Solid-Type Primary Intraosseous Squamous-Cell Carcinoma in the Mandible: Report of a Rare Case

Abstract

Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant neoplasm that has an exquisitely exclusive affection to the jawbone. It is defined as squamous cell carcinoma arising within the jaw and developing from residual odontogenic epithelium or from a preexisting odontogenic cyst or tumor. The solid-type of this tumor is a central jaw carcinoma arising de novo and has no initial connection with the oral mucosa. Herein, we report a case of solid-type PIOSCC involving the mandible in a 37-year-old male patient elucidating its histopathological and imaging findings. The patient underwent surgical resection followed by post-operative adjuvant radiotherapy. The close 2-year follow up of the patient revealed neither locoregional nor distant metastasis.

Full text

CASE REPORT
Solid-type primary intraosseous squamous-
cell carcinoma in the mandible: Report of a
rare case
Ohoud Alotaibi
a,*
, Nabil Al-Zaher
b
, Faiza Alotaibi
b
, Hatim Khoja
c
,
Ahmed Qannam
a
a
Department of Oral Medicine and Diagnostic Sciences, College of Dentistry, King Saud University, Riyadh, Saudi Arabia
b
Department of Otolaryngology, Head and Neck Surgery, King Faisal Specialist Hospital & Research Centre, Riyadh,
Saudi Arabia
c
Department of Pathology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia
Received 22 May 2015; accepted 12 December 2015
Available online 16 January 2016
KEYWORDS
Primary intraosseous
squamous cell carcinoma;
Solid type;
Mandible;
Intraosseous neoplasms
Abstract
Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant neoplasm that has
an exquisitely exclusive affection to the jawbone. It is defined as squamous cell carcinoma aris-
ing within the jaw and developing from residual odontogenic epithelium or from a preexisting
odontogenic cyst or tumor. The solid-type of this tumor is a central jaw carcinoma arising
de novo and has no initial connection with the oral mucosa. Herein, we report a case of
solid-type PIOSCC involving the mandible in a 37-year-old male patient elucidating its
histopathological and imaging findings. The patient underwent surgical resection followed by
post-operative adjuvant radiotherapy. The close 2-year follow up of the patient revealed
neither locoregional nor distant metastasis.
Ó2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an
open access article underthe CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-
nd/4.0/).
Introduction
Primary intraosseous squamous-cell carcinoma (PIOSCC) is a
rare jawbone malignant neoplasm uniquely exclusive to the
mandible and the maxilla. It was first designated by Loos [1]
in 1913 as central epidermoid carcinoma. Willis [2], in 1948,
http://dx.doi.org/10.1016/j.hemonc.2015.12.005
1658-3876/Ó2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
*Corresponding author at: Department of Oral Medicine and
Diagnostic Sciences, College of Dentistry, King Saud University, P.O.
Box 60169, Riyadh 11545, Saudi Arabia.
E-mail address: drotaibi@ksu.edu.sa (O. Alotaibi).
Hematol Oncol Stem Cell Ther (2016) 9, 118–122
Available at www.sciencedirect.com
ScienceDirec
t
journal homepage: www.elsevier.com/locate/hemonc

used the term intra-alveolar epidermoid carcinoma for this
lesion. Later, in 1971, Pindborg et al. [3] regarded it as a
primary intraosseous carcinoma. It was classified as an
odontogenic carcinoma and defined as ‘‘a squamous cell
carcinoma that arises within the jaw, with no initial connec-
tion with the oral mucosa and presumably develop from
residues of the odontogenic epithelium”[3]. In the latest
World Health Organization classification, PIOSCC is subdi-
vided according to its potential origin into three types:
solid-type carcinoma, carcinoma arising from a keratocystic
odontogenic tumor, and carcinoma arising from an odonto-
genic cyst [4]. The diagnosis of PIOSCC is often challenging,
as other lesions need to be excluded, such as neoplasms
that metastasize to the jaws, gingival carcinomas that have
invaded the bone from the oral mucosa, and neoplasms that
originate from the maxillary sinus.
The solid-type PIOSCC is a central jaw carcinoma arising
de novo and has no initial connection with the oral mucosa.
It has been hypothesized that this lesion originates from
odontogenic epithelial rests, including rests of Malassez,
rests of Serres, and the reduced enamel epithelium
surrounding a tooth prior to eruption [5]. The solid-type
PIOSCC has been occasionally reported in the literature
[6], as it is the rarest type among PIOSCCs [7]. This report
describes a solid-type PIOSCC as a rare lesion arising from
the posterior area of the mandible, and reviews the
literature.
Case report
A 37-year-old male patient presented to the dental clinic at
the College of Dentistry, King Saud University, Riyadh, Saudi
Arabia, with a 3-month history of a left-cheek swelling that
has recently been associated with pain and impairment of
the sensation of his lower lip. Prior to his visit to our clinic,
the patient had been seen by a dentist elsewhere who
thought that the jaw swelling was due to infection, and
was managed with a tooth extraction without improvement.
The patient’s past medical history was otherwise unremark-
able. His intraoral examination showed a firm, nonfluctuant
swelling in the left retromolar area extending to the ramus
of the left mandible. No mucosal lesion or abnormalities
were present. The Orthopantomograph panoramic radio-
graph revealed an ill-defined irregular radiolucency at the
left mandibular ramus (Figure 1). The differential diagnoses
of the lesion included osteomyelitis, osteosarcoma,
intraosseous carcinoma, and ameloblastic carcinoma of
the mandible. Incisional biopsy from the ramus was per-
formed, which demonstrated features of squamous-cell car-
cinoma. Based on the clinical, radiographic, and histological
correlations, the diagnosis of intraosseous squamous-cell
carcinoma was established.
The patient was referred to a tertiary-care center that
specializes in head and neck cancer treatment, King Faisal
Specialist Hospital & Research Centre in Riyadh, for further
management. His head and neck computed-tomography
scan confirmed the presence of an irregular, destructive
soft-tissue mass lesion centered at the ramus of the left
mandible, which extended from the distal aspect of the
second molar to the posterior border of the ramus and from
the sigmoid notch to the angle of the mandible (Figure 2).
The computed-tomography scan of the chest, abdomen,
and pelvis showed no distant metastasis. There was a posi-
tive uptake in the mass lesion and bilateral upper jugulodi-
gastric lymphadenopathy on positron-emission-tomography
scan. The patient underwent tracheostomy, total radical
parotidectomy, left hemimandibulectomy, zygomatic-arch
excision, and manipulating fixation and wide local excision
of tumor in the left infratemporal and pterygoid fossae.
The histopathological assessment of the surgical specimen
confirmed that the tumor was an intraosseous squamous-
cell carcinoma, well to moderately differentiated (Figure 3).
The islands of the tumor cells extended into the bone
(Figure 4), and perineural invasion was noted. The tumor
size was 5.5 cm in the maximum dimension. The dissected
surgical specimen of the neck showed reactive lymph nodes
with no evidence of malignancy. Postoperative adjuvant
radiotherapy was administered, and the close follow-up of
the patient for nearly 2 years by the multidisciplinary team
revealed neither locoregional nor distant metastasis. The
patient is presently scheduled for facial reanimation.
Discussion
Solid-type PIOSCC is a rare malignant disease that arises from
remnants of the odontogenic apparatus in the jaw [5]. The
diagnosis of such lesion is challenging, as precise clinical
and pathological details are important in eliminating an oral
Figure 1 Panoramic radiograph showing a radiolucent area with ill-defined margins in the left mandibular ramus.
Squamous cell carcinoma in mandible 119

mucosal origin. Additionally, the histological sections should
be thoroughly examined to exclude the presence of any pre-
existing odontogenic lesions. Furthermore, a careful review
of the history and radiographic investigations is mandatory
Figure 2 Computed-tomography images: (A) axial computed-tomography slice; (B) coronal computed-tomography slice. An
ill-defined osteolytic lesion is observed at the center of the left mandibular ramus.
Figure 3 Photomicrograph of the lesion shows well to moderately differentiated squamous cells with cellular atypia and
pleomorphism. Keratin-pearl formation is seen (hematoxylin-and-eosin stain).
Figure 4 Microscopic examination showing infiltrative squamous-cell nests invading the bone (hematoxylin-and-eosin stain).
120 O. Alotaibi et al.

in excluding metastatic lesion from a distant site. Suei et al.
[8] proposed widely used criteria for diagnosing this type
of PIOSCC: (a) an intact overlying oral mucosa preceding
diagnosis, except due to tooth extraction or trauma; (b)
squamous-cell carcinoma with no histological evidence of
cystic components or other odontogenic tumor cells; and
(c) no metastatic deposit from a distant primary at the time
of diagnosis and throughout a follow-up period of more than
6 months. The present case fulfilled these criteria, and
hence, was classified as solid-type PIOSCC.
It is difficult to determine the real incidence of the solid-
type PIOSCC, owing to the paucity of well-documented
cases; however, it is the rarest type among PIOSCCs [7].
Lugakingira et al. [6] reviewed the literature between
1996 and 2010, and found 32 cases were of the solid-type
PIOSCC, 29 of mandibular, and four of maxillary types.
The most commonly affected site is the posterior mandible.
It has been reported that males were more commonly
affected than females [6]. Our patient had the expected
findings of male gender and involvement of the posterior
mandible. The age of affected patients ranged from
24 years to 76 years, where most of the cases occurred in
patients older than 50 years [6]. The patient who is the sub-
ject of this report was 37 years old at the time of diagnosis,
which is not a common finding. Few reports have described
solid-type PIOSCC in patients younger than 40 years [9–11].
The lesion at early stages is usually asymptomatic 10;
however, the most common presenting features of
advanced cases may include pain, swelling, numbness, and
trismus [6,8,11,12]. Our patient experienced a persistent
pain and paresthesia of the lower lip. He reported that he
had a tooth extraction at a private clinic that suspected
his initial symptoms to be due to a dental infection rather
than a tumor. It is worth mentioning that, because odonto-
genic infections occur more frequently, few clinicians
consider other entities; therefore, misdiagnosis and wrong
treatment may occur. It has been reported that inteross-
eous malignancy of the maxillofacial region can mimic
dental infection, and hence, gets misdiagnosed [13,14].
Yamada et al. [15] described cases of PIOSCC where they
were initially diagnosed as dental-infection-related
diseases.
The radiographic and histological investigations are
essential in diagnosing solid-type PIOSCC. Radiographically,
the lesion can show numerous destructive effects on the
bone. It usually exhibits an osteolytic appearance with
ill-defined and noncorticated borders [16,17], and our case
followed this pattern. The microscopic features of the solid-
type PIOSCC are not specific, as all neoplasms that originate
from squamous epithelium need to be considered [8].Itis
characterized by islands of epithelial malignant neoplasm
showing the features of squamous-cell carcinoma [4]. The
specimen should be thoroughly examined to confirm that
there is no evidence of odontogenic cysts or other odonto-
genic tumor components [8].
The mainstay of treatment for oral squamous-cell
carcinoma is usually surgery. External beam radiotherapy
with or without chemotherapy is generally employed in
three situations: (a) adjuvant to primary surgery to enhance
locoregional control for cases with unfavorable pathological
features, (b) primary treatment for cases unable to tolerate
or unsuited for surgery, and (c) salvage treatment in the
persistent or recurrent disease setting [18,19]. The treat-
ment of choice for PIOSCC, in particular, is radical surgical
resection [20]. The prognosis is generally poor, supporting
the need for an initial aggressive surgical intervention to
decrease the local recurrence rate [21]. The overall survival
rates at 1 year, 2 years, and 3 years have been reported as
75.7%, 62.1%, and 37.8%, respectively [11]. Although organ
preservation has become a prevalent treatment approach
for managing locoregionally advanced head and neck cancer
of the hypopharynx, the oropharynx, and the larynx, this
approach has not been widely applied to patients with
oral-cavity squamous-cell carcinoma. There are no
clinical-trial data comparing extensive primary surgical
approaches to organ preservation because of the paucity
of PIOSCC cases. All reports on PIOSCC strongly recommend
aggressive surgical intervention [6,22–24]. The need for
adjuvant chemotherapy and/or radiotherapy is controver-
sial [12,25]. However, several studies recommend postoper-
ative radiotherapy, which significantly improves the rates of
local and regional control and disease-free survival [20,26].
Zwetyenga et al. [21] concluded that surgery and postoper-
ative radiation therapy might have the best results.
In conclusion, we presented a case of solid-type PIOSCC
affecting the mandible, which may contribute to the
expanding database of this rare neoplasm. Although PIOSCC
is rare, it is important to be considered in the differential
diagnoses of any jaw radiolucency.
Conflicts of interest
The authors have no conflict of interest to declare.
References
[1] Loos D. Central epidermoid carcinoma of the jaws. Dtsch
Monatschr Zahnheilk 1913;31:308.
[2] Willis RA. Pathology of tumors. St. Louis: C.V. Mosby; 1948, p.
760–801.
[3] Pindborg JJ, Kramer IRH, Torloni H. Histological typing of
odontogenic tumours, jaw cysts, and allied
lesions. Geneva: World Health Organization; 1971.
[4] Barnes L, Eveson JW, Reichart P, Sidransky D. Pathology and
genetics of tumours of the head and neck. Lyon: World Health
Organization classification of tumours; 2005, p. 9.
[5] Eversole LR. Malignant epithelial odontogenic tumors. Semin
Diagn Pathol 1999;1(16):317–24.
[6] Lugakingira M, Pytynia K, Kolokythas A, Miloro M. Primary
intraosseous carcinoma of the mandible: case report and review
of the literature. J Oral Maxillofac Surg 2010;68:2623–9.
[7] Reichart PA, Philipsen HP. Odontogenic tumors and allied
lesions. London: Quintessence; 2004.
[8] Suei Y, Tanimoto K, Taguchi A, Wada T. Primary intraosseous
carcinoma: review of the literature and diagnostic criteria. J
Oral Maxillofac Surg 1994;52:580–3.
[9] Mu
¨ller S, Waldron CA. Primary intraosseous squamous carci-
noma. Report of two cases. Int J Oral Maxillofac Surg
1991;20:362–5.
[10] Nolan R, Wood NK. Central squamous cell carcinoma of the
mandible: report of case. J Oral Surg 1976;34:260–4.
[11] Thomas G, Pandey M, Mathew A, Abraham E, Francis A,
Somanathan T, et al. Primary intraosseous carcinoma of the
jaw: pooled analysis of world literature and report of two new
cases. Int J Oral Maxillofac Surg 2001;30:349–55.
Squamous cell carcinoma in mandible 121

[12] Nomura T, Monobe H, Tamaruya N, Kishishita S, Saito K,
Miyamoto R, et al. Primary intraosseous squamous cell carci-
noma of the jaw: two new cases and review of the literature.
Eur Arch Otorhinolaryngol 2013;270:375–9.
[13] Bhadage CJ, Vaishampayan S, Kolhe S, Umarji H. Osteosarcoma
of the mandible mimicking an odontogenic abscess: a case
report and review of the literature. Dent Update
2013;40:216–8.
[14] Selden HS, Manhoff DT, Hatges NA, Michel RC. Metastatic
carcinoma to the mandible that mimicked pulpal/periodontal
disease. J Endod 1998;24:267–70.
[15] Yamada T, Ueno T, Moritani N, Mishima K, Hirata A, Matsumura
T. Primary intraosseous squamous cell carcinomas: five new
clinicopathologic case studies. J Craniomaxillofac Surg
2009;37:448–53.
[16] Matsuzaki H, Katase N, Matsumura T, Hara M, Yanagi Y,
Nagatsuka H, et al. Solid-type primary intraosseous squamous
cell carcinoma of the mandible: a case report with histopatho-
logical and imaging features. Oral Surg Oral Med Oral Pathol
Oral Radiol 2012;114:e71–7.
[17] Kaffe I, Ardekian L, Peled M, Machtey E, Laufer D. Radiological
features of primary intra-osseous carcinoma of the jaws.
Analysis of the literature and report of a new case. Dentomax-
illofac Radiol 1998;27:209–14.
[18] Bessell A, Glenny A, Furness S, Clarkson JE, Oliver R, Conway
DI, et al. Interventions for the treatment of oral and oropha-
ryngeal cancers: surgical treatment. Cochrane Database Syst
Rev 2011;9:CD006205.
[19] Robertson AG, Soutar DS, Paul J, Webster M, Leonard AG,
Moore KP, et al. Early closure of a randomized trial: surgery
and postoperative radiotherapy versus radiotherapy in the
management of intra-oral tumours. Clin Oncol (R Coll Radiol)
1998;10:155–60.
[20] To EHW, Brown JS, Avery BS, Ward-Booth RP. Primary
intraosseous carcinoma of the jaws. Three new cases and a
review of the literature. Br J Oral Maxillofac Surg
1991;29:19–25.
[21] Zwetyenga N, Pinsolle J, Rivel J, Majoufre-Lefebvre C, Faucher
A, Pinsolle V. Primary intraosseous carcinoma of the jaws. Arch
Otolaryngol Neck Surg 2001;1(127):794–7.
[22] Chaisuparat R, Coletti D, Kolokythas A, Ord RA, Nikitakis NG.
Primary intraosseous odontogenic carcinoma arising in an
odontogenic cyst or de novo: a clinicopathologic study of six
new cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod
2006;101:194–200.
[23] Huang JW, Luo HY, Li Q, Li TJ. Primary intraosseous squamous
cell carcinoma of the jaws: clinicopathologic presentation and
prognostic factors. Arch Pathol Lab Med 2009;133:1834–40.
[24] Mohyuddin N, Yao M. Primary intraosseous carcinoma of the
anterior maxilla: an unusual case and review of the literature.
Ear Nose Throat J 2011;90:35–7.
[25] Bernier J, Domenge C, Ozsahin M, Matuszewska K, Lefe
`bvre JL,
Greiner RH, et al. Postoperative irradiation with or without
concomitant chemotherapy for locally advanced head and
neck cancer. N Engl J Med 2004;350:1945–52.
[26] Cooper JS, Pajak TF, Forastiere AA, Jacobs J, Campbell BH,
Saxman SB, et al. Postoperative concurrent radiotherapy and
chemotherapy for high-risk squamous-cell carcinoma of the
head and neck. N Engl J Med 2004;350:1937–44.
122 O. Alotaibi et al.