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Chondrosarcoma of mandible misdiagnosed as chondromyxoid fibroma
Int. J. Oral Surg. 1980: 9: 221-224 (Key words: tumor, malignant: malignancies,· sarcoma,' surgery, oral)
Chondrosarcoma of mandible misdiagnosed as chondromyxoid fibroma LOU GALLIA, HENK TIDEMAN AND FRANS BRONKHORST University of Washington, Department of Oral and Maxillofacial Surgery, Seattle, Wash.} U.S.A. and Clinic for Oral and Maxillofacial Surgery and Department of Pathology, Gemeente Ziekenhuis, Arnhem, The Netherlands
The diagnosis of chondrosarcoma of the jaws is often difficult due to the often indistinct boundaries between benign and malignant chondromatous tumors. In view of the often considerable variation in histological characteristics within a tumor, repeated biopsies and step sections through a specimen are recommended in difficult cases. This paper is a follow-up of the case described by DAVIS & TIDEMAN (1978) in this joumal6•
ABSTRACT -
(Received for publication 25 April, accepted 25 August 1979)
Chondrosarcoma is notorious for being underdiagnosed, because histological differentiation between benign and malignant chondromatous tumors is difficult1 2 • Because of the lethal nature of this tumor in the jaws, accurate microscopic evaluation of adequate biopsy material is essential to ensure appropriate surgical procedures. The following report illustrates a case of chondrosarcoma originally reported as chondromyxoid fibroma.
Case report DAVIS & TIDEMAN 6 reported a rare case of chondromyxoid fibroma of the mandible in a 16-year-old Caucasian male. The lesion, which extended from the left first premolar to the right canine, presented as a painless swelling. Histological evaluation of the excisional biopsy was controversial, with differing opinions of benign and malignant chondromatous tumor
presented. Although the lesion was finally reported as a chondromyxoid fibroma, considerable doubt remained conceming the origin of the tumor. Accordingly, the tumor was resected from the left first premolar to the right canine with preservation of the inferior mandibular border. The bony defect was grafted with autogenous cancellous bone from the right iliac crest. The patient remained asymptomatic for 3 years, then presented with a 1month history of recurrent painless mass of the anterior mandibular alveolar ridge which inter· fered with placement of the patient's mandibular partial denture. General physical and laboratory examinations were negative. Intraoral examination disclosed a 1 X 2 cm firm erythematous sessile mass arising from the midline superior mandibular alveolus in the region of the bonegraft (Fig. 1). The lesion was nontender and nonulcerated. X-ray examination reo vealed an intact cortex and normal bony archi· tecture underlying the lesion (Figs. 2,3). Incisional biopsy (Fig. 4) was reported as consistent with low grade chondrosarcoma, mainly because of the increased number of mi-
0300·9785180/030221·04$02,50/0 © 1980 Munksgaard, Copenhagen
222
GALLI A, TIDEMAN AND BRONKHORST
Fig• .1. The recurrent lesion.
totic figures and the sporadic occurrence of biand multiulcerated cells. Review of the original resected tumor demonstrated in some minor fragments a similar histological pattern. Accordingly, an anterior mandibular resection was performed from the second premolar to the left second premolar, including mandibular border. Sufficient labial and floor of the mouth tissues were excised to encompass the lesion with a 2 cm cuff of clinically normal tissue. The defect was bridged with a cortico-cancellous antogenous graft from the left iliac crest secured with interosseous wires and extraoral pin fixation. Three months postoperatively the patient underwent sequestrotomy for partial devitalization of the grafted bone, with insertion of additional cancellous bone obtained from the right iliac crest. Histological examination of the removed tissues was negative for tumor. One year postresection the patient remains tumor free.
Figs. 2, 3. There was no radiographic evidence
of bony destruction.
HISTOPATHOLOGY The tumor shows a mixed appearance (Figs. 5, 6, 7) and there are indications that it originates in the periosteum rather than in the osseous tissue. Generally the tumor is recognized as a chondrosarcoma. In the major part the tumor resembles a mesechymal chondrosarcoma, mainly because of the mixed appearances of the intercellular substance. There is an extensive spreading of the tumor into the surrounding soft tissue and locally also in the underlying bone. Careful microscopic examination of the resection planes of the bone and the soft tissue shows, however, no pres-
ence of tumor, so that the removal can be considered complete.
Discussion All aspects of chondrosarcoma of the jaws have recently been reviewed12 • A case of chondrosarcoma of the condyle3 was described by other authors. The diagnosis of chondrosarcoma is often difficult due mainly to the indefinite boundaries between be-
CHONDROSARCOMA
Fig. 4. The resected specimen from the left first premolar to the right canine.
nign and malignantl l . The histological features of chondrosarcoma and chondromyxoid fibroma have also been described o,13. An important feature of chondromyxoid fibroma is the presence of evident nuclear atypism with one or more hyperchromatic nuclei, often globular or monstrous, simulating a malignant tumor; however, mitotic figures are rare13 • It has been pointed out! that chondrosarcoma has been misdiagnosed as benign more commonly than any other tumor of bone. It was found 4 that 20 % of the cases were inadvertently underdiagnosed at first
Fig. 5. Tumor growing from below into the
dermis of the overlying mucosa. The arrangement of cords of cells in a slightly fibrillar matrix is clearly visible (HE, 40 x).
223
Fig. 6. Irregular shaped cells are lying in clear spaces. There is a very marked nuclear pleomorphism and between the cells partly hyalin, partly fibrillar material is deposited; the overall impression of the tumor is that of a mesenchymal chondrosarcoma. An abnormal annular mitosis is seen in the left side of the figure (HE, 250 x).
microscopic examination. This error may result in insufficiently wide surgical margins, thus jeopardizing the patient's life. Most series report a 5-year survival rate below 50 % for chondrosarcoma of the jawsl , 4,7,12.
Radical resection is in a direct ratio to the width of supposedly normal tissue12 • Conversely, the diagnosis of malignancy in a benign tumor would result in an unnecessarily mutilating operation. Chondramyxoid fibroma was defined o as a "distinctive benign tumor likely to be mistaken for chondrosarcoma". The ideal treatment of chondromyxoid fibroma is a sufficiently wide excision to include a rim of normal bone; although the tumor is benign, a recurrence rate of 25 % has been recorded throughout the body5; of the seven lesions diagnosed as chondromyxoid fibroma l of the mandible reported in the literature 2 ,8, only one has recurred, this after simple curettement. It has been emphasized that car-
224
GALLIA, TIDEMAN AND BRONKHORST
tilaginous tumors may show considerable variation in different areas of the tumor, therefore microscopic examination may lead to an improper diagnosis if insufficient tissue is available ror study. Repeat biopsy and multiple step sections through the specimen have been recommended in difficult cases.
References 1. ACKERMAN, L. V. & SPJUT, H. J.: Tumors of bone and cartilage. Atlas of tumor pathology. Section II. Fascicle 4. Washington, D. C., Armed Forces Institute of Pathology. 2. BROWNIE, R. M. & RIvAS, P. H.: Chondromyxoid fibroma of the mandible: a case report. Br. l. Oral Surg. 1977-78: 15: 19-25. 3. CADENAT, H., CoMBBLLES, G., FABERT, M. & CLOUET, M.: Chondrosarcoma du condyle. Rev. Stomatol. Chir. Maxillofac. 1979: 80: 20-22. 4. CHAUDRY, A. P., ROBINOVlTCH, M. R., MITCHELL, D. F. & VICKERS, R. A: Chondrogenic tumors of the jaws. Am. J. Surg. 1961: 102: 403-411. 5. DAHLIN, D. C.: Chondromyxoid fibroma. In Bone tumors. 2nd ed. pp. 48-57. Charles C. Thomas, Springfield. Ill. 6. DAVIS, G. B. & 1'IDEMAN, H.: Chondromyxoid fibroma of the mandible. Int. l. Oral Surg. 1978: 7: 23-26. 7. F'RoNSTIN, M. H., HUTCHESON, J. B. & SANDERS, H. L.: Chondrosarcoma of the mandibular symphysis. Report of a case. Oral Surg. 1968: 25: 665-669.
8. GROTEPASS, F. W., FARMAN, A G.·& NORTJE, C. J.: Chondromyxoid fibroma of the mandible. J. Oral Surg. 1976: 34: 988-994. 9. JAFFE, H. L. & L!CHTENS'11EIN, L.: Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch. Patho!. 1948: 541-551. 10. KRACH, L. V., DAHLIN, D. L. & EiEJucH, J. B.: Cartilaginous tumors of the jaws and facial regions. Am. l. Surg. 1960: 99: 852856. 11. LUCAS, R. B.: Pathology of tumors oj the oral tissues. 3rd. ed. Churchill Livingstone, Edinburgh-London-New York 1976. 12. SATO, K., NUKAGA, H. & HORIlWSHI, T.: Chondrosarcoma of the jaws and facial skeleton: a review of the Japanese literature. J. Oral Surg. 1977: 35: 892-897. 13. SCHAJOWICS, F. & GALI..A.RDO, H.: Chondromyxoid fibroma (fibromyxoid chondroma) of bone: a clinieo-pathological study of 32 cases. J. Bone Joint Surg. 1971: 53: 198216. 14. SHAFER, W. G., HINE, M. K. & LEVY, B. M.: A text book of oral pathology. 3rd. ed. W. B. Saunders Co., Philadelphia-LondonToronto 1974.
Address:
Henk Tideman Department of Oral and Maxillo-Facial Surgery Gemeente Ziekenhuis Arnhem The Netherlands
Chondrosarcoma of mandible misdiagnosed as chondromyxoid fibroma LOU GALLIA, HENK TIDEMAN AND FRANS BRONKHORST University of Washington, Department of Oral and Maxillofacial Surgery, Seattle, Wash.} U.S.A. and Clinic for Oral and Maxillofacial Surgery and Department of Pathology, Gemeente Ziekenhuis, Arnhem, The Netherlands
The diagnosis of chondrosarcoma of the jaws is often difficult due to the often indistinct boundaries between benign and malignant chondromatous tumors. In view of the often considerable variation in histological characteristics within a tumor, repeated biopsies and step sections through a specimen are recommended in difficult cases. This paper is a follow-up of the case described by DAVIS & TIDEMAN (1978) in this joumal6•
ABSTRACT -
(Received for publication 25 April, accepted 25 August 1979)
Chondrosarcoma is notorious for being underdiagnosed, because histological differentiation between benign and malignant chondromatous tumors is difficult1 2 • Because of the lethal nature of this tumor in the jaws, accurate microscopic evaluation of adequate biopsy material is essential to ensure appropriate surgical procedures. The following report illustrates a case of chondrosarcoma originally reported as chondromyxoid fibroma.
Case report DAVIS & TIDEMAN 6 reported a rare case of chondromyxoid fibroma of the mandible in a 16-year-old Caucasian male. The lesion, which extended from the left first premolar to the right canine, presented as a painless swelling. Histological evaluation of the excisional biopsy was controversial, with differing opinions of benign and malignant chondromatous tumor
presented. Although the lesion was finally reported as a chondromyxoid fibroma, considerable doubt remained conceming the origin of the tumor. Accordingly, the tumor was resected from the left first premolar to the right canine with preservation of the inferior mandibular border. The bony defect was grafted with autogenous cancellous bone from the right iliac crest. The patient remained asymptomatic for 3 years, then presented with a 1month history of recurrent painless mass of the anterior mandibular alveolar ridge which inter· fered with placement of the patient's mandibular partial denture. General physical and laboratory examinations were negative. Intraoral examination disclosed a 1 X 2 cm firm erythematous sessile mass arising from the midline superior mandibular alveolus in the region of the bonegraft (Fig. 1). The lesion was nontender and nonulcerated. X-ray examination reo vealed an intact cortex and normal bony archi· tecture underlying the lesion (Figs. 2,3). Incisional biopsy (Fig. 4) was reported as consistent with low grade chondrosarcoma, mainly because of the increased number of mi-
0300·9785180/030221·04$02,50/0 © 1980 Munksgaard, Copenhagen
222
GALLI A, TIDEMAN AND BRONKHORST
Fig• .1. The recurrent lesion.
totic figures and the sporadic occurrence of biand multiulcerated cells. Review of the original resected tumor demonstrated in some minor fragments a similar histological pattern. Accordingly, an anterior mandibular resection was performed from the second premolar to the left second premolar, including mandibular border. Sufficient labial and floor of the mouth tissues were excised to encompass the lesion with a 2 cm cuff of clinically normal tissue. The defect was bridged with a cortico-cancellous antogenous graft from the left iliac crest secured with interosseous wires and extraoral pin fixation. Three months postoperatively the patient underwent sequestrotomy for partial devitalization of the grafted bone, with insertion of additional cancellous bone obtained from the right iliac crest. Histological examination of the removed tissues was negative for tumor. One year postresection the patient remains tumor free.
Figs. 2, 3. There was no radiographic evidence
of bony destruction.
HISTOPATHOLOGY The tumor shows a mixed appearance (Figs. 5, 6, 7) and there are indications that it originates in the periosteum rather than in the osseous tissue. Generally the tumor is recognized as a chondrosarcoma. In the major part the tumor resembles a mesechymal chondrosarcoma, mainly because of the mixed appearances of the intercellular substance. There is an extensive spreading of the tumor into the surrounding soft tissue and locally also in the underlying bone. Careful microscopic examination of the resection planes of the bone and the soft tissue shows, however, no pres-
ence of tumor, so that the removal can be considered complete.
Discussion All aspects of chondrosarcoma of the jaws have recently been reviewed12 • A case of chondrosarcoma of the condyle3 was described by other authors. The diagnosis of chondrosarcoma is often difficult due mainly to the indefinite boundaries between be-
CHONDROSARCOMA
Fig. 4. The resected specimen from the left first premolar to the right canine.
nign and malignantl l . The histological features of chondrosarcoma and chondromyxoid fibroma have also been described o,13. An important feature of chondromyxoid fibroma is the presence of evident nuclear atypism with one or more hyperchromatic nuclei, often globular or monstrous, simulating a malignant tumor; however, mitotic figures are rare13 • It has been pointed out! that chondrosarcoma has been misdiagnosed as benign more commonly than any other tumor of bone. It was found 4 that 20 % of the cases were inadvertently underdiagnosed at first
Fig. 5. Tumor growing from below into the
dermis of the overlying mucosa. The arrangement of cords of cells in a slightly fibrillar matrix is clearly visible (HE, 40 x).
223
Fig. 6. Irregular shaped cells are lying in clear spaces. There is a very marked nuclear pleomorphism and between the cells partly hyalin, partly fibrillar material is deposited; the overall impression of the tumor is that of a mesenchymal chondrosarcoma. An abnormal annular mitosis is seen in the left side of the figure (HE, 250 x).
microscopic examination. This error may result in insufficiently wide surgical margins, thus jeopardizing the patient's life. Most series report a 5-year survival rate below 50 % for chondrosarcoma of the jawsl , 4,7,12.
Radical resection is in a direct ratio to the width of supposedly normal tissue12 • Conversely, the diagnosis of malignancy in a benign tumor would result in an unnecessarily mutilating operation. Chondramyxoid fibroma was defined o as a "distinctive benign tumor likely to be mistaken for chondrosarcoma". The ideal treatment of chondromyxoid fibroma is a sufficiently wide excision to include a rim of normal bone; although the tumor is benign, a recurrence rate of 25 % has been recorded throughout the body5; of the seven lesions diagnosed as chondromyxoid fibroma l of the mandible reported in the literature 2 ,8, only one has recurred, this after simple curettement. It has been emphasized that car-
224
GALLIA, TIDEMAN AND BRONKHORST
tilaginous tumors may show considerable variation in different areas of the tumor, therefore microscopic examination may lead to an improper diagnosis if insufficient tissue is available ror study. Repeat biopsy and multiple step sections through the specimen have been recommended in difficult cases.
References 1. ACKERMAN, L. V. & SPJUT, H. J.: Tumors of bone and cartilage. Atlas of tumor pathology. Section II. Fascicle 4. Washington, D. C., Armed Forces Institute of Pathology. 2. BROWNIE, R. M. & RIvAS, P. H.: Chondromyxoid fibroma of the mandible: a case report. Br. l. Oral Surg. 1977-78: 15: 19-25. 3. CADENAT, H., CoMBBLLES, G., FABERT, M. & CLOUET, M.: Chondrosarcoma du condyle. Rev. Stomatol. Chir. Maxillofac. 1979: 80: 20-22. 4. CHAUDRY, A. P., ROBINOVlTCH, M. R., MITCHELL, D. F. & VICKERS, R. A: Chondrogenic tumors of the jaws. Am. J. Surg. 1961: 102: 403-411. 5. DAHLIN, D. C.: Chondromyxoid fibroma. In Bone tumors. 2nd ed. pp. 48-57. Charles C. Thomas, Springfield. Ill. 6. DAVIS, G. B. & 1'IDEMAN, H.: Chondromyxoid fibroma of the mandible. Int. l. Oral Surg. 1978: 7: 23-26. 7. F'RoNSTIN, M. H., HUTCHESON, J. B. & SANDERS, H. L.: Chondrosarcoma of the mandibular symphysis. Report of a case. Oral Surg. 1968: 25: 665-669.
8. GROTEPASS, F. W., FARMAN, A G.·& NORTJE, C. J.: Chondromyxoid fibroma of the mandible. J. Oral Surg. 1976: 34: 988-994. 9. JAFFE, H. L. & L!CHTENS'11EIN, L.: Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch. Patho!. 1948: 541-551. 10. KRACH, L. V., DAHLIN, D. L. & EiEJucH, J. B.: Cartilaginous tumors of the jaws and facial regions. Am. l. Surg. 1960: 99: 852856. 11. LUCAS, R. B.: Pathology of tumors oj the oral tissues. 3rd. ed. Churchill Livingstone, Edinburgh-London-New York 1976. 12. SATO, K., NUKAGA, H. & HORIlWSHI, T.: Chondrosarcoma of the jaws and facial skeleton: a review of the Japanese literature. J. Oral Surg. 1977: 35: 892-897. 13. SCHAJOWICS, F. & GALI..A.RDO, H.: Chondromyxoid fibroma (fibromyxoid chondroma) of bone: a clinieo-pathological study of 32 cases. J. Bone Joint Surg. 1971: 53: 198216. 14. SHAFER, W. G., HINE, M. K. & LEVY, B. M.: A text book of oral pathology. 3rd. ed. W. B. Saunders Co., Philadelphia-LondonToronto 1974.
Address:
Henk Tideman Department of Oral and Maxillo-Facial Surgery Gemeente Ziekenhuis Arnhem The Netherlands