case report J Bras Patol Med Lab. 2018 Apr; 54(2): 111-115.

Chondroblastic osteosarcoma of the mandible:

10.5935/1676-2444.20180021
case report
Osteossarcoma condroblástico em mandíbula: relato de caso

Loara Gabriela R. Oliveira1; John Lennon S. Cunha1; Bruno T. Bezerra1; Maria de Fátima B. Melo2; Juliana B. M. Fonte2; Ricardo Luiz C. Albuquerque Jr.1

1. Universidade Tiradentes, Sergipe, Brazil. 2. Universidade Federal de Sergipe, Sergipe, Brazil.

abstract
We report the case of a 32-year-old male patient, who was subjected to cone-beam tomography for planning the removal of an unerupted
tooth. Cone-beam CT scans revealed the presence of a mixed lesion with poorly defined limits in the left posterior mandibular region,
with “sun-ray” periosteal reaction. An incisional biopsy was performed and the diagnosis was chondroblastic osteosarcoma. In this paper,
a discussion of the differential diagnosis criteria and treatment of this unusual malignant neoplasm is performed. Thus, it is intended to
provide information that will help in the study of management protocols for the increasingly effective treatment of gnathic osteosarcomas.

Key words: head and neck neoplasms; bone neoplasms; osteosarcoma.

Introduction of unresectable local recurrences. The use of chemotherapy in OS

of the jaws has also been reported, since it may be an important
tool for the treatment of this neoplasm, but consensus is still to be
Osteosarcoma (OS) is the most common primary malignant
reached concerning the timing of its application, whether before
bone tumor, occurring more frequently in long bones and rarely
or after surgery(2, 12, 13).
in the maxillofacial region(1). It represents less than 1% of all
biopsies in the maxilla and mandible(2, 3). The most common Based on the above, this article aims to report a case of
signs and symptoms of OS are pain or paresthesia, rapid growth, chondroblastic osteosarcoma of the mandible, as well as to discuss
swelling and expansion of cortical bone, facial asymmetry, nasal the morphological criteria of differential diagnosis and factors
obstruction, displacement and mobility of associated teeth(4). involved in the prognosis of this lesion.

Radiographically, the tumors appear as a radiolucent,

radiopaque or mixed image, with irregular and poorly defined Case report
margins and periosteal osteophytic reaction in an spiculated pattern
perpendicular to the cortical bone (“sun ray” appearance)(5, 6).
A 32-year-old mulatto male patient attended the private
From a histopathological perspective, the OS is characterized by the
dental office for extraction of tooth 38 partially erupted. During
proliferation of ovoid, spindle-shaped cells or with stellar shaped
the intraoral clinical examination, no noticeable changes were
irregularly morphologies, with varying degrees of atypia, associated
observed. Cone-beam computed tomography (CT) was ordered
with direct osteoid matrix deposition, by tumor cells(7-10).
(Figure 1), and demonstrated, in a panoramic reconstruction, the
The treatment of choice for head and neck OS is aggressive presence of a mixed image with poorly defined limits, located in
surgical resection, with wide margin of safety, associated with the posterior mandible area, on the left side. Furthermore, partial
adjuvant chemo and radiotherapy(11). Radiotherapy should be resorption of the distal root of tooth 37 was observed. In coronal
reserved for the treatment of OS of the jaws, with the possibility of sections, it was possible to observe perforation of buccal and lingual
positive margins for tumor cells after surgical resections or in cases cortical plate and osteophytic reaction. In the axial sections, in

First submission on 12/11/17; last submission on 12/12/17; accepted for publication on 02/25/18; published on 04/20/18

111
 Chondroblastic osteosarcoma of the mandible: case report

turn, change was observed in the density of the trabecular bone, second evaluation with another professional. Four months after
which was hyperdense. In 3D reconstruction (Figure 2), the the incisional biopsy, the patient returned to the dental surgeon
loss of lingual and vestibular cortical continuity was confirmed. presenting, in the extraoral clinical examination, a noticeable
Therefore, the diagnostic hypotheses established by the dentist at increase in volume, affecting the middle and lower third of the left
the clinical and imaging examination were osteosarcoma and side of the face, with hard consistency on palpation (Figure 4). In
osteomyelitis. The incisional biopsy was performed and followed the intraoral examination, the patient had an increase in volume
by histopathological evaluation. with lining leukoerythroplastic mucosa, bleeding on touch, in the
left posterior mandibular region (Figure 5). Faced with the new
situation, the patient was immediately referred to the oncologist,
who established pre-surgical chemotherapy as a treatment, in
an attempt to reduce tumor size, and, later, mandibulectomy
associated with adjuvant radiotherapy.

A B

C D

Figure 1 − Cone-beam CT scan of the patient

A) panoramic reconstruction, showing a mixed lesion in the mandible posterior region,
with poorly defined limits, which caused partial reabsorption of the distal root of the tooth
37; B) coronal sections exhibiting periosteal osteophytic reaction with “sun ray” appearance
in the mandibular lingual cortical; C) axial section showing hyperdense mandibular
medullary bone, on the left side.
CT: computed tomography.
Figure 3 − Histological sections stained with HE
A) deposition of basophilic and eosinophilic material consistent with chondroid and
osteoid matrix, respectively; B) intense deposition of interspersed mineralized trabecular
tissue through a hypercellularized connective tissue; C) osteoid matrix and presence of
atypical osteoblasts with ovoid to fusiform morphology; D) irregular gaps within the
chondroid matrix (chondroplasts) containing cells predominantly compatible with atypical
chondroblasts.
HE: hematoxylin and eosin.

Figure 2 − Three-dimensional reconstruction of left posterior mandibular region,

showing loss of continuity of buccal (A) and lingual (B) cortical plates

Histological sections stained with hematoxylin and eosin (HE)

(Figure 3) revealed a proliferation of mesenchymal cells with
ovoid to fusiform morphology, exhibiting moderate cytological
atypia. The parenchymal component was associated with focal
deposition of trabecular eosinophilic matrix, interpreted as
osteoid, as well as abundant basophilic amorphous matrix,
forming gaps that contain pleomorphic ovoid cells, consistent with
neoplastic chondroid tissue. Both matrices suffered extensive areas
of mineralization, forming irregular trabeculae of tumor bone
tissue. The established diagnosis was chondroblastic osteosarcoma. Figure 4 − Frontal image of the patient, showing volume increase in the middle and
The patient refuted the diagnosis and reported that he needed a lower third of the face, left side, with no cardinal signs

112
 Loara Gabriela R. Oliveira; John Lennon S. Cunha; Bruno T. Bezerra; Maria de Fátima B. Melo; Juliana B. M. Fonte; Ricardo Luiz C. Albuquerque Jr.

From the imagining perspective, the symmetrical thickening

of the periodontal ligament space and the loss of continuity of the
lamina dura of involved teeth have been pointed out as one of
the earliest signs of development of this malignant neoplasm(16, 17).
Furthermore, Arora et al. (2013)(18) argue that, as observed in the
present case, the formation of periosteal reaction as bone spicules
radiating from the cortical bone, commonly referred to as “sun-ray”,
is a frequent feature in Gnathic OS, especially in mandibular
tumors. This latter characteristic can be better visualized in
occlusal radiographs or axial and coronal CT scans(9), which again
corroborates the findings described in this case report.

Figure 5 − Intraoral photography, showing volume increase with presence of

The histopathological aspects of this study led to the
leukoerythroplastic lining mucosa with ulcerated areas classification of the tumor as a chondroblastic variant of OS.
In fact, a number of studies have pointed to this histological
subtype as the most common form of OS in maxillary
bones(2, 8, 15, 19). However, large-scale production of the chondroid
About one year and a half after the initial diagnosis, the matrix can make stressful the distinction between chondroblastic
patient was diagnosed with brain metastasis and was referred to OS and chondrosarcoma(7). In the morphological perspective, the
a new cycle of chemotherapy. However, he died two months later identification of foci of osteoid matrix being deposited by tumor
due to complications secondary to chemotherapy, culminating in cells, as occurred in the present case, independently of forming or
multiple organ failure. not the bone trabeculae tumor by endochondral ossification of the
cartilage matrix, it is the main criterion to establish the diagnosis
of chondroblastic OS to the detriment of chondrosarcoma(20).
Discussion The identification of malignant osteoid matrix associated with the
integration of clinical and imaging data continues to be
OS is a primary malignant bone tumor characterized by the cornerstone for the diagnosis of osteosarcoma and, to date,
direct formation of immature bone and osteoid tissue by the no immunohistochemical marker has equaled the diagnostic
tumor cells. Although it affects mainly long bones, about 6%-7% value of this important morphological marker(21). Furthermore,
of these malignant tumors occur in the maxillofacial region(14). it is important to emphasize that the differential diagnosis
The OS of the jaws most frequently affects males, with a mean between these two entities does not constitute a purely academic
age of 27.2 ± 13.6 years, with no significant difference between exercise. In fact, chondrosarcomas are tumors that are resistant
maxillary (30.7 ± 15.8) and mandibular (23.4 ± 10.1) lesions, to radiotherapy, whereas osteosarcomas are radiosensitive,
affecting both gnathic bones equally(2). Very similar data were that makes the precise distinction between these two entities a
also reported by Azizi et al. (2009)(15). Since the reported case particularly important issue capable of greatly influencing the
refers to a 32-year-old male patient, it is possible to state that he treatment and the prognosis of the lesion(22).
is in agreement with the demographic profile presented in the The option for the radical surgical approach, represented by
literature on this tumor. mandibulectomy, adopted in this case, is in line with that suggested
In the present case, the patient was asymptomatic at the by the specialized literature for cases of mandibular OS(23-25).
time of diagnosis. This is in agreement with the study developed Chemotherapy and, in particular, postoperative radiotherapy are
by Ajura & Lau (2010)(8), in which 59 cases of gnathic OS were also complementary therapeutic procedures commonly used in
analyzed and observed that most cases (77%) did not report any the treatment of head and neck OS(26). The present case is also
in agreement with the literature, in view of the option for
type of symptomatology, while only 15% of tumors cases presented
radiotherapy after surgical removal of the tumor.
pain and 8% paresthesia. Moreover, these authors still mentioned
that in 26% of the cases the initial suspicion of the lesion was, as in Pre-surgical chemotherapy has been a procedure used for
the present case, based on eminently imaging findings. These data large tumors in an attempt to minimize tumor volume and provide
suggest that routine imaging examinations may play a key role in a less aggressive surgical approach(27). In view of this possibility,
the early diagnosis of central lesions of the jaws. this strategy was adopted in the present case. However, the tumor

113
 Chondroblastic osteosarcoma of the mandible: case report

was resistant to chemotherapy. Although it represents an unusual In the light of the above, case reports bringing information
outcome, resistance to pre-surgical chemotherapy is a phenomenon about diagnostic criteria, therapeutic response and clinical course
well-documented in the literature, probably due to the specific of OS are extremely relevant tools for the better knowledge of
selection of chemoresistant aggressive tumor clones(28). Accordingly, these tumors. Thus, it is possible to bring in subsidies that will
the possibility that such a biological phenomenon contributed to the contribute to the establishment of increasingly effective protocols
fatal outcome, in the present case, it can not be ruled out(2, 29). for OS treatment, which will reflect a better prognosis for patients.

resumo
Relatamos o caso de um paciente do sexo masculino, 32 anos de idade, que realizou tomografia cone-beam para planejamento
de remoção de dente incluso. O exame revelou presença de lesão mista de limites mal definidos na região posterior mandibular
esquerda, com reação periosteal em “raios de sol”. Foi realizada biópsia incisional, e o diagnóstico foi de osteossarcoma
condroblástico. Neste trabalho, será realizada uma discussão dos critérios de diagnóstico diferencial e do tratamento dessa neoplasia
maligna. Pretende-se, dessa forma, trazer informações que auxiliem no estudo de protocolos de conduta para o tratamento cada
vez mais efetivo dos osteossarcomas gnáticos.

Unitermos: neoplasias de cabeça e pescoço; neoplasias ósseas; osteossarcoma.

References 9. Soares RC, Soares AF, Souza LB, Santos ALV, Pinto LP. Osteossarcoma de
mandíbula inicialmente mimetizando lesão do periápice dental: relato
de caso. Rev Bras Otorrinolaringol. 2005; 71(2005): 242-5.
1. Valente R, Abreu TC, Real FH. Osteossarcoma em mandíbula – relato de
10. Chaudhary M, Chaudhary SD. Osteosarcoma of jaws. J Oral Maxillofac
caso. Rev Cir Traumatol Bucomaxilofac. 2011; 11(4): 37-42. ISSN 1808-
Pathol. 2012; 16(2): 233-8. doi: 10.4103/0973-029X.99075.
5210.
11. Ferrari D, Codecà C, Battisti N, et al. Multimodality treatment of
2. Ogunlewe MO, Ajayi OF, Adeyemo WL, Ladeinde AL, James O. Osteogenic
osteosarcoma of the jaw: a single institution experience. Med Oncol. 2014
sarcoma of the jaw bones: a single institution experience over a 21-year
Sep; 31(9): 171. Epub 2014 Aug 19.
period. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Jan;
101(1): 76-81. 12. Berner K, Hall KS, Monge OR, Weedon-Fekjær H, Zaikova O,
Bruland ØS. Prognostic factors and treatment results of high-grade
3. Martins GE, Perez SV. Acompanhamento do paciente tratado de
osteosarcoma in norway: a scope beyond the “classical” patient. Sarcoma.
osteossarcoma. Acta Ortop Bras. 2012; 20(4): 235-9.
2015; 2015: 516843. doi: 10.1155/2015/516843.
4. Lukschal LF, Barbosa RMLB, Alvarenga RL, Horta MCR. Osteossarcoma
13. Lee JA. Osteosarcoma in Korean children and adolescents. Korean J
em maxila: relato de caso. Rev Port Estomatol Med Dent Cir Maxilofac.
Pediatrics. 2015; 58(4): 123-8. doi:10.3345/kjp.2015.58.4.123.
2013; 54(1): 48-52. doi:10.1016/j.rpemd.2012.08.001.
5. Petrikowski CG, Pharoah MJ, Lee L, Grace MG. Radiographic 14. Picci P. Osteosarcoma (osteogenic sarcoma). Orphanet J Rare Dis.
differentiation of osteogenic sarcoma, osteomyelitis, and fibrous dysplasia 2007 Jan 23; 2: 6.
of the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995 Dec; 15. Azizi T, Motamedi M, Jafari SM. Gnathic osteosarcomas: a 10-year
80(6): 744-50. multi-center demographic study. Indian J Cancer. 2009; 46: 231-3.
6. Duong LM, Richardson LC. Descriptive epidemiology of malignant 16. Tabatabaei SH, Jahanshahi G, Dehghan Marvasti F. Diagnostic
primary osteosarcoma using population-based registries, United States, challenges of low-grade central osteosarcoma of jaw: a literature review.
1999-2008. J Registry Manag. 2013 Summer; 40(2): 59-64. J Dent (Shiraz). 2015 Jun; 16(2): 62-7.
7. Yildiz FR, Avci A, Dereci O, Erol B, Celasun B, Gunhan O. Gnathic 17. Samraj L, Kaliamoorthy S, Venkatapathy R, Oza N. Osteosarcoma of
osteosarcomas, experience of four institutions from Turkey. Int J Clin Exp the mandible: a case report with an early radiographic manifestation.
Pathol. 2014 May 15; 7(6): 2800-8. Imaging Sci Dent. 2014; 44(1): 85-8. doi: 10.5624/isd.2014.44.1.85.
8. Ajura AJ, Lau SH. A retrospective clinicopathological study of 59 18. Arora P, Rehman F, Girish KL, Kalra M. Osteosarcoma of mandible:
osteogenic sarcoma of jaw bone archived in a stomatology unit. Malays J detailed radiographic assessment of a case. Contemp Clin Dent. 2013 Jul;
Pathol. 2010; 32: 27-34. 4(3): 382-5. doi: 10.4103/0976-237X.118355.

114
 Loara Gabriela R. Oliveira; John Lennon S. Cunha; Bruno T. Bezerra; Maria de Fátima B. Melo; Juliana B. M. Fonte; Ricardo Luiz C. Albuquerque Jr.

19. Chittaranjan B, Tejasvi MA, Babu BB, Geetha P. Intramedullary 25. Amaral MB, Buchholz I, Freire-Maia B, et al. Advanced osteosarcoma
osteosarcoma of the mandible: a clinicoradiologic perspective. J Clin of the maxilla: a case report. Med Oral Patol Oral Cir Bucal. 2008 Aug 1;
Imaging Sci. 2014 Dec 31; 4(Suppl 2): 6. 13(8): E492-5.
20. Wadhwa N. Osteosarcoma: diagnostic dilemmas in histopathology 26. Pedruzzi PAG, Costa PB, Oliveira BV, Ramos GHA, Sassi LM, Orlandi D.
and prognostic factors. Indian J Orthop. 2014; 48(3): 247-54. Osteossarcoma da cabeça e pescoço. Rev Bras Cir Cabeça Pescoço. 2006;
doi:10.4103/0019-5413.132497. 35(4): 255-9.
21. Machado I, Navarro S, Picci P, Llombart-Bosch A. The utility of
27. Chen Y, Yang Y, Yuan Z, Wang C, Shi Y. Predicting chemosensitivity
SATB2 immunohistochemical expression in distinguishing between
in osteosarcoma prior to chemotherapy: An investigational study of
osteosarcomas and their malignant bone tumor mimickers, such as
biomarkers with immunohistochemistry. Oncol Lett. 2012 May; 3(5):
Ewing sarcomas and chondrosarcomas. Pathol Res Pract. 2016 Sep;
212(9): 811-6. 1011-6.
22. Chhabra S, Chopra R, Handa U, Punia RS, Mohan H. Cytomorphologic 28. Goorin AM, Schwartzentruber DJ, Devidas M, et al. Presurgical
features of chondroid neoplasms: a comparative study. Acta Cytol. 2010 chemotherapy compared with immediate surgery and adjuvant
Nov-Dec; 54(6): 1101-10. chemotherapy for nonmetastatic osteosarcoma: Pediatric Oncology
23. Kalburge JV, Sahuji SK, Kalburge V, Kini Y. Osteosarcoma of mandible. Group Study POG-8651. J Clin Oncol. 2003; 21(8): 1574-80. doi: 10.1200/
J Clin Diagn Res. 2012; 6(9): 1597-9. doi:10.7860/JCDR/2012/3922.2574. JCO.2003.08.165.
24. Nirmala S, Nuvvula S, Kumar K, Babu M, Chilamakuri S. 29. Granowski-Lecornu M, Chuang SK, Kaban LB, August M. Osteosarcoma
Osteosarcoma of mandible in a 10-year-old girl. J Indian Soc Pedod Prev of the jaws: factors influencing prognosis. J Oral Maxillofac Surg. 2011;
Dent. 2014; 32: 74-8. 69: 2368-75. doi: 10.1016/j.joms.2010.10.023.

Corresponding author

John Lennon Silva Cunha

Rua João Geniton da Costa, 501; Jabotiana; CEP: 49095-796; Aracaju-SE, Brasil; e-mail: lennonrrr@live.com.

This is an open-access article distributed under the terms of the Creative Commons Attribution License.

115