas is extremely rare which suggest that attempts
to classify odontogenic tumors on the basis of induction will
be unsound.[6,8,13]
Conclusion
Our case clearly demonstrated a marked and distinct vascular
component in histological sections along with dentinoid‑like
material. Hemangiomatous ameloblastoma and other lesions
with an extensive vascular component may lead to fatality
even following minor procedures. Therefore, it is paramount
to keep such rare variants in mind whenever a differential
diagnosis of SMA is contemplated. The biologic behavior
of HA is thought to be similar to that of the conventional
ameloblastoma, but because very few cases have been
reported with no long‑term follow‑up, the pathogenesis and
clinical features are not yet fully comprehended. Its biologic
behavior is also unpredictable. So far, it is still ambiguous
regarding its status as a distinct entity or just a vascular
variant of ameloblastoma which certainly demands further
documentation and scrutiny.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form, the legal guardian has given
his consent for images and other clinical information to be
reported in the journal. The guardian understands that names
and initials will not be published and due efforts will be made to
conceal patient identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
International Journal of Medical and Oral Research ¦ Volume 8 ¦ Issue 1 ¦ January-June 2023
 Gupta, et al.: Haemangiomatous ameloblastoma with dentinoid formation
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