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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
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CASE REPORT  
Year : 2017  |  Volume : 21  |  Issue : 1  |  Page : 182
 

Primary intraosseous odontogenic squamous cell carcinoma of the mandible


Department of Oral and Maxillofacial Surgery, Granada Virgen de las Nieves University Hospital, Granada, Spain

Date of Submission20-Nov-2016
Date of Acceptance13-Dec-2016
Date of Web Publication5-Apr-2017

Correspondence Address:
Paolo Cariati
Department of Oral and Maxillofacial Surgery, Granada Virgen de las Nieves University Hospital, Carretera de Jaen s/n, Granada 18013
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.203777

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   Abstract 


Primary intraosseous odontogenic squamous cell carcinoma (PIOSCC) is a rare tumor. The incidence is low, and approximately 200 cases are reported in literature. The etiology is associated with the malignant degeneration of embryological remains. Differential diagnosis includes alveolar carcinomas, jaw metastases from other locations, odontogenic tumors, and tumors of the maxillary sinus. However, the diagnosis could be delayed due to the absence of symptoms in early stages. Surgery represents the first choice treatment. Whereas, postoperative radiotherapy could be helpful to improve the overall survival. The prognosis is generally poor. In this report, we describe the case of a 77-year-old man accidentally diagnosed PIOSCC. The patient referred a tooth extraction and cystectomy 3 years before. The pathological examination of the cyst tissue evidenced an inflammatory cyst. However, no patient follow-up was performed. Hence, is important to stress that routine monitoring of patients affected by large inflammatory cysts of the jaw bones should be mandatory.


Keywords: Diagnostic delay, extirpative surgery, postoperative radiotherapy, primary intraosseous squamous cell carcinoma


How to cite this article:
Cariati P, Fernandez AB, Tara MP, Solis JF, Lara IM. Primary intraosseous odontogenic squamous cell carcinoma of the mandible. J Oral Maxillofac Pathol 2017;21:182

How to cite this URL:
Cariati P, Fernandez AB, Tara MP, Solis JF, Lara IM. Primary intraosseous odontogenic squamous cell carcinoma of the mandible. J Oral Maxillofac Pathol [serial online] 2017 [cited 2019 Aug 22];21:182. Available from: http://www.jomfp.in/text.asp?2017/21/1/182/203777





   Introduction Top


Primary intraosseous odontogenic squamous cell carcinoma (PIOSCC) is a rare and malignant tumor. The incidence is low and approximately 200 cases are reported in literature.[1] It affects the jaws, regardless of the interest of oral mucosa.[2] The etiology seems to be related to the malignant degeneration of embryological remains. In this line, epithelial rests of Malassez, dental lamina and epithelium of the dental follicle represent potential suspects.[3]

According to the 2005, the World Health Organization Classification of tumors, three subtypes of PIOSCC exist:[4]

  1. Solid tumor that invades the marrow spaces and provokes osseous absorption
  2. Squamous cell carcinoma (SCC) originated from the lining of an odontogenic cyst. Specifically, this subgroup includes two variants: keratocystic odontogenic tumor and odontogenic cysts
  3. SCC associated with other benign epithelial odontogenic tumors.


The diagnostic criteria of PIOSCC included undamaged oral mucosa and absence of distant metastasis during the follow-up. Moreover, the switch from the epithelium of the cyst to SCC is essential for a proper diagnosis.[5] Differential diagnosis includes alveolar carcinomas, jaw metastases from other locations, odontogenic tumors and tumors of the maxillary sinus. In this sense, diagnosis is often delayed by the absence of symptoms in early disease.[6] Fortunately, neck metastases from PIOSCC are uncommon. Two-thirds of the cases do not present ganglion involvement at presentation. However, patients show a poor prognosis.[7] In fact, due to the rarity of this disease, there are no established treatment protocols. The adequate surgical resection remains the gold standard for the treating this tumor.[8]

In this report, we describe the case of a 77-year-old man accidentally diagnosed PIOSCC. The patient referred a tooth extraction and cystectomy 3 years before. The pathological examination of the cyst tissue evidenced an inflammatory cyst. However, no patient follow-up was performed. Hence, this case underlines the importance of routine monitoring of patients presenting large inflammatory cyst. Although rarely this lesions might experience a neoplastic transformation.


   Case Report Top


The article describes a case of PIOSCC of the mandible affecting a 77-year-old man. More in detail, the patient was referred at maxillofacial outpatient department of Granada University Hospital by his general practitioner. In fact, the patient reported a progressive painless swelling in the left side of mandible. Thus, a careful anamnesis and oral inspection were carried out. Oral and extraoral examination evidenced an abnormal mandibular ballooning in the left side. In view of that, an orthopantomography and a computed tomography (CT) scan of the cervicofacial area were performed for allowing the proper diagnosis. Panoramic radiography evidenced the presence of a large cyst in the left molar region and the lack of several tooth. CT scan evidenced an aggressive lesion invading body and ascendant branch of the jaw [Figure 1],[Figure 2],[Figure 3]. After analyzing this data, anamnesis and physical examination were repeated. Patient referred a tooth extraction of tooth 38, 3 years ago because of the presence of a pericoronal cyst. The histopathological analysis of the cyst tissue revealed an inflammatory cyst. However, no patient follow-up was carried out. Bearing this in mind, we carried out an incisional biopsy of the lesion. The result showed a poorly differentiated PIOSCC. After due consideration of all data, we staged cancer as cT3N0M0. In the oncological committee of our hospital, we decided to treat the case with a wide surgical resection, neck dissection and postoperative radiotherapy. Postoperative examination confirmed the diagnosis of PIOSCC poorly differentiated [Figure 4] with resection margins free. The patient has no clinical signs of recurrence 27 months after surgery.
Figure 1: Three-dimensional computed tomography image showing an intraosseous odontogenic squamous cell carcinoma of the mandible. At the physical examination, the patient presented a bone consistency with swelling of vestibular region in the left side of the jaw

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Figure 2: Axial computed tomography image of mandibular intraosseous odontogenic squamous cell carcinoma. The lesion affects the body and the ascendant branch of the mandible in the left side

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Figure 3: Orthopantomogram of mandibular intraosseous odontogenic squamous cell carcinoma

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Figure 4: Histopathological image of mandibular intraosseous odontogenic squamous cell carcinoma with H&E stain

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   Discussion Top


The incidence of PIOSCC is rare. Only approximately 200 cases have been reported to date. The clinical behavior is variable. However, the majority (50%) are well-grade malignant tumors. The median age of presentation is between the sixth and eight decade of life and it occurs more often in males.[5]

According to the literature, the time diagnosis is often delayed due to the absence of symptoms provoked by pathology. In this line, a review of 30 cases evidenced that PIOSCC showed the following forms of presentation: asymptomatic (26,7%), asymptomatic mass (26.7%), mass (16.7%), pain (13.3%), nerve involvement (10%) and expansion (6.7%). Moreover, PIOSCC might be confused with other inflammatory pathologies.[9] In our case, a patient referred a tooth extraction and cystectomy 3 years before the diagnosis. The pathological examination of cyst evidenced an inflammatory cyst. Unfortunately, any follow-up was performed. Thus, it is possible that the tumor could have been confused with a pericoronal cyst.

Panoramic X-ray and CT scan are essentials for a proper diagnosis and analysis of the tumor extension. In addition, an incisional biopsy is needed to complete the diagnosis.

A number of other lesions should be considered in the differential diagnosis such as alveolar carcinomas, jaw metastases, odontogenic tumors and maxillary sinus tumors.[6]

Wide surgical resection is currently the gold standard for treating PIOSCC. Moreover, several reports suggest that postoperative radiotherapy could be helpful to improve the overall survival. The role of postoperative chemotherapy is questionable. Survival rate of 3 years is described to be 37.8%.[10]

We treat the case with surgical resection, neck dissection and postoperative radiotherapy. Considering the wide surgical margins and lack of evidence about the effectiveness of chemotherapy, we decided to treat the patient only with surgery and postoperative radiotherapy. No signs of recurrence or metastases were evidenced 27 months after surgery. Despite this a close follow-up is mandatory.

Resuming, this case underlines the importance of routine follow-up of patients presenting large inflammatory cyst. Although rarely, this lesions might experience a neoplastic transformation. This practice allows early diagnosis. This is essential for increasing the overall survival of these patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
González-García R, Sastre-Pérez J, Nam-Cha SH, Muñoz-Guerra MF, Rodríguez-Campo FJ, Naval-Gías L. Primary intraosseous carcinomas of the jaws arising within an odontogenic cyst, ameloblastoma, and de novo: Report of new cases with reconstruction considerations. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:e29-33.  Back to cited text no. 1
    
2.
Bodner L, Manor E, Shear M, van der Waal I. Primary intraosseous squamous cell carcinoma arising in an odontogenic cyst: A clinicopathologic analysis of 116 reported cases. J Oral Pathol Med 2011;40:733-8.  Back to cited text no. 2
    
3.
Eversole LR. Malignant epithelial odontogenic tumors. Semin Diagn Pathol 1999;16:317-24.  Back to cited text no. 3
    
4.
Eversole LR, Siar CH, van der Waal I. Primary intraosseous squamouscell carcinomas. In: Barnes L, Evson JW, Reichart P, Sidransky D, eds. World Health Organization classification of tumors. Pathology and genetics head and necktumors. World Health Organization International Agency for Researchon Cancer. Lyon: IACR Press, cell carcinoma arising in a dentigerous cyst in a 16-month-old girl. Otolaryngol Head Neck Surg. 2002;127:463-4.  Back to cited text no. 4
    
5.
Spinelli G, Arcuri F, Rocchetta D, Agostini T. Primary intraosseous squamous cell carcinoma of the mandible. Is adjuvant therapy still necessary to improve a 5-year survival rate? J Craniofac Surg 2014;25:1137-9.  Back to cited text no. 5
    
6.
Charles M, Barr T, Leong I, Ngan BY, Forte V, Sándor GK. Primary intraosseous malignancy originating in an odontogenic cyst in a young child. J Oral Maxillofac Surg 2008;66:813-9.  Back to cited text no. 6
    
7.
Scheer M, Koch AM, Drebber U, Kübler AC. Primary intraosseous carcinoma of the jaws arising from an odontogenic cyst – A case report. J Craniomaxillofac Surg 2004;32:166-9.  Back to cited text no. 7
    
8.
Yasuoka T, Yonemoto K, Kato Y, Tatematsu N. Squamous cell carcinoma arising in a dentigerous cyst. J Oral Maxillofac Surg 2000;58:900-5.  Back to cited text no. 8
    
9.
Baker RD, D'Onofrio ED, Corio RL, Crawford BE, Terry BC. Squamous-cell carcinoma arising in a lateral periodontal cyst. Oral Surg Oral Med Oral Pathol 1979;47:495-9.  Back to cited text no. 9
    
10.
Thomas G, Pandey M, Mathew A, Abraham EK, 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.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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