Int. J. Oral Maxillofac. Surg.

2007; 36: 864–866

doi:10.1016/j.ijom.2007.03.002, available online at http://www.sciencedirect.com

Case Report
Clinical Pathology

Aggressive maxillary squamous B.

F.
M.
Ruhin1,2,, G. Raoul3,
Kolb4, O. Casiraghi8,
Lecomte-Houcke7,
Ghoul2,5, M. Auriol6,
odontogenic tumour in a child: S.
J.
1
Ferri3
Stomatology and Maxillofacial Surgery
Department, Pitié-Salpêtrière University

histological dilemma and Hospital, Paris VI University, Paris, France;

2
Orofacial Biology and Pathology
Department, INSERM U 714, Paris, France;

adaptative surgical behaviour

3
Stomatology and Maxillofacial Surgery
Department, Roger Salengro University
Hospital, Lille, France; 4Oncology, Cervical,
Facial and Plastic Surgery Department,
Gustave-Roussy Institute, Paris-Villejuif,
France; 5Histology and Embryology
B. Ruhin, G. Raoul, F. Kolb, O. Casiraghi, M. Lecomte-Houcke, S. Ghoul, M. Auriol, Department, Dental Faculty of Monastir,
J. Ferri: Aggressive maxillary squamous odontogenic tumour in a child: histological Monastir, Tunisia; 6Anatomopathology and
dilemma and adaptative surgical behaviour. Int. J. Oral Maxillofac. Surg. 2007; 36: Histology Department, Pitié-Salpêtrière
864–866. # 2007 International Association of Oral and Maxillofacial Surgeons. University Hospital, Paris VI University, Paris,
Published by Elsevier Ltd. All rights reserved. France; 7Anatomopathology and Histology
Department, University B Hospital, Lille,
France; 8Head and Neck Anatomopathology
and Histology Department, Gustave-Roussy
Institute, Paris-Villejuif, France

Abstract. A case of a maxillary osteolytic tumour is described in a 9-year-old boy.

Histological analysis led to an initial diagnosis of benign squamous odontogenic
tumour, although this was not straightforward due to swelling, and cellular pseudo-
malignant and non-specific signs. Because of the young age of the patient, a local
surgical tumourectomy was first chosen with respect to the mixed dentition. For 10
months, the evolution was satisfactory. Then, a very aggressive tumoural recurrence
with lip and palate infiltration led to doubts as to the histologic nature of the tumour.
Efficient collaboration between several specialized pathologist teams finally
confirmed that this was a squamous odontogenic tumour but in a very aggressive Accepted for publication 2 March 2007
form. Radical surgery was then carried out. Available online 16 May 2007

Squamous odontogenic tumour (SOT) is a
rare, benign but locally infiltrative epithe-
lial tumour that develops from remnants of
the dental lamina, or the Malassez or
gingival epithelium8. To date, only 39
cases have been reported in the litera-
ture2,5,7. The particularly aggressive evo-
lution of this case in a young patient led
practitioners to revise the histological
diagnosis, treatment and prognosis.
Case report
A 9-year-old boy was referred to the Depar-
tment of Maxillofacial Surgery because of a
painful swelling of the left maxillary gin-
givae evolving over the previous 3 months.
There was no relevant clinical history.
Intraoral examination revealed a 1-in. left
maxillary tumescence concerning both the
anterior maxilla cortical bone and the hard
palate cortical plates, with mobility of the
following maxillary left teeth: central and
lateral incisors, canine, molar and canine
lacteal teeth. There was no cervical gang-
lion. Dental panoramic and alveolar radio-
graphs revealed a well-defined unilocular
1-in. radiolucent lesion. Tomodensito-
metric exploration showed a large osteoly-
tic lesion. A bulbing osteolysis of the left
maxillar alveolar wall was also noted.

0901-5027/090864 + 03 $30.00/0 # 2007 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
 Aggressive maxillary squamous odontogenic tumour in a child 865

Surgical biopsy under local anaesthesia

revealed a specific organized strands,
nests and trabeculae lying in a fibroblastic
stroma with a surrounding swelling reac-
tion. Two weeks later, after orthodontic
contention of the upper left teeth and
general anaesthesia, biopsic curettage
allowed the diagnosis of SOT made up
of numerous mature islands of benign
squamous epidermoid epithelium with a
plate cuboidal peripheral band. Kerati-
nized areas were not rare, but the squa-
mous cells did not display malignant
features, although there was some cellular
pleomorphism. Evolution controls were
satisfactory but, 1 year later, a recurrence
occurred infiltrating the adjacent median
upper lip, left nasogenian area, half the
anterior part of the right nasal floor, and
the anterior and left palate until the left
maxillary tuberosity (Fig. 1). Right
Fig. 1. Clinical photograph of the 9-year-old boy showing tumefaction of the left maxilla,
(2 cm  2.5 cm) and left (1 cm  1 cm) incisor–canine gingival mucosa ulceration and labial infiltration.
infra-hyoid adenopathies were also
detected by cervical palpation.
A second histological analysis revealed
some verrucous mucosal surface covered sive (as in this case)3,6,8. X-ray typically liferation could be suspected as well. For
by moderately ortho-keratinized stratified shows a non-specific triangular or semi- this reason, pathologists often give this
squamous epithelium (Fig. 2). A mild circular radiolucent area of bone destruc- lesion such a name as benign epithelial
degree of anisonucleosis and hyperchro- tion adjacent to the roots of an erupted odontogenic tumour, acanthomatous ame-
matism was found in some islands. The tooth and bordered by a dense sclerotic loblastoma, epithelial odontogenic tumour
final diagnosis was that of a SOT in an rim4,7. or acanthomatous ameloblastic fibroma.
aggressive form. Reductive first intention SOT or epithelial odontogenic tumour As similar to ameloblastoma as it may
chemotherapy was chosen (three doses of is often mistaken for acanthomatosis ame- appear, the lesion-specific histological
5-fluorouracil and Cysplatyl), to achieve loblastoma or well differentiated epider- appearance justifies the term of SOT8.
efficient tumoural reduction of 80–90%. moid carcinoma. Even if it is likely that Similar squamous cell proliferations
Then, a large surgical tumoural excision this benign odontogenic neoplasma has have been described in the parietal struc-
was realized on the anterior and left max- developed from Malassez epithelial rem- ture of odontogenic cysts, as in follicular
illae, superior lip, and anterior and inferior nants, an epithelial hamartomatosis pro- or apical cysts9,10. But these proliferations
nasal area with columella and inferior part
of nasal wings. Bilateral cervical lympha-
denectomy was also performed. Excision
of the invasive tumour was completed
with 1.5-cm margins, no osseous exten-
sion, no lymphatic migration and no peri-
nervous extension. Facial reconstruction
was performed by a dorsal and scapula
free flap. No postoperative radiotherapy
was required. The young boy is regularly
operated on for aesthetic and reconstruc-
tive surgery, including a growth-adapta-
tive palatal prosthesis, and evolution
controls. For 7 years, the state of the
patient has remained stable and satisfac-
tory with no suspect lesions.

Discussion
This case occurred in the youngest ever
reported patient with an SOT: the range in
the literature is from 11 to 67 years with a
mean of 37.4 years. Characterized by its
dentally close relationship, the maxillary
lesion has a strong predilection for the
incisive and canine area and can be inva-
Fig. 2. Photomicrograph showing numerous islands of benign, well differentiated, stratified,
squamous epithelium in an abundant, mature, connective tissue stroma, with mild chronic
inflammatory reaction. There are numerous islands of squamous cells without cytologic atypia
and surrounded by fibrous tissue. Each lobule is limited by basal or cuboid epithelial cells. No
cylindrical cell was detected refuting the diagnosis of ameloblastoma. The well differentiated
epithelial cells are united by desmosomal union bridges. There is no atypic nucleus and no
mitosis (hematoxylin and eosin stain, magnification 5).
866 Ruhin et al.
never develop into a real tumour, and
evaluation of the entire clinicopathologic
picture is necessary to exclude the diag-
nosis of squamous odontogenic tumour-
like proliferations in an odontogenic
cyst3,10. With regard to differential diag-
nosis with ameloblastoma, it was found in
the present case that the epithelial nests of
SOT are not lined by palisaded columnar
cells with reverse nuclear and subnuclear
vacuoles. These epithelial islands also do
not display the swirled centres that are
often seen in desmoplastic ameloblas-
toma8.
Patient youth, absence of cytological
abnormalities and normal mitotic rate,
refuted a diagnosis of primary intraosseous,
well differentiated squamous cell carci-
noma. Despite having the same histological
appearance and very quick clinical evolu-
tion, epithelial verrucous carcinoma was
not accepted because of negative P53
immunohistochemistry results and nega-
tive polymerase chain reaction analyses
for human papillomavirus.
SOT prognosis depends on local aggres-
sive recurrence. This lesion must be trea-
ted as a pseudo-malignant tumour,
especially if it is located in the maxilla
or is invading adjacent cortical bone1.
Treatment consists first of rigorous and
extensive surgical curettage saving the
teeth (as in this case)8. Then, in case of
recurrence and local infiltration, the treat-
ment must be curative by complete
tumoural excision with cervical adenect-
omy.
Acknowledgements. We thank all histo-
pathological specialists having accepted
to review the histological slides:
- Pr Lecomte-Houcke and Dr Leroy
(Claude Huriez University Hospital,
Lille).
- Dr Anne De Roquancourt (Saint Louis
University Hospital, Paris).
- Pr Morgan and Dr Odell (Guy’s, King’s
and St Thomas Dental Institute, Lon-
don).
- Pr Terrier-Lacombe (Gustave-Roussy
Institute, Paris-Villejuif).
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Address:
Blandine Ruhin
Stomatology and Maxillofacial Surgery
Department
Pitié-Salpêtrière University Hospital
Paris VI University
47-83 boulevard de l’Hôpital
75651 Paris Cedex 13
France
Tel: +33 1 42 16 13 05
Fax: +33 1 42 16 13 69.
E-mail: blandine.ruhin@psl.aphp.fr