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Fibrosarcoma in the mandible—A case report
British Journal of Oral Surgery (rg75), 13, 78-81
FIBROSARCOMA IN THE MANDIBLE-A
CASE REPORT
Z. HAIDAR,D.D.S.(U. Dasmascus) Maxilla-Facial
and Plastic
Unit, Bangour General Hospital, West Lothian
Surgery
FIBROSARCOMA in the oral cavity is a rare lesion and few have been reported in the literature. Although the tumour is not encapsulated, in practice, it is very difiicult to assess infiltration until the tumour is quite large. Fibrosarcoma can arise wherever there is tissue of mesenchymal origin and in the bone, it can arise either from the periosteum (peripheral) or from the central bone (central). According to MacFarlane (I g72), there were 18 cases reported in the literature of fibrosarcoma affecting the jaws: 14 involved the mandible and four involving the maxilla. Males were affected in IO cases. In his survey, MacFarlane found metastases in two cases only. The sarcomas as a group differ from malignant epithelial neoplasms by their typical occurrence in relatively young persons and their greater tendency to metastasise through the blood stream as well as the lymphatics, thereby producing more widespread foci of secondary tnmour growth. Clinical Features. The tumour, when affecting the oral cavity, appears as an irregular, slightly red swelling; this may be pedunculated. Secondary infiltration, ulceration, haemorrhage and rapid growth are features. Radiographic Examination. The radiographic changes in the affected bone are not diagnostic of fibrosarcoma. The lesion may appear as an irregular and There is no clear line of demarcation diffuse radiolucency of patchy appearance. from the normal surrounding bone. CASE REPORT History. The patient was a 66-year-old retired major, referred to Edinburgh Royal Infirmary in November 1972 complaining of pain and ulceration affecting the anterior part of his mandible. He had been involved in a road traffic accident one year previously as a result of which his lower anterior teeth were broken, but he had not sought dental advice at that time. He had suffered pain in relation to these teeth for 3 weeks before attending his dental surgeon. The first unusual complaint was slight paraesthesia affecting the chin area which gradually worsened. His dental practitioner removed the remaining broken teeth but the sockets did not heal and subsequently he developed a swelling in the anterior part of the mandible extending to the floor of the mouth. Examination. General-The patient appeared to be a healthy man; his medical history and general examination revealed no significant abnormality. Extra-ma&A slight swelling could be seen over the chin area. No other extra-oral abnormalities were to be seen. No regional lymph nodes could be palpated. Intra-oral-The patient was edentulous. A firm ulcerated swelling was present in the anterior part of the mandible which extended from the right to the left canine Received
17.7.74.
78
Accepted
8.11.74
FIBROSARCOMA
IN THE MANDIBLE
79
FIG. I The swelling which was present in the anterior part of the mandible. FIG. 2 Occlusal radiograph of the mandible showing the lesion. regions
(Fig. I). The buccal expansion was greater than the lingual one. The oral mucosa in the rest of the mouth was normal. A swelling was present in the anterior part of the sublingual region.
Investigations. The radiographs showed a poorly demarcated lesion involving the body of the mandible and the alveolar ridge in the area of the symphysis (Fig. 2). Patchy radiolucent areas were present, the appearance of which was suggestive of an osteomyelitic lesion. Chest X-ray-The lung fields were clear and the cardiac size was normal. 2.2 per cent M.C.V., E.S.R. and W.B.C. within normal Huematology-Reticulocytes limits. A provisional diagnosis of osteomyelitis was made and the patient was put on antibiotics. The lesion showed no response to this therapy after 2 weeks and a biopsy from the area was therefore taken.
Histology. Under general anaesthesia, a biopsy was carried out from the affected area. The histological sections showed a whorled mass of highly cellular tissue with spindle-shaped nuclei showing marked pleomorphism and hyperchromatism in places. The appearances were those of fibrosarcoma which could be arising either within bone or from the soft tissue (Fig. 3). The patient was referred to the radiotherapy department in January Treatment. 1973 and was referred back having completed a course of 4 M.C.V. X-ray therapy to the anterior part of his lower jaw. He subsequently developed a marked erythema in the skin covering his chin. He was then discharged from the ward but kept under observation. In April 1973 another biopsy was taken as the swelling had not become smaller and this showed a mass of vital fibrosarcoma. Surgical intervention was then decided upon. In May 1973 the tumour was excised en bloc. The summary of the operation was as follows: (I) Tracheostomy. (2) Resection of the mandible to the angle on both sides with resection en bloc of fibrosarcoma of the middle portion of the mandible and the floor of the mouth 13/1--F
80
A, Photomicrograph B, Photomicrograph
BRITISH
JOURNAL OF ORAL SURGERY
FIG. 3 showing the whorled cellular pattern of the tumour. (H. & E. showing pleomorphic fibroblast nuclei with mitoses. (H. & E.
x 120.)
x 480.)
FIBROSARCOMA
IN THE MANDIBLE
81
and with it, the involved hyoid muscles, the contents of both submandibular triangles and an overlying ellipse of skin. (3) A Kirchner wire bar space maintainer was fixed to the mandibular stumps. The patient was fed post-operatively by a naso-gastric tube. He made remarkable progress but later developed a defect in the floor of his mouth when the wound failed to heal properly. He was provided with an obturator. In August 1973 a chest X-ray was taken and this showed radiopacities suggestive of metastases to both lungs. His general condition started to deteriorate after that. The patient lost 6 stones in weight over a period of 4 months. In November 1973 his pain was so severe that the only effective analgesic was Morphine. Further X-rays showed massive radiopaque foci affecting the lungs. The patient died in January rg74> 14 months after his first biopsy.
DISCUSSION The early diagnosis of fibrosarcoma may be very diflicult on clinical and radiological grounds alone. The pathologist also may find it difficult on occasions to give a certain diagnosis. Radiographically, the early bony lesion may be very similar to and should be differentiated from an osteomyelitic lesion. ESR and WBC values are sometimes higher than normal. Any inflammatory and ulcerated lesion which does not respond promptly to normal antibiotic therapy should be biopsied without further delay. Sarcomata in the facial region do not metastasise very often according to MacFarlane’s survey. The case presented was unusual in its rapid lung involvement. Although the lesions in the lungs in this particular case were not biopsied there can be little doubt that they were metastases to the region. As far as treatment is concerned, the results of radiotherapy are unpredictable and there may be a need for repeated biopsy during the healing phase. Surgery is the other choice, but usually has to be mutilating. The patient described underwent extensive mutilating surgery. The absence of metastases at the time of operating is not a prognostic guide. Such cases present a dilemma for the surgeon and the radiotherapist. ACKNOWLEDGEMENTS I would like to thank Dr J. F. Gould and Mr A. 3. DufJ for allowing me to have access to the case notes, and also to Mr A. Hunter of the Oral Pathology Department, Edinburgh Dental Hospital, for the photographs.
REFERENCE MACFARLANE,
W. I. (1972).
British Journal of Oral Surgery, IO, 168.
FIBROSARCOMA IN THE MANDIBLE-A
CASE REPORT
Z. HAIDAR,D.D.S.(U. Dasmascus) Maxilla-Facial
and Plastic
Unit, Bangour General Hospital, West Lothian
Surgery
FIBROSARCOMA in the oral cavity is a rare lesion and few have been reported in the literature. Although the tumour is not encapsulated, in practice, it is very difiicult to assess infiltration until the tumour is quite large. Fibrosarcoma can arise wherever there is tissue of mesenchymal origin and in the bone, it can arise either from the periosteum (peripheral) or from the central bone (central). According to MacFarlane (I g72), there were 18 cases reported in the literature of fibrosarcoma affecting the jaws: 14 involved the mandible and four involving the maxilla. Males were affected in IO cases. In his survey, MacFarlane found metastases in two cases only. The sarcomas as a group differ from malignant epithelial neoplasms by their typical occurrence in relatively young persons and their greater tendency to metastasise through the blood stream as well as the lymphatics, thereby producing more widespread foci of secondary tnmour growth. Clinical Features. The tumour, when affecting the oral cavity, appears as an irregular, slightly red swelling; this may be pedunculated. Secondary infiltration, ulceration, haemorrhage and rapid growth are features. Radiographic Examination. The radiographic changes in the affected bone are not diagnostic of fibrosarcoma. The lesion may appear as an irregular and There is no clear line of demarcation diffuse radiolucency of patchy appearance. from the normal surrounding bone. CASE REPORT History. The patient was a 66-year-old retired major, referred to Edinburgh Royal Infirmary in November 1972 complaining of pain and ulceration affecting the anterior part of his mandible. He had been involved in a road traffic accident one year previously as a result of which his lower anterior teeth were broken, but he had not sought dental advice at that time. He had suffered pain in relation to these teeth for 3 weeks before attending his dental surgeon. The first unusual complaint was slight paraesthesia affecting the chin area which gradually worsened. His dental practitioner removed the remaining broken teeth but the sockets did not heal and subsequently he developed a swelling in the anterior part of the mandible extending to the floor of the mouth. Examination. General-The patient appeared to be a healthy man; his medical history and general examination revealed no significant abnormality. Extra-ma&A slight swelling could be seen over the chin area. No other extra-oral abnormalities were to be seen. No regional lymph nodes could be palpated. Intra-oral-The patient was edentulous. A firm ulcerated swelling was present in the anterior part of the mandible which extended from the right to the left canine Received
17.7.74.
78
Accepted
8.11.74
FIBROSARCOMA
IN THE MANDIBLE
79
FIG. I The swelling which was present in the anterior part of the mandible. FIG. 2 Occlusal radiograph of the mandible showing the lesion. regions
(Fig. I). The buccal expansion was greater than the lingual one. The oral mucosa in the rest of the mouth was normal. A swelling was present in the anterior part of the sublingual region.
Investigations. The radiographs showed a poorly demarcated lesion involving the body of the mandible and the alveolar ridge in the area of the symphysis (Fig. 2). Patchy radiolucent areas were present, the appearance of which was suggestive of an osteomyelitic lesion. Chest X-ray-The lung fields were clear and the cardiac size was normal. 2.2 per cent M.C.V., E.S.R. and W.B.C. within normal Huematology-Reticulocytes limits. A provisional diagnosis of osteomyelitis was made and the patient was put on antibiotics. The lesion showed no response to this therapy after 2 weeks and a biopsy from the area was therefore taken.
Histology. Under general anaesthesia, a biopsy was carried out from the affected area. The histological sections showed a whorled mass of highly cellular tissue with spindle-shaped nuclei showing marked pleomorphism and hyperchromatism in places. The appearances were those of fibrosarcoma which could be arising either within bone or from the soft tissue (Fig. 3). The patient was referred to the radiotherapy department in January Treatment. 1973 and was referred back having completed a course of 4 M.C.V. X-ray therapy to the anterior part of his lower jaw. He subsequently developed a marked erythema in the skin covering his chin. He was then discharged from the ward but kept under observation. In April 1973 another biopsy was taken as the swelling had not become smaller and this showed a mass of vital fibrosarcoma. Surgical intervention was then decided upon. In May 1973 the tumour was excised en bloc. The summary of the operation was as follows: (I) Tracheostomy. (2) Resection of the mandible to the angle on both sides with resection en bloc of fibrosarcoma of the middle portion of the mandible and the floor of the mouth 13/1--F
80
A, Photomicrograph B, Photomicrograph
BRITISH
JOURNAL OF ORAL SURGERY
FIG. 3 showing the whorled cellular pattern of the tumour. (H. & E. showing pleomorphic fibroblast nuclei with mitoses. (H. & E.
x 120.)
x 480.)
FIBROSARCOMA
IN THE MANDIBLE
81
and with it, the involved hyoid muscles, the contents of both submandibular triangles and an overlying ellipse of skin. (3) A Kirchner wire bar space maintainer was fixed to the mandibular stumps. The patient was fed post-operatively by a naso-gastric tube. He made remarkable progress but later developed a defect in the floor of his mouth when the wound failed to heal properly. He was provided with an obturator. In August 1973 a chest X-ray was taken and this showed radiopacities suggestive of metastases to both lungs. His general condition started to deteriorate after that. The patient lost 6 stones in weight over a period of 4 months. In November 1973 his pain was so severe that the only effective analgesic was Morphine. Further X-rays showed massive radiopaque foci affecting the lungs. The patient died in January rg74> 14 months after his first biopsy.
DISCUSSION The early diagnosis of fibrosarcoma may be very diflicult on clinical and radiological grounds alone. The pathologist also may find it difficult on occasions to give a certain diagnosis. Radiographically, the early bony lesion may be very similar to and should be differentiated from an osteomyelitic lesion. ESR and WBC values are sometimes higher than normal. Any inflammatory and ulcerated lesion which does not respond promptly to normal antibiotic therapy should be biopsied without further delay. Sarcomata in the facial region do not metastasise very often according to MacFarlane’s survey. The case presented was unusual in its rapid lung involvement. Although the lesions in the lungs in this particular case were not biopsied there can be little doubt that they were metastases to the region. As far as treatment is concerned, the results of radiotherapy are unpredictable and there may be a need for repeated biopsy during the healing phase. Surgery is the other choice, but usually has to be mutilating. The patient described underwent extensive mutilating surgery. The absence of metastases at the time of operating is not a prognostic guide. Such cases present a dilemma for the surgeon and the radiotherapist. ACKNOWLEDGEMENTS I would like to thank Dr J. F. Gould and Mr A. 3. DufJ for allowing me to have access to the case notes, and also to Mr A. Hunter of the Oral Pathology Department, Edinburgh Dental Hospital, for the photographs.
REFERENCE MACFARLANE,
W. I. (1972).
British Journal of Oral Surgery, IO, 168.