- Email: [email protected]
Osteosarcoma of facial bones
Int. J. Oral 511rg. 1983: 12: IQ6-Hl9 (Key words: osteosarcoma; maiignancy; maxilla: mandible; surgery, oral)
Osteosarcoma of facial bones K. KOTESHWER RAO, KAMALA G. PILLAI, B. H. SRIPATHI RAO AND R. G. NAYAK Departments oj Radiotherapy and Oncology, Dental Radiology & Oral Diagnosis, Oral and Maxillo-facial Surgery and Pathology, Kasturba Medical College Hospital. Manipal, Karnataka, India
ABSTRACT - Osteosarcoma, involving mandible and zygoma in 3 patients is presented and discussed; their clinical, radiological and treatment results are detailed. As this disease is uncommon in these bones, the difficulty in the management merits attention.
(Received Jor publication 19 April, accepted 8 October 1982)
Osteosarcoma is the most commonly seen malignant neoplasm of the bone 2 , 12 . It spreads mainly by the blood stream, resulting in pulmonary metastasis in 80% of the patients within 1 to 2 years, and in 75% of them, there will be lung secondaries at the time of first presentation. Fortunately less than 10% of these tumours occur in jaw bones, more commonly in the mandible4 •S , IO . 11 . 1•• It is primarily a tumour of the younger age group, with a slight prediliction for rnalesv". The osteosarcoma of the jaw grows rapidly but remains locally invasive for some time before metastasising--v. Despite the possible early diagnosis, better histological differentiation and improved management of this disease, the survival rates are not satisfactory. The observed 5-year survival rate is around 10-30%708.11.15. During the period 1975 to 1981, only 3 patients were seen with osteosarcoma out of 138 cases of bone tumor involving the head and neck region. Hence, we report these 3 cases of osteosarcoma for their clinical interest and difficulty in management.
Case reports Case 1. A 45-year-old female patient presented in
September 1977 with a gradually increasing swelling over the left lowerjaw of 6 months duration. This had been preceeded by extraction of painful left lower posterior teeth. There was no history of fever or preexistent benign lesions. There was diffuse enlargement of the left mandible, and the skin over the lesion was normal. Intraorally, an ulcerated large mass, covering the left lower alveolus in the premolar and molar regions was seen. Roentgenologically, an extensive radiolucent area was seen in the mandible with a pathological fracture (Fig. 1). Chest X-ray was normal. Other relevant investigations were within normal limits. An incisional biopsy of the lesion with the presence of osteoid bordered by plump hyperchromatic cells and increased mitosis and gaint cells, confirmed the diagnosisof osteosarcoma. Left partial mandiblectomy and suprahyoid block dissectionwith a sufficient soft tissue clearance was performed. The patient was kept on chemotherapy using vincristine and methotrexate combination at monthly intervals for 14 months, 18 months later, she developed a recurrence in the left maxilla for which partial resection of the maxilla was performed and chemotherapy restarted. 6 months later, she once again developed secondaries in the left temporal bone (Fig. 2) with raised intracranial tension (suspected brain secondaries). She expired a few days later.
OSTEOSARCOMA
107
Fig. 2. X-Ray photograph of the skull of ease I, showing an osteolyticlesion of the left temporal bone (metastasis).
Fig. 1. Osteosarcoma of the left mandible (osteolytic)
showing irregular destruction of the cortex with pathological fracture.
Case 2. A 45-year-old male patient consulted us for loss of sensation over the left half of his face, and a swelling of the left mandible of 4 months duration. There was no history of trauma or fever.There was a 6 x 6 em hard, fixed and well-defined mass on the left mandible with normal skin cover. There was left VII nerve (UMN) palsy and tongue deviation to left side, without trismus. A roentgenogram of the chest was normal. X-ray of the left mandible was suggestive of osteosarcoma (Fig. 3). Other investigations were within normal limits except for increased serum alkaline phosphatase. An open biopsy of the lesion confirmed the diagnosis of osteosarcoma. A wide excision of the tumour, in the form of left heminandiblectorny and suprahyoid clearance, was carried out. Becauseof unsatisfactory surgical excision, postoperative external radiotherapy was planned, and delivered 6000 cGy in 30 fractions over 6 weeks,using wedge fields. The patient was also kept on postoperative chemotherapy using vincristine and methotrexate combination given at monthly intervals. The patient has so far received6 courses of chemotherapy, and on regular follow-up there is no evidence to suggest progression of disease. Case 3. A 42-year-old male was seen by us for a painful swelling over the left zygoma of 3 months duration. There was no history of fever or trauma. The swelling was fixed to the left zygoma and was 5 x 3 em in size with ill-defined borders. The skin over
the lesion wasnormal. X-rays of theparanasal sinuses and zygoma were suggestiveof osteosarcoma (Fig. 4). Chest X-ray and other blood investigations were normal. A confirmatory open biopsy was performed. Since the lesion was found to be inoperable, external radiotherapy 6000 cGy in 30 fractions over 6 weeks, was given. The patient was also given chemotherapy using vincristine and methotrexate combination during and after radiotherapy at monthly intervals. So far 6 courses have been completed with a definite growth-restraining effect.
Discussion All the 3 patients in this report are in their 40s in contrast to the report ofDARAMOLA et a1. 4 • It is also observed that none of the 3 patients had pre-existent benign lesions such as Paget's disease, fibrous dysplasia or history of trauma, as observed by GARlNGTON et al.· and DARAMOLA et al.', The primary objective of treatment of osteosarcoma is to control the primary tumour and to prolong the survival period. Local tumour ablation can be achieved either by surgery or high-dose megavoltage radiotherapy. The addition of chemotherapy to these two modalities in varied combinations has improved the outlook of disease-free survival rate':":". DE FRIES et al? felt that wide excision
108
RAG, PILLA!, RAO AND NAY AK
Fig. 3. X-ray photograph of the left mandible with
typical radiating spicules of new bone (sunburst appearance) suggesting osteosarcoma.
Fig . 4. Osteoblastic osteosarcoma arising from left zygoma bone. Diffuse sclerosis may be noted along the extension into soft tissues.
ofthe growth is not feasible in the head and neck reg ion and suggested pre-operative radiotherapy followed by surgery and chemotherapy. Encouraging results have also been obtained by giving radiotherapy either prior to or after surgery by TuoWAy I 6 , GOMEZ et al", BOYER et al.' and Roy ESTER et al. 13 . The osteolytic osteosarcoma seen in our first case was rapidly growing and bulky. The metastatic spread was to rare sites like the maxilla, temporal bone and brain, and was free from lung disease. In spite of our intensive therapy, case 1 survived only for 3 years. The remaining 2 cases had the rapidlygrowing osteoblastic type oflesion. In one case, surgical excision was incomplete; the other case was found to be inoperable. Both cases received external radiotherapy and are now on chemotherapy. The non-availability of citrovorum factor for use in high-dose methotrexate treatment, and the very high cost of other drugs restricted us to use of a combination of vincristine 2 mg i.v. and methotrexate 50 mg i.v. as an adjuvant to either surgery or radiotherapy. Though it is rather early to comment on the efficacy of radiotherapy and chemotherapy
in these 2 cases , as they have completed only a 6 months follow-up period, the early results are encouraging. Acknowledgements - We are thankful to Professor V.
Balasundaram, Consultant Radiation Oncologist, Professor M. N. Nayak, Consultant Surgeon and Professor C. B. Rao, Consultant Surgeonof Oral and Maxillo-facial Surgery, for their expert help and advice.We also wishto thank Mrs. Pushpa Bhandary for her secretarial assistance.
References 1. ALLEN, C. V. & STEVENS, K. R.: Preoperative irradiation for osteogenicsarcoma. Cancer. 1973: 31 : )364-1366. 2. BENNETT, J. E., TIGNOR, S. P. & SCHAFER, W. G. : Osteogenic sarcoma of the facial bones. Am . J. Surg . 1968: 116: 538-541.
3. BOYER, C. W., BRICKNER, T. F. & WRAITEN, G. P. : The treatment of osteogenic sarcoma of the mandible. Am. J. Roentgenol. 1967: 99: 326-332 . 4. DARAMOLA, J. 0., AGHADIUNO, P. D., AJAGBE , H . A., OLUWASAMMI, J. 0. , OBISESAN, A. A. & LAGUNDOYE, S. B.: Osteogenic sarcoma of the jaws in Ibadan, Nigeria. Br. J. Oral Surg , 1976: 14 : 23-30.
OSTEOSARCOMA 5. DE FRIES, H. 0., PERLIN, E. & IEIDEL, S. A.: Treatment of osteogenic sarcoma of the mandible. Arch. Otolaryngol. 1978: 105: 358-359. 6. DOUGLASS, H. 0., JR., WANG, J., TAKITA, R., WALLACE, R. J., FRIEDMAN, M. & MINDELL, E.: Improvement in the results of treatment of osteogenic sarcoma. Surg. Gynaecol. Obstet, 1975: 140: 693-700.
7. FRIEDBERG, M. J., SERLIN, O. & TRAVAGLINE, E. A.: Osteosarcoma of the maxilla. Oral. Surg, 1962: 15: 883-891.
8. GARRINGTON, G. E., SCOFIElD, H. H., CORNYN, J. & HOOKER, S. P.: Osteosarcoma of the jaws; analysis of 56 cases. Cancer 1967: 20: 377-391. 9. GOMEZ, A. c., YOUMANS, R. D. & CHAMBERS, R. G.: Osteosarcoma of the mandible. Am. J. Surg, 1960: 100: 613-616. 10. JAGADEESAN, M., LAWRENCE, B. Y., SOMARAJAN, K. & SOLRHE, A. G.: Management of osteogenic sarcoma. Ind. J. Radiol. 1981: 35: 289-292. 11. KRAGH, L. Y., DAHLIN, D. C. & ERICH, J. B.: Osteosarcoma of the jaw and facial bones. Am. J. Surg. 1958: 96: 496-505. 12. POTDAR, G. G.: Osteogenic sarcoma of the jaws. Oral Surg. 1970: 30: 381.
109
13. ROYSTER, R. L., KING, E. R., EBERSOLE, J., DE GIORGI, L. S. & LEVITT, S. H.: High-dose preoperative supervoltage irradiation of osteogenic sarcoma. An. J. Roentgenol. Radium Ther. Nllc/. Med. 1972: 114: 536-543. 14. ROWE, N. H. & HUNGER FORD, R. W.: Osteosarcoma of the mandible. Oral. Surg, 1963: 21: 42-49. 15. SCHWARTZ, D. T. & ALPERT, M.: The clinical course of mandibular osteogenic sarcoma. Oral Surg. 1963: 16: 769-776.
16. TunWAY, R. C.: The place of external irradiation in the treatment of osteogenic sarcoma. J. Bone Joint. Surg, 1953: 35: 9-21.
Address: K. Koteshwer Rao Department of Radiotherapy & Oncology Kasturba Medical College Hospital Manipal-576 119, Karnataka State India
Osteosarcoma of facial bones K. KOTESHWER RAO, KAMALA G. PILLAI, B. H. SRIPATHI RAO AND R. G. NAYAK Departments oj Radiotherapy and Oncology, Dental Radiology & Oral Diagnosis, Oral and Maxillo-facial Surgery and Pathology, Kasturba Medical College Hospital. Manipal, Karnataka, India
ABSTRACT - Osteosarcoma, involving mandible and zygoma in 3 patients is presented and discussed; their clinical, radiological and treatment results are detailed. As this disease is uncommon in these bones, the difficulty in the management merits attention.
(Received Jor publication 19 April, accepted 8 October 1982)
Osteosarcoma is the most commonly seen malignant neoplasm of the bone 2 , 12 . It spreads mainly by the blood stream, resulting in pulmonary metastasis in 80% of the patients within 1 to 2 years, and in 75% of them, there will be lung secondaries at the time of first presentation. Fortunately less than 10% of these tumours occur in jaw bones, more commonly in the mandible4 •S , IO . 11 . 1•• It is primarily a tumour of the younger age group, with a slight prediliction for rnalesv". The osteosarcoma of the jaw grows rapidly but remains locally invasive for some time before metastasising--v. Despite the possible early diagnosis, better histological differentiation and improved management of this disease, the survival rates are not satisfactory. The observed 5-year survival rate is around 10-30%708.11.15. During the period 1975 to 1981, only 3 patients were seen with osteosarcoma out of 138 cases of bone tumor involving the head and neck region. Hence, we report these 3 cases of osteosarcoma for their clinical interest and difficulty in management.
Case reports Case 1. A 45-year-old female patient presented in
September 1977 with a gradually increasing swelling over the left lowerjaw of 6 months duration. This had been preceeded by extraction of painful left lower posterior teeth. There was no history of fever or preexistent benign lesions. There was diffuse enlargement of the left mandible, and the skin over the lesion was normal. Intraorally, an ulcerated large mass, covering the left lower alveolus in the premolar and molar regions was seen. Roentgenologically, an extensive radiolucent area was seen in the mandible with a pathological fracture (Fig. 1). Chest X-ray was normal. Other relevant investigations were within normal limits. An incisional biopsy of the lesion with the presence of osteoid bordered by plump hyperchromatic cells and increased mitosis and gaint cells, confirmed the diagnosisof osteosarcoma. Left partial mandiblectomy and suprahyoid block dissectionwith a sufficient soft tissue clearance was performed. The patient was kept on chemotherapy using vincristine and methotrexate combination at monthly intervals for 14 months, 18 months later, she developed a recurrence in the left maxilla for which partial resection of the maxilla was performed and chemotherapy restarted. 6 months later, she once again developed secondaries in the left temporal bone (Fig. 2) with raised intracranial tension (suspected brain secondaries). She expired a few days later.
OSTEOSARCOMA
107
Fig. 2. X-Ray photograph of the skull of ease I, showing an osteolyticlesion of the left temporal bone (metastasis).
Fig. 1. Osteosarcoma of the left mandible (osteolytic)
showing irregular destruction of the cortex with pathological fracture.
Case 2. A 45-year-old male patient consulted us for loss of sensation over the left half of his face, and a swelling of the left mandible of 4 months duration. There was no history of trauma or fever.There was a 6 x 6 em hard, fixed and well-defined mass on the left mandible with normal skin cover. There was left VII nerve (UMN) palsy and tongue deviation to left side, without trismus. A roentgenogram of the chest was normal. X-ray of the left mandible was suggestive of osteosarcoma (Fig. 3). Other investigations were within normal limits except for increased serum alkaline phosphatase. An open biopsy of the lesion confirmed the diagnosis of osteosarcoma. A wide excision of the tumour, in the form of left heminandiblectorny and suprahyoid clearance, was carried out. Becauseof unsatisfactory surgical excision, postoperative external radiotherapy was planned, and delivered 6000 cGy in 30 fractions over 6 weeks,using wedge fields. The patient was also kept on postoperative chemotherapy using vincristine and methotrexate combination given at monthly intervals. The patient has so far received6 courses of chemotherapy, and on regular follow-up there is no evidence to suggest progression of disease. Case 3. A 42-year-old male was seen by us for a painful swelling over the left zygoma of 3 months duration. There was no history of fever or trauma. The swelling was fixed to the left zygoma and was 5 x 3 em in size with ill-defined borders. The skin over
the lesion wasnormal. X-rays of theparanasal sinuses and zygoma were suggestiveof osteosarcoma (Fig. 4). Chest X-ray and other blood investigations were normal. A confirmatory open biopsy was performed. Since the lesion was found to be inoperable, external radiotherapy 6000 cGy in 30 fractions over 6 weeks, was given. The patient was also given chemotherapy using vincristine and methotrexate combination during and after radiotherapy at monthly intervals. So far 6 courses have been completed with a definite growth-restraining effect.
Discussion All the 3 patients in this report are in their 40s in contrast to the report ofDARAMOLA et a1. 4 • It is also observed that none of the 3 patients had pre-existent benign lesions such as Paget's disease, fibrous dysplasia or history of trauma, as observed by GARlNGTON et al.· and DARAMOLA et al.', The primary objective of treatment of osteosarcoma is to control the primary tumour and to prolong the survival period. Local tumour ablation can be achieved either by surgery or high-dose megavoltage radiotherapy. The addition of chemotherapy to these two modalities in varied combinations has improved the outlook of disease-free survival rate':":". DE FRIES et al? felt that wide excision
108
RAG, PILLA!, RAO AND NAY AK
Fig. 3. X-ray photograph of the left mandible with
typical radiating spicules of new bone (sunburst appearance) suggesting osteosarcoma.
Fig . 4. Osteoblastic osteosarcoma arising from left zygoma bone. Diffuse sclerosis may be noted along the extension into soft tissues.
ofthe growth is not feasible in the head and neck reg ion and suggested pre-operative radiotherapy followed by surgery and chemotherapy. Encouraging results have also been obtained by giving radiotherapy either prior to or after surgery by TuoWAy I 6 , GOMEZ et al", BOYER et al.' and Roy ESTER et al. 13 . The osteolytic osteosarcoma seen in our first case was rapidly growing and bulky. The metastatic spread was to rare sites like the maxilla, temporal bone and brain, and was free from lung disease. In spite of our intensive therapy, case 1 survived only for 3 years. The remaining 2 cases had the rapidlygrowing osteoblastic type oflesion. In one case, surgical excision was incomplete; the other case was found to be inoperable. Both cases received external radiotherapy and are now on chemotherapy. The non-availability of citrovorum factor for use in high-dose methotrexate treatment, and the very high cost of other drugs restricted us to use of a combination of vincristine 2 mg i.v. and methotrexate 50 mg i.v. as an adjuvant to either surgery or radiotherapy. Though it is rather early to comment on the efficacy of radiotherapy and chemotherapy
in these 2 cases , as they have completed only a 6 months follow-up period, the early results are encouraging. Acknowledgements - We are thankful to Professor V.
Balasundaram, Consultant Radiation Oncologist, Professor M. N. Nayak, Consultant Surgeon and Professor C. B. Rao, Consultant Surgeonof Oral and Maxillo-facial Surgery, for their expert help and advice.We also wishto thank Mrs. Pushpa Bhandary for her secretarial assistance.
References 1. ALLEN, C. V. & STEVENS, K. R.: Preoperative irradiation for osteogenicsarcoma. Cancer. 1973: 31 : )364-1366. 2. BENNETT, J. E., TIGNOR, S. P. & SCHAFER, W. G. : Osteogenic sarcoma of the facial bones. Am . J. Surg . 1968: 116: 538-541.
3. BOYER, C. W., BRICKNER, T. F. & WRAITEN, G. P. : The treatment of osteogenic sarcoma of the mandible. Am. J. Roentgenol. 1967: 99: 326-332 . 4. DARAMOLA, J. 0., AGHADIUNO, P. D., AJAGBE , H . A., OLUWASAMMI, J. 0. , OBISESAN, A. A. & LAGUNDOYE, S. B.: Osteogenic sarcoma of the jaws in Ibadan, Nigeria. Br. J. Oral Surg , 1976: 14 : 23-30.
OSTEOSARCOMA 5. DE FRIES, H. 0., PERLIN, E. & IEIDEL, S. A.: Treatment of osteogenic sarcoma of the mandible. Arch. Otolaryngol. 1978: 105: 358-359. 6. DOUGLASS, H. 0., JR., WANG, J., TAKITA, R., WALLACE, R. J., FRIEDMAN, M. & MINDELL, E.: Improvement in the results of treatment of osteogenic sarcoma. Surg. Gynaecol. Obstet, 1975: 140: 693-700.
7. FRIEDBERG, M. J., SERLIN, O. & TRAVAGLINE, E. A.: Osteosarcoma of the maxilla. Oral. Surg, 1962: 15: 883-891.
8. GARRINGTON, G. E., SCOFIElD, H. H., CORNYN, J. & HOOKER, S. P.: Osteosarcoma of the jaws; analysis of 56 cases. Cancer 1967: 20: 377-391. 9. GOMEZ, A. c., YOUMANS, R. D. & CHAMBERS, R. G.: Osteosarcoma of the mandible. Am. J. Surg, 1960: 100: 613-616. 10. JAGADEESAN, M., LAWRENCE, B. Y., SOMARAJAN, K. & SOLRHE, A. G.: Management of osteogenic sarcoma. Ind. J. Radiol. 1981: 35: 289-292. 11. KRAGH, L. Y., DAHLIN, D. C. & ERICH, J. B.: Osteosarcoma of the jaw and facial bones. Am. J. Surg. 1958: 96: 496-505. 12. POTDAR, G. G.: Osteogenic sarcoma of the jaws. Oral Surg. 1970: 30: 381.
109
13. ROYSTER, R. L., KING, E. R., EBERSOLE, J., DE GIORGI, L. S. & LEVITT, S. H.: High-dose preoperative supervoltage irradiation of osteogenic sarcoma. An. J. Roentgenol. Radium Ther. Nllc/. Med. 1972: 114: 536-543. 14. ROWE, N. H. & HUNGER FORD, R. W.: Osteosarcoma of the mandible. Oral. Surg, 1963: 21: 42-49. 15. SCHWARTZ, D. T. & ALPERT, M.: The clinical course of mandibular osteogenic sarcoma. Oral Surg. 1963: 16: 769-776.
16. TunWAY, R. C.: The place of external irradiation in the treatment of osteogenic sarcoma. J. Bone Joint. Surg, 1953: 35: 9-21.
Address: K. Koteshwer Rao Department of Radiotherapy & Oncology Kasturba Medical College Hospital Manipal-576 119, Karnataka State India