Case Report

Basal Cell Ameloblastoma: A Rare Histological

Variant of an Uncommon Tumor
Meela Sridhar, L. Raja Bhaskar Reddy1, Sagar Kharat2, B.S. Mahesh3, Lakshmi Gandi4,
Ashish Mahendra5, Pankhuri Nigam6, Parveen Grewal6
Department of Oral and Maxillofacial Surgery, HKDET’S Dental College, Humnabad, Bidar, Karnataka, 1Department of
Oral Medicine and Radiology, Kalinga Institute of Dental Sciences, Bhuvneswar, 2Department of Orthodontics, Triveni
Dental College, Bilaspur, Chhatisgarh, 3Department of Oral Medicine and Radiology, Vydehi Institute of Dental Sciences
and Research Center, Bangalore, 4Department of Oral and Maxillofacial Surgery, Vyas Dental College and Hospital,
Jodhpur, Rajasthan, 5Department of Oral Pathology, Chandra Dental College, Barbanki, 6Department of Oral Pathology and
Microbiology, Shree Bankey Bihari Dental College, Ghaziabad, Uttar Pradesh, India

Address for correspondence:

Abstract Dr. Meela Sridhar,
Oral and Maxillofacial Surgery, HKDET’S Dental College,
Ameloblastomas are an inscrutable group of oral tumors. Humnabad, Bidar, Karnataka, India.
Basal cell ameloblastoma is a rare variant of ameloblastoma E‑mail: [email protected]
with very few cases reported until date. The tumor is
composed of more primitive cells and has less conspicuous
peripheral palisading. It shows remarkable similarity to
Access this article online
basal cell carcinoma, basal cell adenoma and intra‑osseous
adenoid cystic carcinoma. This report describes the case of a Quick Response Code:
27‑year‑old male with an ameloblastoma in the right posterior Website: www.nigerianjsurg.com
mandible. Orthopantomography computed tomography and
finally histopathological examination directed us toward the
confirmatory diagnosis of basal cell variant of ameloblastoma. DOI:
Considering the rarity of the lesion and histological paradox *****
regarding its diagnosis, we report here an interesting and rare
case of basal cell ameloblastoma of the mandible with emphasis
on differential diagnosis from other entities with basaloid
differentiation having varying prognosis. After surgery, for (2.6%) cases.[2] Basal cell ameloblastoma is a rare variant with
long‑term follow‑up at regular intervals is recommended as only a few cases described till date with insufficient information
no sufficient statistical information regarding the behavior of about his biological behaviour. It is reported to occur primarily
this tumor is available. in peripheral locations, but has been seen intraosseously, albeit
rarely.[3] Histologically, basal cell ameloblastoma consists of darkly
Keywords: Ameloblastoma, basal cells, desmoplastic, stained cells distributed predominantly in a trabecular pattern
odontogenic
with little evidence of palisading at the periphery.[2] Although
the small number of cases makes a precise assessment difficult,
the tendency for recurrence or malignant transformation in
this variant is considered to be the same as other variants
Introduction of ameloblastoma.[4] The microscopic features of basal cell
Odontogenic tumors comprise of a complex group of lesions of ameloblastoma, however, are similar to those of several malignant
diverse histopathologic types and clinical behavior. Ameloblastoma tumors, including basaloid squamous cell carcinoma (BSCC),[5,6]
represents 1% of all tumors and cysts that involve the cutaneous basal cell carcinoma (BCC) and solid‑type adenoid
maxillomandibular area and about 10% of odontogenic tumors. cystic carcinoma (ACC).[1] The pathologist may sometimes fail to
WHO defines it as a benign, but locally invasive polymorphic differentiate it from intraoral BCC leading to erroneous diagnosis.
neoplasm consisting of proliferating odontogenic epithelium, Hence, careful analysis of all the clinical and pathological data
which usually has a follicular or plexiform pattern, lying in a should be carried out before arriving at final diagnosis.
fibrous stroma.[1] Adebiyi et  al. analyzed histological variants
of ameloblastoma and found that follicular ameloblastoma Case Report
was the most common histological type (64.9%), followed by
plexiform meloblastoma (13.0%), desmoplastic (5.2%) and A 27‑year‑old male patient complained of painless swelling in
acanthomatous (3.9%) while the basal cell variant accounted relation to left lower mandibular posterior region. Past medical,

66
Jan‑Jun 2015 | Volume 21 | Issue 1 Nigerian Journal of Surgery
 Sridhar, et al.: Basal cell ameloblastoma

dental and family history of the patient was unremarkable. of flat plane [Figure 3b]. From these clinical and radiographic
There was no history of trauma or pus discharge. Extra‑oral findings, differential diagnoses of ameloblastoma or odontogenic
examination revealed facial asymmetry due to swelling on the keratocyst were considered. Fine needle aspiration was carried
left side of the face extending anteroposteriorly from 3 cm out, but it did not yield any fluid.
anterior to the ear till the corner of mouth and superior‑inferiorly,
extended from infraorbital margin to inferior border of mandible. To obtain a specific diagnosis, an incisional biopsy was done.
Clinical examination revealed firm to bony hard swelling in the H and E stained sections showed lesional tissue composed of
left mandibular region with normal overlying skin. Intra‑oral nests of uniform basaloid cells. No stellate reticulum was seen in
examination revealed obliteration of buccal sulcus in the region the central portion of the nests. The peripheral cells were cuboidal
of 34, 35, 37 as seen in Figure 1. Orthopantomograph as to short columnar with reversal of polarity. Fibrous septa divided
observed in Figure 2 revealed multiple multilocular radiolucencies the lesional tissue giving it a lobular pattern [Figure 4]. Based on
in the left side of mandibular body and ramus area involving the available supporting evidence, final diagnosis of basal cell
coronoid and condylar process. There was thinning of inferior Ameloblastoma was given.
border of mandible. Posterior border of ramus and right side
of mandible appeared normal. Coronal slice of computed Under general anesthesia, tumor mass was exposed buccally and
tomography (CT) scan showed expansion of medial and lateral lingually and osteotomy cut was placed and completed buccally
border of the left side of ramus with thick and curved bony septa and lingually and tumor mass was excised with bone margin
and homogenous density [Figure 3a]. Axial slice CT at level of of 1.5 cm and also by encompassing surrounding healthy soft
mandible showed soft tissue mass in left side of mandible with tissue. Free fibula osteocutaneous graft of 16 × 4.5 × 3 cm in
complete destruction of buccal and lingual plates and remnant dimension was harvested from right leg along with peroneal
of bone within mass extending into adjacent soft tissue with loss artery and vessels. Antibiotics, analgesics and antiinflammatory

Figure 2: Orthopantomograph showing multiple multilocular

radiolucencies in left side of body of mandible and ramus area involving
Figure 1: Intra-oral photograph showing swelling in the left mandibular coronoid and condylar process
region with obliteration of buccal sulcus

a b
Figure 3: (a) Coronal slice of computed tomography (CT) scan showing
expansion of medial and lateral border of left side of ramus with thick
and curved bony septa and homogenous density; (b) Axial slice CT at
level of mandible showing soft tissue mass in left side of mandible with Figure 4: Photomicrograph showing nests of uniform basaloid cells
complete destruction of buccal and lingual plate and remnant of bone (H and E, scanner view) and inset showing basaloid cells with fibrous
within mass extending into adjacent soft tissue with loss of flat plane septa divided the lesional tissue giving it a lobular pattern (H and E, ×10)

67
Nigerian Journal of Surgery Jan‑Jun 2015 | Volume 21 | Issue 1
 Sridhar, et al.: Basal cell ameloblastoma

drugs were given postoperatively. Histopathological examination published in literature do not satisfy most of the strict criteria
of excised specimen reconfirmed the initial diagnosis. Surgical for the diagnosis.
wound healed uneventfully, and sutures were removed on
10th postoperative day. The postoperative recovery of the patient If basal cell type of growth occurs in the jaws, special care is
was uneventful and followed‑up of 2 years revealed no evidence necessary to distinguish between this type of ameloblastoma
of recurrence. and an intra‑osseous (ACC.[1] BSCC and ACC are a well‑defined,
highly malignant tumor both clinically and histologically.
Hence, distinction between basal cell ameloblastoma and these
Discussion differentially considered lesions is of paramount importance.
Published data showed that the ameloblastoma is a rare benign Immunohistochemical analysis of lesion with histological
odontogenic tumor, representing only 1% of all tumors and perplexity can direct pathologists toward confirmatory diagnosis.
cysts of maxilla and mandible. It attacks either sex and can Table 1 shows expression of different markers in basal cell
attack at any age, 50% occurs between age of 20 and 30 years. ameloblastoma, BSCC, ACC and BCC.[2,5,6,9,10]
Adebiyi et  al. clinicopathologically analyzed the histological
variants of ameloblastoma and observed that basal cell variant Recently Sandra et al. immunohistochemically compared proliferative
of ameloblastoma occurs most commonly in mandible of males activity of different variants of amelobalstoma using monoclononal
antiproliferating cellular nuclear antigen (anti‑PCNA) antibody and
with 31-40 year age as common age of occurrence.[2,3] The age,
monoclonal anti‑Ki‑67 antibody which are considered as reliable
sex and site in the present case of basal cell ameloblastoma
markers for cell proliferation, the authors found that the basal cell
described here agree with the data given in the literature.
solid multicystic ameloblastoma (SMA) had the highest labeling
indices for both PCNA and Ki‑67, indicating that the basal cell
The basal cell ameloblastoma is a rare variant of ameloblastoma.[3]
type is the most actively proliferating type and therefore the most
Histological feature is apparently benign, constitutes a puzzling
immature cells in an (SMA).[11]
paradox. Basal cell ameloblastoma tends to grow in an island like
pattern. The basaloid appearing cells in basal cell ameloblastoma
It is very difficult to predict the prognosis of Basal cell type of
tend to stain deeply basophilic and are nearly equivalent in ameloblastoma as very few cases of basal cell subtype has been
staining intensity with the peripheral layer of cells. The cells reported for valid statistical analysis, but recurrence of this lesion
in the central portion may be polyhedral to spindle shape but has been reported. There has been some debate regarding the
stellate reticulum like areas are notably absent. The typical cellular most appropriate method for management of ameloblastomas.
morphology and nuclear orientation of the peripheral cells as Conservative modalities include curettage, enucleation,
seen in other ameloblastomas are often altered. They tend to cryotherapy, cautery, laser usage, radiotherapy, chemotherapy
be low columnar to cuboidal and usually do not demonstrate and radical approach encompasses marginal, segmental and
reverse nuclear polarity with sub‑nuclear vacuole formation. composite resections.[12] Some authors favor the conservative
However, hyperchromatism and palisading of the nuclei normally approach as they believe that ameloblastomas though, locally
are retained.[7] Our histological findings were in unison with the invasive, are essentially benign in nature. Therefore, they should
described features. Basal cell ameloblastoma shows a remarkable be treated as such because of serious cosmetic, functional and
resemblance to BCC. Ide et al.[6] disagreed with the diagnosis reconstructive problems associated with it. Proponents of the
of oral BCC, the case earlier reported by Wedenberg et  al.[5] radical approach to the treatment of ameloblastomas argue
believing it to be a basal cell ameloblastoma on the basis of that, albeit these tumors are histologically benign, but are locally
available clinical and pathological data. They believed that it may aggressive, and their clinical behavior may be regarded as lying
often be difficult to establish an accurate diagnosis of basal cell somewhere between benign and malignant lesions.[13] Satisfactory
ameloblastoma. They claimed that at least two alleged cases of results have been achieved either with radical treatment or more
oral BSCC reported by Wedenberg et al.[5] and de Araújo et al.[8] conservative approaches whereas enucleation and curettage have

Table 1: Immunohistochemical expression of different markers in basal cell ameloblastoma, BSCC, adenoid cystic
carcinoma, basal cell carcinoma
Lesion Immunohistochemical marker
Ber‑EP4 C‑kit CK Others
(epithelial specific antigen) (CD 117)
Basal cell Negative Negative Positive for AE1/AE3, KL1, 34, E12, and MNF116 CK; ‑
ameloblastoma devoid of CK7, CK8, CK10, CK18, CK20, and EMA
BSCC Negative Negative Positive for high molecular weight CK Weak p63 positivity
Adenoid cystic Negative Positive Positive for CK 7, 14 Strong p63 positivity
carcinoma
Basal cell Positive Negative Predominant expression of K17 and the frequent Negative for EMA
carcinoma expression of K8 and K19 little K6/K16 and K1/K10
EMA: Epithelial membrane antigen, BSCC: Basaloid squamous cell carcinoma, CK: Cytokeratin

68
Jan‑Jun 2015 | Volume 21 | Issue 1 Nigerian Journal of Surgery
 Sridhar, et al.: Basal cell ameloblastoma

been reported with the highest recurrence rate of 55-90% of the 3. Saify F, Sharma N. Basal cell ameloblastoma: A rare case report
cases. Metastases following conservative management have also and review of literature. Oral Maxillofac Pathol J 2010;1:1-7.
been reported.[12,13] In addition to being a radio‑resistant tumor, the 4. Reichart PA, Philipsen HP, Sonner S. Ameloblastoma: Biological
profile of 3677 cases. Eur J Cancer B Oral Oncol 1995;31B: 86‑99.
intra‑osseous location of the ameloblastoma prevents the use of
5. Wedenberg C, Jesslén P, Lundqvist G, Lundgren J, Hellquist HB.
radiotherapy as an effective therapeutic option because radiation Basaloid squamous cell carcinoma of the maxilla. Oral Oncol
enhances the potential development of secondary tumors but 1997;33:141‑4.
may be performed in cases when surgery is not considered to be a 6. Ide F, Shimoyama T, Horie N. Basaloid squamous cell carcinoma
method of choice.[12,13] There is a need to run more evidence‑based versus basal cell ameloblastoma. Oral Oncol 1998;34:154‑5.
clinical studies of clinical practice guidelines as there is a lack of 7. Kessler HP. Intraosseous ameloblastoma. Oral Maxillofac Surg
complete consensus regarding appropriate treatment modality for Clin North Am 2004;16:309‑22.
ameloblastomas. It is very difficult to predict the prognosis of 8. de Araújo VC, Biazolla ER, Moraes NP, Furuse TA, Melhado RM.
it as very few cases of basal cell subtype were reported for valid Basaloid squamous carcinoma of the oral cavity. Report of a case.
Oral Surg Oral Med Oral Pathol 1993;75:622‑5.
statistical analysis, but recurrence of it is reported.[10]
9. Del Rosario RN, Barr RJ, Jensen JL, Cantos KA. Basal cell carcinoma
of the buccal mucosa. Am J Dermatopathol 2001;23:203‑5.
Conclusion 10. Emanuel P, Wang B, Wu M, Burstein DE. p63 Immunohistochemistry
in the distinction of adenoid cystic carcinoma from basaloid
Rarity of basal cell variant of ameloblastoma in conjunction squamous cell carcinoma. Mod Pathol 2005;18:645‑50.
with atypical histological feature constitutes a puzzling 11. Sandra F, Mitsuyasu T, Nakamura N, Shiratsuchi Y, Ohishi M.
paradox. Hence, diagnosis should be based not only on clinical Immunohistochemical evaluation of PCNA and Ki‑67 in
ameloblastoma. Oral Oncol 2001;37:193‑8.
and radiographic appearance, but also on histopathological
12. Ueda M, Kaneda T. Combined chemotherapy and radiotherapy
findings. Long‑term follow‑up at regular intervals after surgery for advanced maxillary ameloblastoma. A  case report.
is recommended. J Craniomaxillofac Surg 1991;19:272‑4.
13. Vohra FA, Hussain M, Mudassir MS. Ameloblastomas and their
References management: A review. J Surg Pak Int 2009;14:136‑42.

1. Kramer IR, Pindborg JJ, Shear M. Histological Typing of How to cite this article: ????
Odontogenic Tumors. 2nd ed.. Berlin: Springer‑Verlag; 1992. p. 11, 13.
2. Adebiyi KE, Ugboko VI, Omoniyi‑Esan GO, Ndukwe KC,
Oginni FO. Clinicopathological analysis of histological variants
of ameloblastoma in a suburban Nigerian population. Head Face
Med 2006;2:42. Source of Support: Nil, Conflicts of Interest: None declared.

69
Nigerian Journal of Surgery Jan‑Jun 2015 | Volume 21 | Issue 1