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Table of Contents
CASE REPORT
Year : 2013  |  Volume : 7  |  Issue : 2  |  Page : 83-85

Osteosarcoma of the maxilla: A case report and review of literature


1 Department of Surgical Oncology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Radiation Oncology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
3 Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Web Publication23-May-2014

Correspondence Address:
Ashish Singhal
Department of Surgical Oncology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Vibhuti Khand, Gomtinagar, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0331-3131.133103

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   Abstract 

Osteosarcoma (OS) is the most common primary bone tumor of the jaw. It is rare, and the biological behavior differs from other skeletal bone tumors. OS of jaw is characterized by lower incidence of metastasis and better prognosis than OS of long bones. Surgery with adjuvant radiotherapy is the mainstay of treatment. The complicated three-dimensional anatomy of the head and neck region makes it difficult to obtain wide resection margins, leading to higher chances of recurrence. Death is usually caused by the uncontrollable local spread. We present a case of 61-year-old man with diabetes mellitus, who presented with an upper jaw swelling, which was histologically confirmed as OS of the maxilla. He underwent surgery and adjuvant radiation for the same. 1 year later, patient developed recurrence at the base of the skull which was inoperable. He received palliative chemotherapy for the same and is now under follow-up.

Keywords: Maxilla, osteosarcoma, radiation, surgery


How to cite this article:
Singhal A, Hadi R, Mahajan C, Mehrotra KP. Osteosarcoma of the maxilla: A case report and review of literature. Ann Nigerian Med 2013;7:83-5

How to cite this URL:
Singhal A, Hadi R, Mahajan C, Mehrotra KP. Osteosarcoma of the maxilla: A case report and review of literature. Ann Nigerian Med [serial online] 2013 [cited 2020 Jan 20];7:83-5. Available from: http://www.anmjournal.com/text.asp?2013/7/2/83/133103


   Introduction Top


Osteosarcoma of jaw (OSJ) represents about 4% of the osteosarcomas (OS) and has a mild male predilection. [1] At the time of diagnosis, the patients are approximately 2 decades older than those with extra-gnathic OS, who have a peak incidence between the ages of 10 and 14 years. [2] OSJ is usually Grade II or III on histology, and associated with a better prognosis than conventional OS. [3] One should suspect OS besides inflammation and osteoblastoma when pain and swelling occurs on the affected site with radiological evidence of a cemento-osseous lesion. Early diagnosis and adequate surgical resection are the keys to better survival. [4] OSJ tends to spread locally within nerves and foramen in the mandible and thin cortices and foramen facilitate penetration and local spread. Biopsy, extraction of teeth and curettage can also cause extra-osseous spread and metastasis. Thus, disease in maxillofacial complex spreads to involve sinuses, nasal cavity and infratemporal fossa. Lymph nodes are rarely involved in this tumor. [5]


   Case Report Top


This is a case of 61-year-old male, chronic smoker and diabetic, who came with progressively increasing swelling over left nostril and cheek with intermittent bleeding from nostril for about 7 months [Figure 1]. He had difficulty in breathing with stuffy nose on the left side. Contrast-enhanced computed tomography face revealed contrast enhanced soft tissue lesion with multiple areas of new bone formation in the maxilla extending medially to involve whole of left nasal cavity with destruction of the nasal septum. The lesion extended into the orbit causing displacement of lateral and inferior rectus muscle and laterally to the infratemporal fossa. There was thinning of left side of cribriform plate with no obvious intracranial extension [Figure 2]. The biopsy from the lesion showed tissue with stratified squamous lining, dispersed in intersecting fascicles as well as sheet of spindle cells with intervening foci of osteoid and chondroid formation consistent with OS. Immunohistochemistry was positive for vimentin, osteonectin and smooth muscle actin, and negative for cytokeratin, desmin, S-100, and glial fibrillary acidic protein supporting the diagnosis of OS. The patient underwent left radical maxillectomy with left orbital exenteration with ethmoidectomy with sphenoid mucosal removal and temporalis flap cover with placement of obturator in the defect [Figure 3]. Intraoperatively, there was a large growth involving the lateral wall of left nose with extension into the maxillary sinus. The extra-ocular muscles were involved with destruction of medial and inferior wall of the orbit. Mucosa of anterior wall of sphenoid was free from tumor on intraoperative frozen section. Histomorphology was consistent with osteogenic sarcoma with negative margins. Postoperative period was uneventful [Figure 4]. After stitch removal, patient developed gaping of the wound in the temporal region which was closed subsequently with a rotation flap. Patient received adjuvant radiation therapy (external beam radiation therapy) 66 Gy/33# in 61/2 weeks at 5#/week. One year later, he developed recurrence at the skull base. The patient is now on metronomic chemotherapy (cyclophosphamide 250 mg BD) and symptomatic treatment.
Figure 1: Clinical photograph showing the growth involving the nasal cavity and maxilla left side

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Figure 2: Contrast-enhanced computed tomography face showing tumor involving the maxilla and nasal cavity with areas of calcification consistent with osteosarcoma

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Figure 3: Postoperative picture showing the residual defect

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Figure 4: The photomicrograph shows pleomorphic spindle cells with osteoid formation (H and E, ×50)

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   Clinical Discussion Top


Osteosarcomas are primary malignant bone tumors in which mesenchymal cells produce osteoid. [1] OSJ occurs over a wide age range, with a peak in the 4 th decade of life. The biological behavior of OS in the jaws differs from tumors of other skeletal bones. Head and neck OS have lower tendency of metastasis than their long bone counterparts, and have a better 5-year survival rate. [4] Regardless of the favorable biological behavior in relation to other skeletal bone OSs, patients with OSJ present with advanced tumors. Patients usually present with swelling and pain (due to superimposed infection) with signs of intra- and/or extra-oral swelling, loosening of teeth, proptosis, neurologic deficits, and trismus. Most cases present at advanced stage due to lack of pain as a prominent symptom. Radiologically, it presents as a radiolucent, radio-opaque or a heterogeneous lesion with poorly defined margins. Several diseases may be included in the differential diagnosis namely chondrosarcoma, Ewing's sarcoma, bone metastasis, fibrous dysplasia, osteomyelitis, and even lesions that do not usually affect the jaw bones such as fibrosarcoma, leiomyosarcoma, or rhabdomyosarcoma. [1] We, therefore, consider that swelling and/or pain in the absence of active odontogenic inflammatory diseases raise the possibility of an underlying OS. [6] An early finding in OSJ and in some chondrosarcomas is the symmetrical widening of the periodontal ligament space (WPLS). [7],[8] WPLS is considered a significant sign which aids the distinction of malignant bone diseases including OSJ from benign diseases, although WPLS is not always found in OSJ. Microscopically, the essential criterion of OS is direct production of osteoid or immature bone by tumor cells. Histologic variants such as osteoblastic, chondroblastic, fibroblastic and rarer subtypes such as epithelioid and telangiectatic have been described but they are not prognostically significant. It is recommended that conventional radiographs be requested on peripheral reactive lesions such as peripheral ossifying fibroma, pyogenic granuloma and epulis fissuratum and adequate biopsy of the lesions suspected to be OS should be taken; and final diagnosis be rendered essentially after correlation of radiographic and pathologic findings. Immunohistochemistry may be of help in excluding other malignancies of the jaw like cytokeratin positivity in poorly differentiated carcinomas, desmin in rhabdomyosarcoma and S-100 in malignant peripheral nerve sheath tumors. Primary treatment consists of aggressive surgical resection with adjuvant radiation. [1] Recurrent disease should be treated by surgery, radiation or chemotherapy. Neck dissection is only necessary if regional lymph nodes are positive. The use of chemotherapy in head and neck OS has been questioned unlike long bone OSs. [2] In addition, adjuvant radiotherapy has no positive effect on the survival, but it decreases the chances of local recurrence. [2],[4] Thus, radiotherapy can be used in association with surgical resection or alone as a palliative treatment in advanced cases with chemotherapy. Median survival of patients with OS of maxilla is 3 years with local recurrence and progression as the major cause of mortality. The current therapy of choice is total surgical removal of the lesion with safe margins. Radiation has an adjunct role and chemotherapy is largely palliative.


   Conclusion Top


OS of the maxilla presents a better prognosis than OS of the long bones. Patients with OS can exhibit advanced tumors, mainly when early diagnosis is not performed. Clinicians and pathologist should be aware of its characteristics and main differential diagnosis to avoid late recognition and improve survival. Surgery should be planned to achieve wider margins keeping in view the complex three-dimensional anatomy of the head and neck region. Since it is an uncommon tumor, large prospective trial on systemic treatment is not possible. Therefore, small retrospective studies and case reports are an opportunity to report and discuss issues of such clinical and diagnostic significance and improve our understanding of this tumor.

 
   References Top

1.Amaral MB, Buchholz I, Freire-Maia B, Reher P, de Souza PE, Marigo Hde A, et al. Advanced osteosarcoma of the maxilla: A case report. Med Oral Patol Oral Cir Bucal 2008;13:E492-5.  Back to cited text no. 1
    
2.Frei C, Bornstein MM, Stauffer E, Lizuka T, Buser D. Osteosarcoma of the maxilla and the maxillary sinus: a case report. Quintessence Int 2004;35:228-33.  Back to cited text no. 2
    
3.Unni KK, Dahlin DC. Grading of bone tumors. Semin Diagn Pathol 1984;1:165-72.  Back to cited text no. 3
    
4.Laskar S, Basu A, Muckaden MA, D'Cruz A, Pai S, Jambhekar N, et al. Osteosarcoma of the head and neck region: Lessons learned from a single-institution experience of 50 patients. Head Neck 2008;30:1020-6.  Back to cited text no. 4
    
5.Arora P, Rehman F, Girish KL, Kalra M. Osteosarcoma of mandible: Detailed radiographic assessment of a case. Contemp Clin Dent 2013;4:382-5.  Back to cited text no. 5
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6.Sciubba JJ, Fantasia JE, Kahn LB. Tumours and cysts of the jaws. Atlas of Tumor Pathology. 3 rd series, Fascicle 29. Washington, DC: American Registry of Pathology; 2001.  Back to cited text no. 6
    
7.Nakayama E, Sugiura K, Ishibashi H, Oobu K, Kobayashi I, Yoshiura K. The clinical and diagnostic imaging findings of osteosarcoma of the jaw. Dentomaxillofac Radiol 2005;34:182-8.  Back to cited text no. 7
    
8.Givol N, Buchner A, Taicher S, Kaffe I. Radiological features of osteogenic sarcoma of the jaws. A comparative study of different radiographic modalities. Dentomaxillofac Radiol 1998;27:313-20.  Back to cited text no. 8
    


    Figures

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



 

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