|Year : 2018 | Volume
| Issue : 3 | Page : 283-288
Gnathic osteosarcoma: An analysis of a single-institutional experience
Adebayo Aremu Ibikunle1, Abdurrazaq Olanrewaju Taiwo1, Ramat Oyebunmi Braimah1, Kabir Abdullahi2, Michael Adeyemi3, Malami A Usman4
1 Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Histopathology, Faculty of Basic Medical Sciences, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
3 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Lagos University Teaching Hospital, Lagos, Nigeria
4 Department of Radiotherapy and Oncology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
|Date of Web Publication||25-Sep-2018|
Dr. Adebayo Aremu Ibikunle
Department of Oral and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Source of Support: None, Conflict of Interest: None
INTRODUCTION: Osteosarcoma is an aggressive primary bone malignancy characterized by osteoid formation. It typically affects long bones, rarely affecting the jaws. OS of the jaws accounts has an estimated incidence of 0.7 per million. Reports of gnathic osteosarcoma from sub-Saharan Africa are few and to the best of our knowledge there is none from Northwestern Nigeria. This study aims to present our experience with gnathic osteosarcoma in our center.
MATERIALS AND METHODS: A review of the records of patients managed on account of histologically diagnosed gnathic osteosarcoma at the Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria, between March, 2010 and March, 2017 was done. Data were recorded and analyzed using IBM SPSS Statistics for Windows version 20 (Armonk, NY, USA: IBM Corp.).
RESULTS: A total of 431 Oral and Maxillofacial lesions were biopsied during the study period, out of which 17 (3.9%) were histologically diagnosed as osteosarcoma. Majority of the patients presented with a chief complaint of jaw swelling 9 (52.9%). The most frequently encountered histological type was the osteoblastic type 9 (52.9%). High grade tumours constituted 10 (58.8%). The patients were followed up for varying periods and recurrences were observed in 3 cases. Five (29.4%) mortalities were recorded.
CONCLUSION: Despite the rarity of gnathic osteosarcomas, they still pose a daunting challenge in Oral and Maxillofacial practice. Patients often present with complaints of facial swelling, bleeding or pain. Late presentation is common in our environment and multi-modal treatment plan is often preferable.
Keywords: Maxillofacial, orofacial, osteosarcoma
|How to cite this article:|
Ibikunle AA, Taiwo AO, Braimah RO, Abdullahi K, Adeyemi M, Usman MA. Gnathic osteosarcoma: An analysis of a single-institutional experience. Indian J Health Sci Biomed Res 2018;11:283-8
|How to cite this URL:|
Ibikunle AA, Taiwo AO, Braimah RO, Abdullahi K, Adeyemi M, Usman MA. Gnathic osteosarcoma: An analysis of a single-institutional experience. Indian J Health Sci Biomed Res [serial online] 2018 [cited 2018 Dec 15];11:283-8. Available from: http://www.ijournalhs.org/text.asp?2018/11/3/283/242045
| Introduction|| |
Osteosarcoma (OS) or osteogenic sarcoma is an aggressive primary bone malignancy which is characterized by osteoid formation.,, Bone malignancies are infrequent, consisting of a variety of tumors, the most common of which is OS.,, OS typically affects the long bones, especially areas of growth such as the metaphysic; however, it rarely affects the jaws. OS of the jaws, also known as gnathic OS, accounts for 6%–9% of all OSs and has an estimated incidence of 0.7 per million., Gnathic OS has a distinct biological behavior from that of long bones.,, It tends to occur a decade or two later than its long bone counterpart which exhibits a bimodal distribution, occurring among patients in their second decade of life and the elderly., Patients present more often with a chief complaint of bony swelling rather than pain; it has a lower rate of metastasis and is said to have higher survival rates.
Reports of gnathic OS from sub-Saharan Africa are few and, to the best of our knowledge, there is none from North-West Nigeria. This study aims to present our experience with gnathic OS at our center.
| Materials and Methods|| |
A review of the record of patients managed on account of histologically diagnosed gnathic osteogenic sarcoma at the Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria, between March 2010 and March 2017, was done. Data retrieved included age, gender, socioeconomic status, symptoms/signs, site, and mode of treatment. Staging of the disease was done in accordance to the 2006 American Joint Committee on Cancer (AJCC) staging system.
Data were recorded and analyzed using IBM SPSS Statistics for Windows version 20 (IBM Corp., Armonk, NY, USA) and the results were presented with descriptive statistics.
| Results|| |
A total of 431 oral and maxillofacial lesions were biopsied during the study period, of which 17 (3.9%) cases were histologically diagnosed as OS. There were 9 (52.9%) males and 8 (47.1%) females, giving a male/female ratio of 1:0.9. The age ranged from 10 to 59 years with a mean (±standard deviation) of 31.4 (±16.1) years [Table 1]. Majority of the patients presented with a chief complaint of jaw swelling (9 [52.9%]), while others had varying complaints at presentation [Figure 1] and [Figure 2]. On examination, tooth mobility was invariably present in all cases, while mucosal ulceration was present in 3 (17.7%) cases.
The duration of symptoms before presentation ranged from 3 to 11 months with a mean (±SD) of 6.53 (±2.18) months [Figure 3]. On palpation, regional lymph node enlargement was observed in 9 (52.9%) patients, although chest radiographs did not reveal the presence of any chest metastasis. Majority of the patients were in Stage IVb (9 [52.9%]), while others were in Stages IIb (4 [23.5%]) and IIa (4 [23.5%]). The mandible was the site of occurrence in 12 (70.6%) cases, while the maxilla was the site of occurrence in 5 (29.4%) cases. Majority of the mandibular lesions were located in the mandibular body/symphysis (8 [66.7%]), while others were situated in the mandibular angle/ramus region. All the maxillary lesions were situated in the premolar/molar region.
The most frequently encountered histological type was the osteoblastic OS (9 [52.9%]), while the telangiectatic type was the least frequently seen type (1 [5.9%]) [Figure 4] and [Figure 5]. High-grade tumors constituted 10 (58.8%), while low-grade tumors represented 7 (41.2%) cases. Twelve of the cases (70.6%) underwent surgical jaw resection with or without immediate reconstruction under general anesthesia, while the other patients were referred for neoadjuvant chemotherapy and/or concurrent chemo-radiotherapy [Figure 6]. All but one of the patients referred for neoadjuvant chemotherapy died during the course of chemotherapy/chemoradiotherapy. The patients have been followed up for varying periods, during which recurrences were observed in three cases, nine patients remained clinically free of disease or were lost to follow-up and five mortalities were recorded.
|Figure 4: The distribution of the histological types of osteosarcoma observed|
Click here to view
|Figure 5: A cellular lesion disposed in sheets with osteoid [Figure 1]a, chondroid [Figure 1]b, fibroblastic [Figure 1]c, and telangiectatic [Figure 1]d areas (H and E ×100)|
Click here to view
|Figure 6: The proportions of treatment options utilized for the patients|
Click here to view
| Discussion|| |
Gnathic OS was not commonly encountered in this study, representing 3.9% of all oral and maxillofacial lesion biopsies done over a period of 7 years. However, the prevalence observed is significantly higher than the report of Ogunlewe et al. who stated that osteogenic sarcomas of the jaws constituted 0.6% of all oral and maxillofacial biopsies done over a 10-year period. The reason for this observation is unclear. Certain bone diseases such as Paget's disease, chronic osteomyelitis, and fibrous dysplasia have been associated with the development of OS, none of which was diagnosed in this series., It is estimated that <1% of OSs develop from the underlying Paget's disease of bone. Previous irradiation has also been linked to the initiation of osteogenic sarcoma. However, none of the patients had undergone any form of radiotherapy before presentation, thus ruling out cases of radiation-induced gnathic OSs. Radiotherapy-induced OS is said to account for about 5.5% of all OSs.,,
A slight male preponderance was noted in this study, which is comparable to some reports in the literature. Conversely, it is at variance with the report of Lee et al. who found a slight female dominance. Interestingly, Clark et al. reported a significant male preponderance, having 42 males and 24 females in their study.
The age characteristics of patients seen in this series are in keeping with several studies in the literature.,, Wang et al. reported an age range and mean age of 9–80 years and 37.8 years, respectively. Jaw swelling was one of the presenting complaints or the sole complaint among all the patients. Indeed, some of our patients presented with large jaw tumors. This may be related to the often painless nature of gnathic OSs, making patients less likely to present at the hospital.,, Padilla and Murrah opined that the tumors often begin as symptomless masses, which eventually become painful in the absence of intervention. Tooth mobility was universally seen among all the patients in this series. This is expected because of the aggressive nature of the tumor and its penchant for causing widening of the periodontal membrane space.,, Indeed, Garrington's sign (widening of the periodontal membrane space) is a common early sign of gnathic osteogenic sarcoma. Similarly, abnormal tooth mobility may be the first sign of the disease.
This was not always the case in this series, considering that all the patients presented in Stage III or IV of the disease and pain was not a universal symptom. This is despite the fact that the mean duration of symptoms before presentation in this series is lower than that observed in their report. This may be related to the observation that majority of the patients in this study presented with high-grade tumors, which are expected to be more aggressive and thus infiltrate neural tissue causing pain earlier than their counterparts. Mucosal ulcerations were noted in a number of cases. These ulcerations may be secondary to trauma from the opposing teeth which were found to often impinge on the mucosa overlying the tumors owing to the large sizes of these tumors.
Bleeding was also a common presenting complaint in this study. This may also be associated with the preponderance of high-grade lesions among the patients. Late presentation was frequently observed among the patients. Several studies have stated varying reasons for late presentations among patients in our environment, ranging from the commonly practiced out-of-pocket payment system occasioned by financial incapacitation to psychosocial factors such as beliefs and culture.,,
Majority of the patients in this series were in the late stages of the disease at presentation, despite the seemingly short duration of the disease before presentation, which was <12 months in all cases. This is probably an attestation to the aggressiveness of this disease. Staging of OSs is often based on the Musculo-Skeletal Tumor Society, the AJCC, or Enneking staging systems rather than the well-known TNM staging system., This is because of the limited applicability of the TNM staging to OSs, owing to the rarity of metastasis to lymph nodes in these tumors. The AJCC system of staging OSs has gained prominence over the years., The advanced disease stages seen among the cases in this study are largely due to the large tumor sizes and regional nodal involvement. Physical palpation was the mode of determination of nodal involvement in this study. Several modes of regional or distant node involvement have been described in the literature, including, physical palpation, ultrasonography (USS), computed tomography (CT) scan, and magnetic resonance imaging.,, In a comparative study by Shetty et al., physical palpation, USS, and CT scan were said to have an accuracy of 72.43, 76.92, and 76.28, respectively. Regional nodal involvement was seen in a significant number of patients. This is contrary to the widely acclaimed views that OS does not characteristically spread to the lymph nodes.
The mandible was the major site of occurrence in this study. This is in tandem with several reports in the literature., However, it is in contrast to the study by Clark et al. who reported a slight maxillary predominance. Majority of the tumors affecting the mandible were seen in the mandibular body/symphysis. This is in consonance with the study by Ogunlewe et al., but contrasts with the report by Yildiz et al. who stated that most of the mandibular tumors were located around the 8th molar, where active mandibular growth occurs., OS is said to have a predilection for areas of bone growth.,, The modal site of occurrence of mandibular OS in this study is not unexpected, since mandibular growth centers are multiple., While the mandibular condylar cartilage is still widely believed to be the major mandibular growth center, the advent of the functional matrix theory has brought new perspectives.,
The osteoblastic variant of OS was the most commonly encountered subtype. This is in agreement with several reports in the literature., However, it in dissonance with the reports by Ogunlewe et al. and Bennett et al. who reported the chondroblastic type to be the most commonly seen in their series., The telangiectatic subtype was the least frequently diagnosed variant in this study. Controversies on the rarest subtypes exist, with the pendulum swinging between the fibroblastic and telangiectatic variants.,,
Majority of the lesions were high-grade tumors. This is in consonance with the report of Arslan et al., in which 89.6% of the cases reviewed were high-grade OSs. However, their study consisted mainly of long bone lesions. Few studies state that the grade of gnathic OS exists in the literature; nonetheless, Padilla and Murrah in a review of seven cases of gnathic OSs stated that 85.7% of the cases were of low grade.
Multimodal treatment with varying treatment combination was often done in this series. In addition, adjuvant radiotherapy of 50 Gy was administered following surgery. Those who presented with unresectable tumors either had neoadjuvant chemotherapy to shrink the tumor or concurrent chemoradiotherapy alone. Although surgery aimed at obtaining tumor-free margins, remains the mainstay of treatment, especially when the patient presents early, and the tumor is accessible, multimodal treatment is gaining popularity. Multimodal treatment is said to improve the disease-free rates from a paltry 10%–20% to as high as 60%. Neoadjuvant chemotherapy was utilized in patients with large tumors which were deemed to be potentially amenable to surgery. Neoadjuvant chemotherapy is useful in achieving downstaging of tumors, making it especially useful in patients with large tumors that were encountered in this study.,, Some authors have also alluded to the lessening effect of neoadjuvant chemotherapy on intraoperative blood loss. The significant number of mortalities following chemotherapy may be because most of the patients sent for neoadjuvant chemotherapy were in advanced stages of the disease, thus the prognosis was poor, with or without the neoadjuvant chemotherapy. In addition, some of the deaths may be attributable to progressive disease, since majority of the patients presented late.
Due to the complex anatomy of the maxillofacial region and the presence of vital structures, especially in the maxillary region, obtaining free margins particularly in patients with large tumors is difficult. Utilization of intraoperative pathological adjuncts such as frozen section has been propounded by some researchers.
The mortality rate recorded in this study was 29.4%. OS of the jaws is reported to exhibit better survival rates than that of the long bones. Several prognostic factors have been proposed for OSs, including the grade of tumor, stage at presentation, loss of heterozygosity of the retinoblastoma gene, and patients' age.,, Some researchers have stated that the most consistent predictor of outcome is the extent of histologic necrosis subsequent to inductive chemotherapy.,,
| Conclusion|| |
Gnathic OSs continue to pose a challenge to oral and maxillofacial surgeons. Late presentation and its penchant for recurrence constitute a formidable cause of poor quality of life and mortality among the patients. Multimodal treatment is often beneficial in the treatment of the management of these cases; despite this, mortality is still high.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Augustine D, Ramesh M, Murali S, Sekar B, Paul G. Osteosarcoma of mandible: A rare report with literature review. World J Dent 2014;5:190-2.
Kalburge JV, Sahuji SK, Kalburge V, Kini Y. Osteosarcoma of mandible. J Clin Diagn Res 2012;6:1597-9.
Mardinger O, Givol N, Talmi YP, Taicher S. Osteosarcoma of the jaw. The Chaim Sheba medical center experience. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:445-51.
Lee RJ, Arshi A, Schwartz HC, Christensen RE. Characteristics and prognostic factors of osteosarcoma of the jaws: A retrospective cohort study. JAMA Otolaryngol Head Neck Surg 2015;141:470-7.
George A, Mani V. Gnathic osteosarcomas: Review of literature and report of two cases in maxilla. J Oral Maxillofac Pathol 2011;15:138-43. [Full text]
Ottaviani G, Jaffe N. The epidemiology of osteosarcoma. In: Jaffe N., Bruland O., Bielack S (Eds). Pediatric and Adolescent Osteosarcoma. Springer, Boston, MA. Cancer Treat Res 2009;152:3-13.
Wang S, Shi H, Yu Q. Osteosarcoma of the jaws: Demographic and CT imaging features. Dentomaxillofac Radiol 2012;41:37-42.
Chakravarthi PS, Kattimani VS, Prasad LK, Satish PR. Juxtacortical osteosarcoma of the mandible: Challenges in diagnosis and management. Natl J Maxillofac Surg 2015;6:127-31.
] [Full text]
Ogunlewe MO, Ajayi OF, Adeyemo WL, Ladeinde AL, James O. Osteogenic sarcoma of the jaw bones: A single institution experience over a 21-year period. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:76-81.
Hansen MF, Seton M, Merchant A. Osteosarcoma in Paget's disease of bone. J Bone Miner Res 2006;21 Suppl 2:P58-63.
Liao LQ, Yan HH, Mai JH, Liu WW, Li H, Guo ZM, et al.
Radiation-induced osteosarcoma of the maxilla and mandible after radiotherapy for nasopharyngeal carcinoma. Chin J Cancer 2016;35:89.
Hong N, Yoo H, Shin SH, Gwak HS, Lee SH. A case of radiation-induced osteosarcoma after the treatment of pineoblastoma. Brain Tumor Res Treat 2015;3:156-9.
Salvati M, Ciappetta P, Capoccia G, Capone R, Raco A. Osteosarcoma of the skull. Report of a post-Paget and post-radiation case in an elderly woman. Neurosurg Rev 1994;17:73-6.
Nthumba PM. Osteosarcoma of the jaws: A review of literature and a case report on synchronous multicentric osteosarcomas. World J Surg Oncol 2012;10:240.
Clark JL, Unni KK, Dahlin DC, Devine KD. Osteosarcoma of the jaw. Cancer 1983;51:2311-6.
Garrington GE, Scofield HH, Cornyn J, Hooker SP. Osteosarcoma of the jaws. Analysis of 56 cases. Cancer 1967;20:377-91.
Adebayo ET, Ajike SO, Adebola A. Maxillofacial sarcomas in Nigeria. Ann Afr Med2005;4:25-30.
Soares RC, Soares AF, Souza LB, Santos AL, Pinto LP. Osteosarcoma of mandible initially resembling lesion of dental periapex: A case report. Braz J Otorhinolaryngol 2005;71:242-5.
Sarkar R. Pathological and clinical features of primary osseous tumours of the jaw. J Bone Oncol 2014;3:90-5.
Sinha R, Roy Chowdhury SK, Chattopadhyay PK, Rajkumar K. Low-grade osteosarcoma of the mandible. J Maxillofac Oral Surg 2010;9:186-90.
Padilla RJ, Murrah VA. The spectrum of gnathic osteosarcoma: Caveats for the clinician and the pathologist. Head Neck Pathol 2011;5:92-9.
Samraj L, Kaliamoorthy S, Venkatapathy R, Oza N. Osteosarcoma of the mandible: A case report with an early radiographic manifestation. Imaging Sci Dent 2014;44:85-8.
Almeida E, Mascarenhas BA, Cerqueira A, Medrado AR. Chondroblastic osteosarcoma. J Oral Maxillofac Pathol 2014;18:464-8.
] [Full text]
Kumar VS, Barwar N, Khan SA. Surface osteosarcomas: Diagnosis, treatment and outcome. Indian J Orthop 2014;48:255-61.
] [Full text]
Ibikunle AA, Taiwo AO, Gbotolorun OM, Braimah RO. Challenges in the management of cervicofacial necrotizing fasciitis in Sokoto, Northwest Nigeria. J Clin Sci2016;13:143. [Full text]
Adisa AO, Arowolo OA, Akinkuolie AA, Titiloye NA, Alatise OI, Lawal OO, et al.
Metastatic breast cancer in a Nigerian tertiary hospital. Afr Health Sci 2011;11:279-84.
Oji C. Late presentation of orofacial tumours. J Craniomaxillofac Surg 1999;27:94-9.
Chaudhary M, Chaudhary SD. Osteosarcoma of jaws. J Oral Maxillofac Pathol 2012;16:233-8. [Full text]
Picci P. Osteosarcoma (osteogenic sarcoma). Orphanet J Rare Dis 2007;2:6.
Shetty D, Jayade BV, Joshi SK, Gopalkrishnan K. Accuracy of palpation, ultrasonography, and computed tomography in the evaluation of metastatic cervical lymph nodes in head and neck cancer. Indian J Dent 2015;6:121-4.
] [Full text]
Zaiton F, Shehata SM, Warda MH, Alekrashy MA. Diagnostic value of MRI for predicting axillary lymph nodes metastasis in newly diagnosed breast cancer patients: Diffusion-weighted MRI. Egypt J Radiol Nucl Med2016;47:659-67.
Salman R, MH, Adil SO. Diagnostic Accuracy of Multislice CT Scan in the Detection of Occult Cervical Lymph Node Metastasis in Head and Neck Cancers. Journal of the College of Physicians and Surgeons Pakistan 2017;27:01.
Yildiz FR, Avci A, Dereci O, Erol B, Celasun B, Gunhan O, et al.
Gnathic osteosarcomas, experience of four institutions from Turkey. Int J Clin Exp Pathol 2014;7:2800-8.
Mizoguchi I, Toriya N, Nakao Y. Growth of the mandible and biological characteristics of the mandibular condylar cartilage. Jpn Dent Sci Rev2013;49:139-50.
Mota RS, Cardoso VA, Bechara CD, Reis JG, Maciel SM. Analysis of mandibular dimensions growth at different fetal ages. Dent Press J Orthod 2010;15:113-21.
Prabhu S, Jose M, Iyengar S. Gnathic osteosarcoma: A retrospective analysis over a 20 year period. Kathmandu Univ Med J (KUMJ) 2013;11:37-40.
Bennett JH, Thomas G, Evans AW, Speight PM. Osteosarcoma of the jaws: A 30-year retrospective review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:323-32.
Liu JJ, Liu S, Wang JG, Zhu W, Hua YQ, Sun W, et al.
Telangiectatic osteosarcoma: A review of literature. Onco Targets Ther 2013;6:593-602.
Weiss A, Khoury JD, Hoffer FA, Wu J, Billups CA, Heck RK, et al.
Telangiectatic osteosarcoma: The St. Jude children's research hospital's experience. Cancer 2007;109:1627-37.
Dengra S, Sharma N, Tekade S. Fibroblastic osteosarcoma in mandible: A rare case report and review of literature. J Pierre Fauchard Acad (India Section)2012;26:162-5.
Arslan HS, Aydin O, Dervışoğlu S, Oksüz DC, Kantarci F, Hiz M, et al.
Low-grade osteosarcoma, review of 15 cases in a series of 156 osteosarcoma cases. Turk Patoloji Derg 2011;27:138-43.
Ferrari D, Codecà C, Battisti N, Broggio F, Crepaldi F, Violati M, et al.
Multimodality treatment of osteosarcoma of the jaw: A single institution experience. Med Oncol 2014;31:171.
Schneider C, Kamaly-Asl I, Ramaswamy V, Lafay-Cousin L, Kulkarni AV, Rutka JT, et al.
Neoadjuvant chemotherapy reduces blood loss during the resection of pediatric choroid plexus carcinomas. J Neurosurg Pediatr 2015;16:126-33.
Tabrizi R, Garajei A, Shafie E, Jamshidi S. Outcome of neoadjuvant chemotherapy on local recurrence and distant metastasis of oral squamous cell carcinoma: A retrospective study. J Dent (Shiraz) 2016;17:207-12.
Vishak S, Rangarajan B, Kekatpure VD. Neoadjuvant chemotherapy in oral cancers: Selecting the right patients. Indian J Med Paediatr Oncol 2015;36:148-53.
] [Full text]
Jaafar H. Intra-operative frozen section consultation: Concepts, applications and limitations. Malays J Med Sci 2006;13:4-12.
Vadillo RM, Contreras SJ, Canales JO. Prognostic factors in patients with jaw sarcomas. Braz Oral Res 2011;25:421-6.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]