Radiation induced osteogenic sarcoma of the maxilla
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Radiation induced sarcoma arise as a long term complication of radiation treatment for other benign or malignant conditions. They are of very rare occurrence in jaw bones and are even rarer in maxilla.
Here we report a case of radiation induced sarcoma in a patient treated for squamous cell carcinoma of buccal mucosa with radiation who developed osteosarcoma of maxillary bone after six years. The patient was treated successfully with surgery.
What should be the best treatment of radiation induced sarcoma is still debatable; however, surgery offers the best chance of cure. Role of reradiation and adjuvant chemotherapy needs to be further evaluated.
KeywordsOsteosarcoma Osteogenesis Imperfecta Buccal Mucosa Fibrous Dysplasia Fibrous Histiocytoma
Osteosarcoma of the head and neck are rare tumours and constitute <10% of all osteosarcomas [1, 2]. They involve mandible and maxilla with equal frequency . Although the pathogenesis is unknown various predisposing factors have been proposed. These include preexisting bone lesions like bone cysts , osteogenesis imperfecta , osteochondroma , fibrous dysplasia , trauma, genetic factors, virus , and previous radiation . Radiation induced sarcomas (RIS) are defined as tumours that develop after a latent period after radiation, with in the field of radiation, and have histological confirmation of a sarcoma . Osteosarcoma is the commonest RIS in bone, however in head and neck area malignant fibrous histiocytoma is more common . Post radiation osteogenic sarcoma of the facial bones have been reported in the bones with preexisting benign diseases such as fibrous dysplasia or Paget's disease. However only few cases have been reported in normal maxilla [3, 12, 13]. We report here a case of maxillary osteosarcoma occurring 6 years after treatment for squamous cell carcinoma of the buccal mucosa.
Osteogenic sarcoma is the primary malignant tumour of the bone with predilection for long bones. Its occurrence in jaw bones is rare [1, 2]. The average latent period between radiation treatment and development of sarcoma has been reported to be 4 to 30 years with average of 12.5 years . The radiation dose varied from 25 to 110 Gy, with a median of 45 Gy . Only few cases have been reported in a normal maxilla after radiation for benign or malignant disease of adjourning sites [3, 11, 12, 13]. The exact mechanism of radiation induced sarcomas is not clear, these may occur after ortho (low energy) or mega voltage (high energy) radiation, however, with ortho voltage the dosage is lower and period is longer . Development is also influenced by other known and yet unknown factors. It is suggested that the patients who harbor the mutation in tumor suppressor genes like p53 and ratinoblatoma gene (Rb) are more prone to develop these tumors. Furthermore children appear to be more susceptible than adults .
The treatment of osteogenic sarcoma of jaw irrespective of etiology includes radical surgery along with adjuvant chemotherapy and radiotherapy [3, 17]. Survival after surgery alone is low however addition of adjuvant treatment show 8 year metastasis free survival rate of 60 to 70% [14, 18] The factors associated with poorer prognosis include neural sensory alteration as a presenting symptom, increasing age of patients and surgical margins less than 5 mm [3, 14].
What should be the ideal treatment for post radiation sarcoma is still debatable. Surgery appears to offer the best chance of cure. However as most of the osteosarcoma metastasize by hematogenous route, and hence there is a rational for addition of adjuvant chemotherapy [3, 19]. Some authors recommend neoadjuvant chemotherapy before a definitive surgery is undertaken .
The ideal treatment of radiation induced osteosarcoma still eludes surgeons. Surgery offers the best chances of cure provided a negative margin is achieved. Adjuvant chemotherapy should be offered to all cases as the hematogenous spread can occur. Some authors suggest use of neoadjuvant chemotherapy as this can help in achieving negative surgical margins.
Patients consent was obtained for publication of this case
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