Hematol Oncol Stem Cell Ther (2016) 9, 118–122

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CASE REPORT

Solid-type primary intraosseous squamous-cell carcinoma in the mandible: Report of arare case

http://dx.doi.org/10.1016/j.hemonc.2015.12.0051658-3876/� 2016 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 andDiagnostic Sciences, College of Dentistry, King Saud University, P.O.Box 60169, Riyadh 11545, Saudi Arabia.

E-mail address: drotaibi@ksu.edu.sa (O. Alotaibi).

Ohoud Alotaibi a,*, Nabil Al-Zaher b, Faiza Alotaibi b, Hatim Khoja c,Ahmed Qannam a

aDepartment of Oral Medicine and Diagnostic Sciences, College of Dentistry, King Saud University, Riyadh, Saudi ArabiabDepartment of Otolaryngology, Head and Neck Surgery, King Faisal Specialist Hospital & Research Centre, Riyadh,Saudi ArabiacDepartment of Pathology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia

Received 22 May 2015; accepted 12 December 2015Available online 16 January 2016

KEYWORDSPrimary intraosseoussquamous cell carcinoma;Solid type;Mandible;Intraosseous neoplasms

Abstract

Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant neoplasm that hasan 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 preexistingodontogenic cyst or tumor. The solid-type of this tumor is a central jaw carcinoma arisingde novo and has no initial connection with the oral mucosa. Herein, we report a case ofsolid-type PIOSCC involving the mandible in a 37-year-old male patient elucidating itshistopathological and imaging findings. The patient underwent surgical resection followed bypost-operative adjuvant radiotherapy. The close 2-year follow up of the patient revealedneither locoregional nor distant metastasis.� 2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is anopen access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-

nd/4.0/).

Introduction

Primary intraosseous squamous-cell carcinoma (PIOSCC) is arare jawbone malignant neoplasm uniquely exclusive to themandible and the maxilla. It was first designated by Loos [1]in 1913 as central epidermoid carcinoma. Willis [2], in 1948,

Squamous cell carcinoma in mandible 119

used the term intra-alveolar epidermoid carcinoma for thislesion. Later, in 1971, Pindborg et al. [3] regarded it as aprimary intraosseous carcinoma. It was classified as anodontogenic carcinoma and defined as ‘‘a squamous cellcarcinoma that arises within the jaw, with no initial connec-tion with the oral mucosa and presumably develop fromresidues of the odontogenic epithelium” [3]. In the latestWorld Health Organization classification, PIOSCC is subdi-vided according to its potential origin into three types:solid-type carcinoma, carcinoma arising from a keratocysticodontogenic 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 neoplasmsthat metastasize to the jaws, gingival carcinomas that haveinvaded the bone from the oral mucosa, and neoplasms thatoriginate from the maxillary sinus.

The solid-type PIOSCC is a central jaw carcinoma arisingde novo and has no initial connection with the oral mucosa.It has been hypothesized that this lesion originates fromodontogenic epithelial rests, including rests of Malassez,rests of Serres, and the reduced enamel epitheliumsurrounding a tooth prior to eruption [5]. The solid-typePIOSCC has been occasionally reported in the literature[6], as it is the rarest type among PIOSCCs [7]. This reportdescribes a solid-type PIOSCC as a rare lesion arising fromthe posterior area of the mandible, and reviews theliterature.

Case report

A 37-year-old male patient presented to the dental clinic atthe College of Dentistry, King Saud University, Riyadh, SaudiArabia, with a 3-month history of a left-cheek swelling thathas recently been associated with pain and impairment ofthe sensation of his lower lip. Prior to his visit to our clinic,the patient had been seen by a dentist elsewhere whothought that the jaw swelling was due to infection, andwas managed with a tooth extraction without improvement.The patient’s past medical history was otherwise unremark-able. His intraoral examination showed a firm, nonfluctuantswelling in the left retromolar area extending to the ramusof the left mandible. No mucosal lesion or abnormalitieswere present. The Orthopantomograph panoramic radio-graph revealed an ill-defined irregular radiolucency at the

Figure 1 Panoramic radiograph showing a radiolucent are

left mandibular ramus (Figure 1). The differential diagnosesof the lesion included osteomyelitis, osteosarcoma,intraosseous carcinoma, and ameloblastic carcinoma ofthe mandible. Incisional biopsy from the ramus was per-formed, which demonstrated features of squamous-cell car-cinoma. Based on the clinical, radiographic, and histologicalcorrelations, the diagnosis of intraosseous squamous-cellcarcinoma was established.

The patient was referred to a tertiary-care center thatspecializes in head and neck cancer treatment, King FaisalSpecialist Hospital & Research Centre in Riyadh, for furthermanagement. His head and neck computed-tomographyscan confirmed the presence of an irregular, destructivesoft-tissue mass lesion centered at the ramus of the leftmandible, which extended from the distal aspect of thesecond molar to the posterior border of the ramus and fromthe 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-tomographyscan. The patient underwent tracheostomy, total radicalparotidectomy, left hemimandibulectomy, zygomatic-archexcision, and manipulating fixation and wide local excisionof tumor in the left infratemporal and pterygoid fossae.The histopathological assessment of the surgical specimenconfirmed 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 tumorsize was 5.5 cm in the maximum dimension. The dissectedsurgical specimen of the neck showed reactive lymph nodeswith no evidence of malignancy. Postoperative adjuvantradiotherapy was administered, and the close follow-up ofthe patient for nearly 2 years by the multidisciplinary teamrevealed neither locoregional nor distant metastasis. Thepatient is presently scheduled for facial reanimation.

Discussion

Solid-type PIOSCC is a rare malignant disease that arises fromremnants of the odontogenic apparatus in the jaw [5]. Thediagnosis of such lesion is challenging, as precise clinicaland pathological details are important in eliminating an oral

a with ill-defined margins in the left mandibular ramus.

Figure 2 Computed-tomography images: (A) axial computed-tomography slice; (B) coronal computed-tomography slice. Anill-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 andpleomorphism. 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.

mucosal origin. Additionally, the histological sections shouldbe thoroughly examined to exclude the presence of any pre-

existing odontogenic lesions. Furthermore, a careful reviewof the history and radiographic investigations is mandatory

Squamous cell carcinoma in mandible 121

in excluding metastatic lesion from a distant site. Suei et al.[8] proposed widely used criteria for diagnosing this typeof PIOSCC: (a) an intact overlying oral mucosa precedingdiagnosis, except due to tooth extraction or trauma; (b)squamous-cell carcinoma with no histological evidence ofcystic components or other odontogenic tumor cells; and(c) no metastatic deposit from a distant primary at the timeof diagnosis and throughout a follow-up period of more than6 months. The present case fulfilled these criteria, andhence, 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-documentedcases; however, it is the rarest type among PIOSCCs [7].Lugakingira et al. [6] reviewed the literature between1996 and 2010, and found 32 cases were of the solid-typePIOSCC, 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 commonlyaffected than females [6]. Our patient had the expectedfindings of male gender and involvement of the posteriormandible. The age of affected patients ranged from24 years to 76 years, where most of the cases occurred inpatients 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 describedsolid-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 ofadvanced cases may include pain, swelling, numbness, andtrismus [6,8,11,12]. Our patient experienced a persistentpain and paresthesia of the lower lip. He reported that hehad a tooth extraction at a private clinic that suspectedhis initial symptoms to be due to a dental infection ratherthan a tumor. It is worth mentioning that, because odonto-genic infections occur more frequently, few cliniciansconsider other entities; therefore, misdiagnosis and wrongtreatment may occur. It has been reported that inteross-eous malignancy of the maxillofacial region can mimicdental infection, and hence, gets misdiagnosed [13,14].Yamada et al. [15] described cases of PIOSCC where theywere initially diagnosed as dental-infection-relateddiseases.

The radiographic and histological investigations areessential in diagnosing solid-type PIOSCC. Radiographically,the lesion can show numerous destructive effects on thebone. It usually exhibits an osteolytic appearance withill-defined and noncorticated borders [16,17], and our casefollowed this pattern. The microscopic features of the solid-type PIOSCC are not specific, as all neoplasms that originatefrom squamous epithelium need to be considered [8]. It ischaracterized by islands of epithelial malignant neoplasmshowing the features of squamous-cell carcinoma [4]. Thespecimen should be thoroughly examined to confirm thatthere is no evidence of odontogenic cysts or other odonto-genic tumor components [8].

The mainstay of treatment for oral squamous-cellcarcinoma is usually surgery. External beam radiotherapywith or without chemotherapy is generally employed inthree situations: (a) adjuvant to primary surgery to enhancelocoregional control for cases with unfavorable pathologicalfeatures, (b) primary treatment for cases unable to tolerateor 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 surgicalresection [20]. The prognosis is generally poor, supportingthe need for an initial aggressive surgical intervention todecrease the local recurrence rate [21]. The overall survivalrates at 1 year, 2 years, and 3 years have been reported as75.7%, 62.1%, and 37.8%, respectively [11]. Although organpreservation has become a prevalent treatment approachfor managing locoregionally advanced head and neck cancerof the hypopharynx, the oropharynx, and the larynx, thisapproach has not been widely applied to patients withoral-cavity squamous-cell carcinoma. There are noclinical-trial data comparing extensive primary surgicalapproaches to organ preservation because of the paucityof PIOSCC cases. All reports on PIOSCC strongly recommendaggressive surgical intervention [6,22–24]. The need foradjuvant chemotherapy and/or radiotherapy is controver-sial [12,25]. However, several studies recommend postoper-ative radiotherapy, which significantly improves the rates oflocal 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 PIOSCCaffecting the mandible, which may contribute to theexpanding database of this rare neoplasm. Although PIOSCCis rare, it is important to be considered in the differentialdiagnoses of any jaw radiolucency.

Conflicts of interest

The authors have no conflict of interest to declare.

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