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Case Report

Desmoplastic ameloblastoma of anterior mandible:

Case report of a rarity
Prashant Nahar
Department of Oral Medicine and Radiology, Pacific Dental College and Hospital, Debari, Udaipur-313 024, Rajasthan, India

ABSTRACT
Ameloblastoma, one of the most common odontogenic tumors of the jaws, presents classical clinical, radiographic and histopathological
diagnostic features exhibiting a benign but locally aggressive and destructive clinical course with a high rate of recurrence. A case
of desmoplastic ameloblastoma of mandible is discussed in this article, which presents itself as a rare variant of ameloblastoma
with unusual inconclusive clinico-radiographic features to be diagnosed as classical ameloblastoma and difficult to differentiate
from other suspected multilocular benign odontogenic or reactive lesions of the jaws. It is the typical histopathological picture of
the lesion exhibiting a blend of desmoplasia (collagenization) and ameloblastoma that leads to its final diagnosis and determines
its management.

Key words: Ameloblastoma, collagenization, desmoplasia, desmoplastic ameloblastoma

DOI: 10.4103/0972-1363.52830
DOI

INTRODUCTION However, it was only in 1984 that Eversole et al. discovered

Ameloblastoma, one of the most common odontogenic
tumors of epithelial origin in the jaws clinically presents as
non tender bony or cystic swelling, reaching enormous size
with passage of time, exhibiting a moderate to slow rate
of growth causing marked facial disfigurement, asymmetry
and displacement of vital adjacent structures.[1] The most
common site and age of occurrence is mandibular molar-
ramus region in 3rd and 4th decade of life. In behavior
they are histologically benign (except the ameloblastic
carcinoma) but locally aggressive and destructive with a
high rate of recurrence.[2,3] Although they may be diagnosed
clinically from their appearance and radiographic features,
the final diagnosis depends on the histopathologic findings,
which usually classify ameloblastoma as following types:[2,3]
1. Follicular
2. Plexiform
3. Acanthomatous (or Squamous metaplastic)
4. Granular Cell
5. Basaloid (or Basal Cell)
6. Cystic
7. Telangiectatic (or Hemangiomatous)
8. Ameloblastic Carcinoma / Malignant Ameloblastoma.
Address for correspondence:
correspondence Dr. Prashant Nahar, “Himadri”, 62, Sarvritu
Vilas, Udaipur-313 001, Rajasthan, India. E-mail: prashantnahar@rediffmail.com
a new and unusual histologic variant known as desmoplastic
ameloblastoma.[3] This discovery was substantiated by
reports of similar cases by El Mofty and Waldron in
1987 and a very few others thereafter.[4] In 1992, WHO
recognized it as a variant of ameloblastoma.[5] The term
‘desmoplastic’ or ‘desmoplasia’ stands for histologic finding
of abundant stroma with pronounced collagenization.
Thus desmoplastic ameloblastoma is a variant of benign
ameloblastoma histologically characterized by extensive
stromal desmoplastic proliferation.[6,7]
CASE REPORT
A middle-aged male about 31 years old sought treatment
for a disfiguring mandibular swelling in the anterior region,
present for 15 years. He revealed a history of insidious onset
as a small, painless, non tender nodule about 15 years ago
gradually reaching the present extent without any pain,
tenderness, paresthesia except drifting and mobility of
mandibular anterior teeth and difficulty while eating food,
speaking and tongue movements due to size of the mass.
There was no relevant history of any trauma or infection
in the region before the inception of the swelling. The
patient did not seek any result-oriented treatment except
extraction of right mandibular lateral incisor about 2 years
ago, which had been drifted and tilted. There was non-
significant past medical and family history. The patient was

146 Journal of Indian Academy of Oral Medicine and Radiology / October - December 2008 / Volume 20 / Issue 4
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Nahar: Desmoplastic ameloblastoma of anterior mandible
found to be of an average build, had normal gait with no
physical handicap and was alert, conscious, cooperative
and responsive to verbal commands with vital parameters
within normal range.
Extra-oral examination of the patient revealed asymmetrical
enlargement on right mandibular anterior region [Figure 1]
which on palpation was found to be non tender, ill defined,
firm to hard in consistency, fixed, non reducible and non
pulsatile roughly extending from right premolar to left
premolar region. The upper 2/3rd of the face, bilateral TMJs,
regional nodes and neck revealed no pathology.
Intra-oral examination revealed fair oral hygiene, a
complete set of permanent dentition with missing right
mandibular lateral incisor and anterior crossbite. A growth
[Figure 2] was revealed in the mandibular anterior premolar
region with distinct labio-buccal and lingual portions
grossly measuring about 8 × 8 cm in size. The labio-
buccal portion extended from 46 to 36 causing elevation
of the floor of the vestibule and cortical expansion. On
palpation it was non tender, firm in consistency overall
but fluctuant in 41 to 34 region, irregular in shape with ill
defined margins, non reducible, non pulsatile and fixed.
There was a depression in 42 region dividing the swelling
into two unequal halves. The lingual portion presented as
a dome shaped singular mass extending from 45 to 35 with
obliteration and elevation of the floor of mouth causing
postero-superior displacement of tongue in rest position.
It had other characteristics similar to the labio-buccal
counterpart. The overlying mucosa appeared stretched
and pale pink with no signs of sinus or discharge. The
overall swelling had led to mobility of 41 to 34 and drifting
of 44 to 31. Maxilla, palate and other components of oral
cavity were normal with no apparent pathology. Based on
history and clinical findings, the provisional diagnosis of
an odontogenic tumor was made.
INVESTIGATIONS
(A) Radiographs:
(1) OPG [Figure 3]: revealed
• an ill defined multilocular radiolucency with
irregularly thickened cortical lining extending from
43 to 36, causing expansion of the lower border
of the mandible in the symphysis region;
• another ill defined mixed radiopaque – radiolucent
mass extending from 46 to 33 exhibiting
irregularly thickened trabeculae with specks of
calcification;
• resorption of roots 32 to 35, mesioangular tipping
of 41.
(2) True occlusal view mandible [Figure 4]: revealed
Journal of Indian Academy of Oral Medicine and Radiology / October - December 2008 / Volume 20 / Issue 4
• an ill defined mixed radiopaque-radiolucent mass
extending from 46 to 35 on lingual aspect and from
46 to 33 on buccal aspect, revealing irregularly
thickened trabeculae with specks of calcification
and bicortical expansion;
• tipping and drifting of 41 horizontally towards 31.
(B) Hemogram and Serum Chemistry values were within
normal range.
(C) FNAC of the swelling yielded clear yellow fluid which on
histopathological examination supported the diagnosis
of an odontogenic tumor.
The overall clinical and radiographic features were
suggestive of a mixed solid and cystic odontogenic
tumor, probably an ameloblastoma, with differential
diagnosis of CEOT, odontogenic myxoma, central giant
cell granuloma, or ameloblastic fibroma.
(D) Incisional Biopsy: the histopathologic evaluation of the
specimen [Figure 5] revealed an abundant collagenous
fibrous connective tissue stroma interspersed with
islands and cords showing peripherally compressed
ameloblast-like cells surrounding a central area of
spindle or polygonal shaped stellate reticulum cells.
Some of the islands demonstrated cystic changes.
The overall histopathological features were suggestive
of ameloblastoma with desmoplasia showing cystic
changes.
FINAL DIAGNOSIS AND TREATMENT PERFORMED
The overall clinical, radiological and histopathological
picture was suggestive of desmoplastic ameloblastoma with
mixed solid and cystic changes. Based on this diagnosis,
the patient underwent resection of mandibular segment
extending from 46 to 36 and extraction of 47 in the
Department of Oral and Maxillofacial Surgery. The missing
segment was surgically reconstructed [Figure 6] and the
resected segment [Figure 7] was sent for histopathological
evaluation, which confirmed the biopsy findings. After
the surgery the patient had an acceptable, esthetic facial
profile [Figure 8] and was put on periodic recall for further
necessary treatment and rehabilitation.
DISCUSSION
Desmoplastic ameloblastoma can be considered as a rarity
with unusual radiographic and histologic features. [3] Its
incidence is very low as compared to that of the follicular
or plexiform variants[1] and till date only a few cases have
been reported in literature. Clinically it usually presents
as a tumor or swelling with a growth potential and local
aggressiveness at par with other variants. No reports of
malignant form have yet been reported.[1,3] The most
commonly reported site of occurrence is maxilla followed
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Nahar: Desmoplastic ameloblastoma of anterior mandible

Figure 1: Frontal profile showing asymmetrical enlargement anterior Figure 2: Intra-oral view showing growth in the mandibular anterior
mandible premolar region with distinct labial and lingual expansion

Figure 3: Pre-operative OPG revealing mixed radiodensity lesion Figure 4: Pre-operative true / cross sectional occlusal view
mandible mandible

Figure 5: Histopathology revealing abundant collagenous fibrous Figure 6: Post-operative OPG revealing mandibular resection and
connective tissue stroma interspersed with islands containing subsequent reconstruction
peripherally compressed ameloblasts surrounding a central area of
stellate reticulum cells

Figure 7: Resected mandibular segment sent for histopathological Figure 8: Post-operative profile of the patient
evaluation

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Nahar: Desmoplastic ameloblastoma of anterior mandible
by mandible in the anterior-premolar region. [4] This is
in striking contrast to that of the usual clinical site of
ameloblastoma. The mean age of occurrence is between
the 3rd and 5th decade with slight female and no racial
predilection and duration ranging from as short as 2
months to as long as 16–18 years.[1] The present case
however differed from this in that the patient was a male
and the site of occurrence was mandible.
Differential diagnosis of radiographic features
The other benign variants of ameloblastoma usually present
as:[2,8]
• well defined or circumscribed unilocular/ multilocular
radiolucency,
• mostly associated with unerupted tooth and
• causing cortical expansion (with erosion sometimes)
and displacement of adjacent vital structures.
The desmoplastic variant exhibits atypical and varied
radiographic features as:[1,3,4]
(a) localized irregular multilocular radiolucency with
indistinct borders, or
(b) a mottled, radiopaque/ radiolucent appearance with ill
defined margins, or
(c) a massive expansile osteolytic lesion with honeycomb,
mottled or multilocular appearance
(a), (b), (c) with unclear boundary between the tumor and
normal tissue.
These varied radiographic appearances at various jaw
sites make desmoplastic ameloblastoma radiographically
resemble ameloblastoma, CEOT, odontogenic myxoma,
central giant cell granuloma or ameloblastic fibroma in
mandible; and AOT, Fibro-osseous lesions, radicular or
globulomaxillary cysts in maxilla.[1]
Differential diagnosis of histopathological features
A classic ameloblastoma (e.g. follicular variety) is
characterized by:[2]
• islands, nests and cords of odontogenic epithelium
rimmed by columnar cells resembling ameloblasts;
• center of these epithelial structures contains loosely
arranged spindle or stellate shaped cells resembling
stellate reticulum of a developing tooth;
• surrounding fibrous connective tissue stroma of mature
collagen.
The desmoplastic ameloblastoma is characterized by:[1,3-5]
• abundant desmoplastic (or collagenous) proliferation
Journal of Indian Academy of Oral Medicine and Radiology / October - December 2008 / Volume 20 / Issue 4
of the stroma;
• loss of cell rich connective tissue;
• tumor elements present at the periphery of the lesion,
absence of capsule;
• existence of some ameloblastoma like structures with
peripherally compressed ameloblasts;
• some cystic or myxoid changes can also be seen.
Thus histologically the desmoplastic ameloblastoma has
to be differentiated from basal cell type ameloblastoma,
ameloblastic/odontogenic fibroma or a squamous odontogenic
tumor.[1,4] Hence a pathologist may fail to recognize and
diagnose desmoplastic variant accurately if the specimen
contains only a small amount of biopsied material.
Such a sort of stromal desmoplasia is not restricted to jaw
bones alone, rather it is encountered in a variety of other
benign and malignant epithelial neoplasms e.g. morphea
variant of basal cell carcinoma, desmoplastic follicular
carcinoma of thyroid, scirrhous carcinoma of the breast,
benign papillary proliferation of the breast.[3]
Significance of Desmoplasia: The mere presence of
extensive desmoplasia in epithelial tumors is of unknown
significance. However, it has been speculated that
such an extensive stromal desmoplasia in these tumors
might inhibit the growth of tumor cells.[3] This has been
indirectly supported by Shivas and Douglas who reported
a longer survival rates in patients with elastosis in breast
carcinoma. [9]
MANAGEMENT
Enucleation or curettage alone of the lesion may lead to
recurrence,[4] as there is indistinct boundary between the
tumor and normal tissue. Therefore complete resection and
regular follow up is recommended.[1,3] Recurrence rate and
prognosis of this variant is unknown because of the small
number of cases reported in the literature and lack of long-
term follow-up.[1,5] However, on the basis of the histological
features, it has been suggested by El Mofty and Waldron
that it may have a propensity to recur at least as often as
other ameloblastic variants.[5]
CONCLUSION
It can be seen from the foregoing discussion that
histopathological studies are mandatory for the correct
diagnosis of desmoplastic ameloblastoma as clinical and
radiographic features alone are inconclusive. Still some
more endeavor and zest is needed for an insight into the
etiopathogenesis, clinical course and prognosis of this rare
and interesting variant of ameloblastoma.
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Nahar: Desmoplastic ameloblastoma of anterior mandible

ACKNOWLEDGMENT 4. El Mofty SK, Waldron CA. A histopathologic study of 116

I am thankful to the Departments of Oral Medicine and Radiology,
Oral and Maxillofacial Surgery and the Department of Oral
Pathology, Nair Hospital Dental College, Mumbai for their
cooperation.
REFERENCES
1. Yoshimura Y, Saito H. Desmoplastic variant of ameloblastoma: Report
of a case and review of literature. J Oral Surg 1990;48:1231-35.
2. Shafer. Textbook of oral pathology. 4th ed. 1993. p. 276-85.
3. Eversole LR, Leider AS, Hansen LS. Ameloblastoma with pronounced
desmoplasia: Case report. J.Oral Maxillofac Surg 1984;42:735-40.
amelobalstomas with specialreference to the desmoplastic variant.
Oral Surg 1987;63:441.
5. Sukashita H, Miyata M, Okabe K, Kurumaya H. Desmoplastic
ameloblastoma in maxilla. J Oral Surg 1998;56:783-6.
6. Regezi JA, Kerr DA, Courtney RM. Odontogenic tumors: Analysis of
706 cases. J.Oral Surg 1978;36:771.
7. Gardner DG, Pecak AM. The treatment of ameloblastoma based on
pathologic and anatomic principles. Cancer 1980;46:2514.
8. Goaz and White. Oral Radiology – Principles and Interpretation.
3rd ed. 1994. p. 429-38.
9. Shivas AA, Douglas TG. The prognostic significance of elastosis in
breast carcinoma. Jr Coll Surg Edinb 1972;17:315-20.
Source of Support: Nil, Conflict of Interest: Nil

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150 Journal of Indian Academy of Oral Medicine and Radiology / October - December 2008 / Volume 20 / Issue 4