Recurrence of chondromyxoid fibroma: a case report B. Danielsen,M. Ritzau* and A. Wenzel Departments of Radiology and 'Oral and Maxillofacial Surgery, Royal Dental College, Aarhus, Denmark
Received 4 June 1990 and in final form 10 August 1990 A case of recurrence of a chondromyxoid fibroma 3 years after its surgical removal is presented. The significance of the clinical, radiographic and histological appearances is discussed. Keywords: Fibroma; mandible; recurrence
Case report A 22-year-old Caucasian woman complained to her dentist of a thumping pain in the lower right molar region when she jogged. On examination, the lower border of the right mandible was tender to palpation; intra-orally, there was swelling lingual to the first and second molars. Both teeth responded similarly to those on the left to electric pulp testing. An intra-oral radiograph revealed a radiolucency related to the apices of both teeth, suggesting that her symptoms were due to chronic periapical infection. However, when the amalgam restorations were removed, both teeth continued to give a positive response to the electric pulp tester. Sedative dressings were therefore inserted. One week later, the second molar failed to respond to the electric pulp tester as well as to a test cavity. However, some vitality was noted as the pulp chamber' was penetrated. Endodontic treatment was completed without complications. Six months later her symptoms remained unchanged and she was therefore referred to the Department of Oral and Maxillofacial Surgery, Royal Dental College, Aarhus, Denmark. On examination, there was a tender swelling at the lower border aOnd on the inner aspect of the body of the right mandible in the molar region. Radiographic examination (Figure 1) revealed a mixed density lesion, S x 3 cm in size, related to the apices of both lower right molars. The lesion contained irregular trabeculation. The margins were well defined with erosion and expansion of the lower border. On the basis of the clinical history and radiographic findings, the lesion was considered a benign tumour, probably an ossifying fibroma, but the patient refused further investigation. She returned 6 months later when there had been a slight increase in the swelling. A further radiograph revealed more marked trabeculation within the lesion and a tendency towards a multilocular appearance. In view of this evidence of increased activity, the tumour was removed with meticulous curettage and apicectomy of the molar teeth was performed and the roots were treated by retrograde root filling (Figure 2). The gross specimen was composed of white lobulated tissue with a few small cystic and haemorrhagic areas. Some calcification was observed. On histological examination, the tissue was heterogeneous in appearance: some
Figure I Part of the panoramic radiograph at the first visit. There is a well-defined, mixed density lesion, 5 x 3 em, related to the lower right first and second molars. The inferior cortical plate is both eroded and expanded
areas were dominated by fibroblasts and a relatively large number of osteoclasts, others by a myxoid matrix with a few star-shaped cells and yet others by regular basophilic cementoid/chondroid tissue, both with varying degrees of maturation (Figure 3). Blood vessels
Figure 2 Postoperative panoramic radiograph following excision of the lesion and retrograde root fillings and apicectomies on 46 and 47
Dentomaxillofac. Radio!., 1991, Vo!. 20, May 65
Recurrence of chondromyxoid fibroma: B. Danielsen et al.
'. I,
,
" , II.
I
..
.' .. ,
.~
."
(
Figure 5 Panoramic radiograph two years after the second operation. The reserved margins are well defined
Figure 3 Histopathologically, the tissue appears heterogeneous. There is fibrous tissue (F), myxoid tissue (M), as well as bone at varying stages of maturation (0) (x 50)
were prominent within the tumour. The histopathological diagnosis was not clear but it was suggested that the lesion was a fibro-osseous tumour with a tendency towards aggressive behaviour. 'At follow-up 1 year later, the patient was symptomfree and the bone appeared to be consolidating. Two years later, she complained of sporadic pain, although neither clinical nor radiographic examination revealed any signs of recurrence. The following year, the patient reported her symptoms had completely resolved but a further panoramic radiograph revealed the excised region to contain another mixed-density lesion with multiple radiopacities, consistent with a recurrence (Figure 4). The question of further exploration was considered. Six weeks later, the patient presented complaining of further pain. On examination, the lower
Figure 4 Panoramic radiograph 3 years following the first operation. The mixed density recurrence is clearly seen
66
Dentomaxillofac. Radiol., 1991, Vol. 20, May
border of the mandible was markedly tender and the tumour was excised with a wide margin of sound bone. Histological examination of the specimen from the second operation revealed that it consisted of fibroblasts of varying shape and a mixture of cell types in a myxoid matrix. There was no evidence of cellular polymorphy or increased mitotic activity. There were elements with basophilic, partly ossified/chondroid material, and areas with many small blood vessels containing erythrocytes. A definitive diagnosis of chondromyxoid fibroma was made and this was supported by retrospective review of the specimen from the first operation. Two years later there is no evidence of further recurrence.
Discussion Chondromyxoid fibroma has been reported in the literature in more than 350 patients, most often in the proximal metaphysis of the long bones where it constitutes approximately 1% of all bone tumours I. It has been rarely found in the craniofacial region, once in the parietal bone 2, once in the base of the skull:', once in the occipital bone ', twice in the frontal bone 4 . 5 and in nine cases in the jaws, in all instances in either the molar or symphysis region of the mandible'"!". The clinical symptoms are non-specific and seldom diagnostic. Radiographically, it appears as a multilocular, mixed density lesion containing trabeculation with a corticated margin I. 15. There are a wide range of microscopic features but typically the tumour shows a high degree of cellular pleomorphism. The cells themselves are divided at the periphery into lobules by narrow, richly vascularized, fibrous bands. Multinuclear giant cells are present as well as others with small nuclei which may be more or less stellate and have prominent ovoid or multipolar nuclei. The cells are embedded in a more or less myxoid or chondroid intercellular matrix and the tumour exhibits varying degrees of collagenization and hyalinization, sometimes imitating cartilage. In other cases there are foci of calcification or ossification 15.16. The tumour may therefore sometimes be mistaken for chondrosarcoma, myxoid chondrosarcoma, endochondrorna, myxoma or osteogenic sarcoma!". Two lesions defined as chondromyxoid fibroma'i!' were later diagnosed as
Recurrence of chondromyxoid fibroma: B. Danielsen et aI. chondrosarcomas 17.18. In the first case, it remains unclear whether this was due to misclassification or malignant transformation, whereas in the second case, the review of the original tumour confirmed the second diagnosis. Chondromyxoid fibroma in the mandible has presented with pain 9.12 and or slowly increasing swelling7-13 • The tumour erodes bone as it expands, thinning and eventualIy breaking through the cortex, but the bone always remains covered by periosteum6.9-11.13.14. Loss of lamina dura, root resorption and displacement of adjacent teeth have alI been reported in association with this tumour7.1O.11.13.14. The adjacent teeth were also described in an earlier case as failing to respond to electric pulp tests II. However, soreness on palpation had not previously been described. The clinical symptoms are thus usually non-specific. Since this tumour is extremely rare in the maxillofacial region and, further, the radiographic appearances are no different from many other benign lesions, a correct provisional diagnosis is hardly likely. Histopathology may well prove definitive, especially if the myxoid and chrondroid elements predominate but, none the less, the tumour may still be mistaken for a chondrosarcoma, myxoma or osteogenic sarcoma 16. The chondromyxoid fibroma is an essentially benign, slow-growing tumour which occasionally erodes bone and causes pain. The combined radiographic and histological findings will therefore be those of a benign lesion with aggressive behaviour. In view of the bony erosion, surgical treatment is mandatory I and various types of treatment have been performed in the mandible, ranging from curettage9.1/).12, enucleation'':7.18 to local block resection 11.13. In the long bones, treatment by curettage has resulted in a recurrence in approximately 20% of cases I9.20, but in the mandible, this has only been reported once!", The natural history of the present case stresses the need for more radical treatment of this tumour consistent with its relatively aggressive nature.
References I. Rahimi A, Beaubout JW, Ivins JC, Dahlin DC. Chondromyxoid fibroma: clinicopathologic study of 76 cases. Cancer 1972; 30: 726-36.
2. Jaffe HL. Chondromyxoid fibroma. In: Jaffe HL, ed. Tumors and Tumorous Conditions of Bones and Joints. Philadelphia: Lea & Febeiger 1958; 203-12. 3. Everke H. Ein Myxochondrom-Chondromyxoid-Fibrom - Der Schadelbasis mit Ausdehnung in der Canalis spinalis. Acta Neurochir 1966; 15: 150-8. 4. Thurner VJ, Lisanti M. Ein Chondromyxoidfibroma der Stirnbeinschuppe. Zentralbl Allg Patho11981; 125: 473-80. 5. Miyamoto E, Kuriyama T, Iwamoto M, Tsuji N, Shizuki K. Cranial chondromyxoid fibroma. Case report. J Neurosurg 1981; 55: 1001-3. 6. Srivastava SP. Tumors of the jaw. Indian Surg 1955; 17: 1-5. 7. Schutt PG, Frost HM. Chondromyxoid fibroma. Clin Orthop 1971; 78: 232-9. 8. Spjut HJ, Dorfman MD, Fechner RE, Ackerman LV. Tumors of bone and cartilage. In: Atlas of Tumor Pathology. Washington DC: Armed Forces Institute of Pathology 1971; 50-9. 9. Grotepass FW, Farman AG, Nortje CJ. Chondromyxoid fibroma of the mandible. J Oral Surg 1976; 34: 988-94. 10. Browne RM, Rivas PH. Chondromyxoid fibroma of the mandible: a case report. Br J Oral Surg 1977; 15: 19-25. II. Davis GB, Tideman H. Chondromyxoid fibroma of the mandible. Int J Oral Surg 1978; 7: 23-6. 12. Thompson SH, Weathers DR, Vatral 11. Chondromyxoid fibroma of the jaws. Head Neck Surg 1982; 4: 330-4. 13. Kearney V. Chondromyxoid fibroma of the mandible. Br J Oral Surg 1983; 21: 304-7. 14. Pinholt E, Eldeeb M, Waite D. Chondromyxoid fibroma. Int J Oral Maxillofac Surg 1986; 15: 553-64. 15. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone. A distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol 1948; 45: 541-51. 16. Lichtenstein L. Classification of primary tumors in bone. Cancer 1951; 4: 335-41. 17. Grotepass FW, Wyma G, Nortje CJ, Farman AG. Chondrosarcoma initially diagnosed as a chondromyxoid fibroma: malignant transformation? Dentomaxillofac Radiol 1988; 17: 139-43. 18. Gallia L, Tideman H, Bronkhorst F. Chondrosarcoma of the mandible misdiagnosed as chondromyxoid fibroma. Int J Oral Surg 1980; 9: 221-4. 19. Ralph LL. Chondromyxoid fibroma of bone. J Bone Joint Surg 1962; 448: 7-24. 20. Dahlin DC. Chondromyxoid fibroma of bone with emphasis on its morphological relationship to benign chondroblastoma. Cancer 1956; 9: 195-203.
Address: Dr Bo Danielsen, Institute of Oral Medicine and Diagnosis, Department of Radiology, Royal Dental College Aarhus, DK-8000 Aarhus C, Denmark.
Dentomaxillofac. Radiol., 1991, Vol. 20, May
67
Received 4 June 1990 and in final form 10 August 1990 A case of recurrence of a chondromyxoid fibroma 3 years after its surgical removal is presented. The significance of the clinical, radiographic and histological appearances is discussed. Keywords: Fibroma; mandible; recurrence
Case report A 22-year-old Caucasian woman complained to her dentist of a thumping pain in the lower right molar region when she jogged. On examination, the lower border of the right mandible was tender to palpation; intra-orally, there was swelling lingual to the first and second molars. Both teeth responded similarly to those on the left to electric pulp testing. An intra-oral radiograph revealed a radiolucency related to the apices of both teeth, suggesting that her symptoms were due to chronic periapical infection. However, when the amalgam restorations were removed, both teeth continued to give a positive response to the electric pulp tester. Sedative dressings were therefore inserted. One week later, the second molar failed to respond to the electric pulp tester as well as to a test cavity. However, some vitality was noted as the pulp chamber' was penetrated. Endodontic treatment was completed without complications. Six months later her symptoms remained unchanged and she was therefore referred to the Department of Oral and Maxillofacial Surgery, Royal Dental College, Aarhus, Denmark. On examination, there was a tender swelling at the lower border aOnd on the inner aspect of the body of the right mandible in the molar region. Radiographic examination (Figure 1) revealed a mixed density lesion, S x 3 cm in size, related to the apices of both lower right molars. The lesion contained irregular trabeculation. The margins were well defined with erosion and expansion of the lower border. On the basis of the clinical history and radiographic findings, the lesion was considered a benign tumour, probably an ossifying fibroma, but the patient refused further investigation. She returned 6 months later when there had been a slight increase in the swelling. A further radiograph revealed more marked trabeculation within the lesion and a tendency towards a multilocular appearance. In view of this evidence of increased activity, the tumour was removed with meticulous curettage and apicectomy of the molar teeth was performed and the roots were treated by retrograde root filling (Figure 2). The gross specimen was composed of white lobulated tissue with a few small cystic and haemorrhagic areas. Some calcification was observed. On histological examination, the tissue was heterogeneous in appearance: some
Figure I Part of the panoramic radiograph at the first visit. There is a well-defined, mixed density lesion, 5 x 3 em, related to the lower right first and second molars. The inferior cortical plate is both eroded and expanded
areas were dominated by fibroblasts and a relatively large number of osteoclasts, others by a myxoid matrix with a few star-shaped cells and yet others by regular basophilic cementoid/chondroid tissue, both with varying degrees of maturation (Figure 3). Blood vessels
Figure 2 Postoperative panoramic radiograph following excision of the lesion and retrograde root fillings and apicectomies on 46 and 47
Dentomaxillofac. Radio!., 1991, Vo!. 20, May 65
Recurrence of chondromyxoid fibroma: B. Danielsen et al.
'. I,
,
" , II.
I
..
.' .. ,
.~
."
(
Figure 5 Panoramic radiograph two years after the second operation. The reserved margins are well defined
Figure 3 Histopathologically, the tissue appears heterogeneous. There is fibrous tissue (F), myxoid tissue (M), as well as bone at varying stages of maturation (0) (x 50)
were prominent within the tumour. The histopathological diagnosis was not clear but it was suggested that the lesion was a fibro-osseous tumour with a tendency towards aggressive behaviour. 'At follow-up 1 year later, the patient was symptomfree and the bone appeared to be consolidating. Two years later, she complained of sporadic pain, although neither clinical nor radiographic examination revealed any signs of recurrence. The following year, the patient reported her symptoms had completely resolved but a further panoramic radiograph revealed the excised region to contain another mixed-density lesion with multiple radiopacities, consistent with a recurrence (Figure 4). The question of further exploration was considered. Six weeks later, the patient presented complaining of further pain. On examination, the lower
Figure 4 Panoramic radiograph 3 years following the first operation. The mixed density recurrence is clearly seen
66
Dentomaxillofac. Radiol., 1991, Vol. 20, May
border of the mandible was markedly tender and the tumour was excised with a wide margin of sound bone. Histological examination of the specimen from the second operation revealed that it consisted of fibroblasts of varying shape and a mixture of cell types in a myxoid matrix. There was no evidence of cellular polymorphy or increased mitotic activity. There were elements with basophilic, partly ossified/chondroid material, and areas with many small blood vessels containing erythrocytes. A definitive diagnosis of chondromyxoid fibroma was made and this was supported by retrospective review of the specimen from the first operation. Two years later there is no evidence of further recurrence.
Discussion Chondromyxoid fibroma has been reported in the literature in more than 350 patients, most often in the proximal metaphysis of the long bones where it constitutes approximately 1% of all bone tumours I. It has been rarely found in the craniofacial region, once in the parietal bone 2, once in the base of the skull:', once in the occipital bone ', twice in the frontal bone 4 . 5 and in nine cases in the jaws, in all instances in either the molar or symphysis region of the mandible'"!". The clinical symptoms are non-specific and seldom diagnostic. Radiographically, it appears as a multilocular, mixed density lesion containing trabeculation with a corticated margin I. 15. There are a wide range of microscopic features but typically the tumour shows a high degree of cellular pleomorphism. The cells themselves are divided at the periphery into lobules by narrow, richly vascularized, fibrous bands. Multinuclear giant cells are present as well as others with small nuclei which may be more or less stellate and have prominent ovoid or multipolar nuclei. The cells are embedded in a more or less myxoid or chondroid intercellular matrix and the tumour exhibits varying degrees of collagenization and hyalinization, sometimes imitating cartilage. In other cases there are foci of calcification or ossification 15.16. The tumour may therefore sometimes be mistaken for chondrosarcoma, myxoid chondrosarcoma, endochondrorna, myxoma or osteogenic sarcoma!". Two lesions defined as chondromyxoid fibroma'i!' were later diagnosed as
Recurrence of chondromyxoid fibroma: B. Danielsen et aI. chondrosarcomas 17.18. In the first case, it remains unclear whether this was due to misclassification or malignant transformation, whereas in the second case, the review of the original tumour confirmed the second diagnosis. Chondromyxoid fibroma in the mandible has presented with pain 9.12 and or slowly increasing swelling7-13 • The tumour erodes bone as it expands, thinning and eventualIy breaking through the cortex, but the bone always remains covered by periosteum6.9-11.13.14. Loss of lamina dura, root resorption and displacement of adjacent teeth have alI been reported in association with this tumour7.1O.11.13.14. The adjacent teeth were also described in an earlier case as failing to respond to electric pulp tests II. However, soreness on palpation had not previously been described. The clinical symptoms are thus usually non-specific. Since this tumour is extremely rare in the maxillofacial region and, further, the radiographic appearances are no different from many other benign lesions, a correct provisional diagnosis is hardly likely. Histopathology may well prove definitive, especially if the myxoid and chrondroid elements predominate but, none the less, the tumour may still be mistaken for a chondrosarcoma, myxoma or osteogenic sarcoma 16. The chondromyxoid fibroma is an essentially benign, slow-growing tumour which occasionally erodes bone and causes pain. The combined radiographic and histological findings will therefore be those of a benign lesion with aggressive behaviour. In view of the bony erosion, surgical treatment is mandatory I and various types of treatment have been performed in the mandible, ranging from curettage9.1/).12, enucleation'':7.18 to local block resection 11.13. In the long bones, treatment by curettage has resulted in a recurrence in approximately 20% of cases I9.20, but in the mandible, this has only been reported once!", The natural history of the present case stresses the need for more radical treatment of this tumour consistent with its relatively aggressive nature.
References I. Rahimi A, Beaubout JW, Ivins JC, Dahlin DC. Chondromyxoid fibroma: clinicopathologic study of 76 cases. Cancer 1972; 30: 726-36.
2. Jaffe HL. Chondromyxoid fibroma. In: Jaffe HL, ed. Tumors and Tumorous Conditions of Bones and Joints. Philadelphia: Lea & Febeiger 1958; 203-12. 3. Everke H. Ein Myxochondrom-Chondromyxoid-Fibrom - Der Schadelbasis mit Ausdehnung in der Canalis spinalis. Acta Neurochir 1966; 15: 150-8. 4. Thurner VJ, Lisanti M. Ein Chondromyxoidfibroma der Stirnbeinschuppe. Zentralbl Allg Patho11981; 125: 473-80. 5. Miyamoto E, Kuriyama T, Iwamoto M, Tsuji N, Shizuki K. Cranial chondromyxoid fibroma. Case report. J Neurosurg 1981; 55: 1001-3. 6. Srivastava SP. Tumors of the jaw. Indian Surg 1955; 17: 1-5. 7. Schutt PG, Frost HM. Chondromyxoid fibroma. Clin Orthop 1971; 78: 232-9. 8. Spjut HJ, Dorfman MD, Fechner RE, Ackerman LV. Tumors of bone and cartilage. In: Atlas of Tumor Pathology. Washington DC: Armed Forces Institute of Pathology 1971; 50-9. 9. Grotepass FW, Farman AG, Nortje CJ. Chondromyxoid fibroma of the mandible. J Oral Surg 1976; 34: 988-94. 10. Browne RM, Rivas PH. Chondromyxoid fibroma of the mandible: a case report. Br J Oral Surg 1977; 15: 19-25. II. Davis GB, Tideman H. Chondromyxoid fibroma of the mandible. Int J Oral Surg 1978; 7: 23-6. 12. Thompson SH, Weathers DR, Vatral 11. Chondromyxoid fibroma of the jaws. Head Neck Surg 1982; 4: 330-4. 13. Kearney V. Chondromyxoid fibroma of the mandible. Br J Oral Surg 1983; 21: 304-7. 14. Pinholt E, Eldeeb M, Waite D. Chondromyxoid fibroma. Int J Oral Maxillofac Surg 1986; 15: 553-64. 15. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone. A distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol 1948; 45: 541-51. 16. Lichtenstein L. Classification of primary tumors in bone. Cancer 1951; 4: 335-41. 17. Grotepass FW, Wyma G, Nortje CJ, Farman AG. Chondrosarcoma initially diagnosed as a chondromyxoid fibroma: malignant transformation? Dentomaxillofac Radiol 1988; 17: 139-43. 18. Gallia L, Tideman H, Bronkhorst F. Chondrosarcoma of the mandible misdiagnosed as chondromyxoid fibroma. Int J Oral Surg 1980; 9: 221-4. 19. Ralph LL. Chondromyxoid fibroma of bone. J Bone Joint Surg 1962; 448: 7-24. 20. Dahlin DC. Chondromyxoid fibroma of bone with emphasis on its morphological relationship to benign chondroblastoma. Cancer 1956; 9: 195-203.
Address: Dr Bo Danielsen, Institute of Oral Medicine and Diagnosis, Department of Radiology, Royal Dental College Aarhus, DK-8000 Aarhus C, Denmark.
Dentomaxillofac. Radiol., 1991, Vol. 20, May
67
