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Chondrosarcoma of the mandibular symphysis
OralSURGERY Oral MEDICINE AND
Oral PATH 0 LO GY
VOLUME
25
NUMBER
5
MAY,
1968
Operative oral surgery
Chondrosarcoma of the mandibular symphysis Report
of a case
Michael H. Pronstin, M.D., James IS. Hutcheson, Harold I,. Sarders, X.D., Tampu, Fin. DIWARTXIEXT
OF
I’ATHOLOGY,
TAI\IPA
GENERAL
N.D., and
HOSI’ITAL
Fig. 1. Benign embryonic fibrocartilaginous symphysis. (Magnification, x150.)
rest
tissue
in curettings
from
mandibular
bundles of benign and occasionally anuclear collagenized chondroid tissue (Fig. 1). This cartilage characterized by plump blended almost imperceptibly into foci of malignant and hyperchromatic nuclei and lacunae in which multinucleated cells were present. Mitoses were not conspicuous (Fig. 2). X-ray examination at the time of the patient’s admission to the hospital revealed a bony defect in the anterior portion of the mandible in the region of the alveolar ridge, extending 1.5 cm. to each side of the symphysis (Fig. 3). On the third hospital day a partial mandibulectomy was performed. The lower lip was split in the midline and the diagastric, hypoglossus, and masseter muscles were incised, after which the first and second molars on each side were extracted. The mandible was then severed in front of the third molar bilaterally. A stainless steel prosthesis was inserted in the place of the resected mandible, and the floor of the mouth, cheek flap, and lower lip were reconstructed. The patient has been followed for 7 years and continues to do well, with no evidence of recurrence or metastasis. Examination of the surgical specimen revealed a portion of a mandible, resected 3 cm. on either side of the midline. A 3 by 2 cm. operative defect replaced the midline dentition and extended 1.5 cm. deep into the bone. Microscopic examination failed to reveal rest of fibrocartilaginous tissue the presence of residual tumor, but a 5 mm. embryonic was demonstrated in the superior lingual margin of the mandible.
DISCUSSION
We believe that this is only the fourth reported case of chondrosarcoma of the mandibular symphysis. Of fifty-five reported chondrosarcomas of the jaws, thirty-four cases originated in the maxilla1-31 6l 7l g and twenty-one in the mandiblc3, 4, lo As compared with other bony tumors of the maxilla or mandible, chondrosarcoma is relatively rare. In a survey of jaw sarcomas in Denmark, only one of forty-three various sarcomas was a chondrosarcoma.10 Kragh and cartilaginous tumors of the jaw and facial area, and associates,G reviewing Henderson and Dahlin5 in a study of 288 cases of chondrosarcoma of bone,
Volume 25 Number 5
Chondrosarcoma
of
mandibular
symphysis
667
Fj,q. 8. Malignant cartilage characterized by binucleated, plump, and hyperchromatic nuclei. (Magnification, x450.)
.:, Hc rrport,ed only two cases of chondrosarcoma of the maxilla and none in the ~r~andihlc. In an extensive review of the English likrature, Chaudhr?; and colleagues3 reported twentp-one and fifteen chondrosarcomas of the maxilla and mandible, respectively. Only eighteen eases of benign chondroma or its variants were documented, indicating that the malignant tumor was more common than its benign counterpart. The pathogcnesis of cartilaginous tumors of the mandible is obscure, inas-
O.S., O.M. & OP. May, 1968
much as cartilage normally is not present after the first year of life. The mandible develops bilaterally- in the fetus as a thin plate of bone. Associated with this development is a, round rod of cartilage (Meckel’s cartilage) which extends from the area of the middle car to the midline, where it is in contact with the cartilage of the other side. Most of this cartilage disappears without contributing to the formation of the bone of the mandible. The two halves of the mandible are joined in the midline by fibrocartilage which is derived from differentiation of connective tissue in the midline. By the end of the first year the t,wo halves of the mandible unite by ossification of the symphyseal fibrocartilage.8 It has been postulated that the chief source of cartilaginous cells are vestigial rests. In the mandibular symphysis, where cartilaginous elements should disappear well before the first year of life, remnants of Meckel’s cartilage may persist. Other sources of cartilage cells include chondroid bone, differentiation of mesenchymal cells into chondroblasts, and metaplasia of connective tissue into c*artilage.3 In the case presented here, the embryonic fibrocartilaginous rest tissue, intimately associated with the tumor and in the lingual margin, is rcgarded as strong evidence of the chondrosarcoma’s origin in either a residuum of Meckcl’s cartilage or the fibrocartilage of the developing mandibular symphysis. Because experience with these lesions has been limited, little is known concerning the prognosis of chondrosarcoma of the jaws. 9 survey of the Iiterature revealed thirty-five cases of chondrosarcoma of the mandible or maxilla which were followed for 2 to 5 years, l-43 6, 7 Twenty-one patients (60 per cent) died of the disease within 5 years. Death was due to either extensive local destruction or distant meta.stasis, most commonly t.o the lungs and bones. In nine patients (26 per cent) there were one or more recurrences. Five year follow-up records after the last recurrence were available for only two patients, in whom no evidence of disease was found. Five patients (14 per cent) were alive and well, without recurrence, more than 5 years after the initial diagnosis was made. Five other patients are reported to have had no recurrence, but these had been followed for less than 5 years. Radical surgical intervention appears to be the treatment of choice. Radiation therapy is indicated only for the treatment of recurrent tumors. There appears to be a good correlation between the prognosis and the histologic grade of the tumor.g Low-grade chondrosarcomas are associated with the more favorable prognosis, but when the tumor is less well differentiated the outlook is poor. In the case presented here, the patient is alive and well, with no recurrence of the tumor, 7 years after removal of a low-grade, well-differentiated chondrosarcoma of the mandibular symphysis. SUMMARY
AND
CONCLUSIONS
1. The fourth known ease of ehondrosareoma arising in the area of the mandibular symphysis is reported. Seven years after resection of a portion of the mandible, there is no evidence of disease, 2. A survey of the literature reveals only thirty-four cases of chondrosarcoma
Volume 25 Number 5
Chontlrosarcomn
of mandibular
synaphysis
669
of the maxilla and twent.y-one cases invol\-in g the mandible. As compared with the incidence of chondrosarcomas elsewhere and various sarcomas of the jaws, c+ondrosarcoma arising in the maxilla or mandible is a, very uncommon lesion. It is, however, more common than the benign chondromas of the jaws. of th 3. Evidence is presented to support the concept that chondrosartoma mandibular symphysis may arise from a, vestigial rest ( AIeckrl’s cartilage or the fibrocartilagc of the developing mandibular symphysis) . 4. %t,y per cent ot’ thirt.y-five patients with ~ltontlrosa~rolna of thP maxilla or mandible died of the tumor within 5 years. Tn 26 per cent of the c.ases there were one or more recurrences. The more favorable prognosis is associated wit,11 the well-differentiated, low-grade tumors. The authors gratefully acknowledge the histologic consultations with Drs. H. L. Jaffe and A. 1). Johnston, Hospital for *Joint Diseases, lVew York, N. Y., and General .J. 31. Blumberg, USA, Armed Forces Institute of Pathology, \Yashington, D. C. REFERENCES
1. Batsakis, J. G., and Dito, W. K.: Chondrosarcoma of the Maxilla, &4rch. Otolarpng. 75: 69-75, 1962. 3. Botstein, Chondrosarcoma of the Maxilla C., and Marcus, N.: A Case of Recurrent Treated Unsuccessfully With Sulfur-35, Am. ,T. Roentgenol. 89: 555558, 1963. A. P., Robinovitch, M. R., Mitchell, D. I?., and Vickers, R. A.: Chondrogenic 3. Chaudhry, Tumors of the Jaws? Am. J. Surg. 102: 403-411, 1961. 4. Cohen, B., and Srnlth, C. J.: Chondrosarcoma of the Mandible, Bnn. Roy. Coll. Surg. Eng. 32: 303-313, 1963. E. I)., and Dahlin, 1). C.: Chondrosarcoma of Bone-A Survey of 358 Cases, 5. Henderson, .T. Bone & Joint Surg. 45A: 1450-1458, 1963. ti. Kragh, L. V., Dahlin, D. C., and Erich, J. B.: Cartilaginous Tumors of the Jaws and Facial Regions, Am. J. Surg. 99: 852-856, 1960. A., Ramm, C., and Fani, IX.: Chondrosarcoma of Maxilla (Case Report), J. 7. T,apitlot, T,aryng. 80: 743-747, 1966. : Oral Ilistology and Embryology, St. Louis, 19-11, The C’. V. Moshy 8. Orban, B. (editor) Companp, pp. 190-196. 9. Phelan, J. T., and Cabrera, A.: (.~hollllro~arc~ollla of Bone. Sing. Gynec. & Obst. 119: 42, 1964. .T. .T.: The Tncitlence Rale of Jaw Sarcomas in T)rnmark, ORAL ST-RO., ORAL 1n. Pindborg, MEI).
R- ORAL PATH.
14:
L"i6-"if? -
> 1961.
Oral PATH 0 LO GY
VOLUME
25
NUMBER
5
MAY,
1968
Operative oral surgery
Chondrosarcoma of the mandibular symphysis Report
of a case
Michael H. Pronstin, M.D., James IS. Hutcheson, Harold I,. Sarders, X.D., Tampu, Fin. DIWARTXIEXT
OF
I’ATHOLOGY,
TAI\IPA
GENERAL
N.D., and
HOSI’ITAL
Fig. 1. Benign embryonic fibrocartilaginous symphysis. (Magnification, x150.)
rest
tissue
in curettings
from
mandibular
bundles of benign and occasionally anuclear collagenized chondroid tissue (Fig. 1). This cartilage characterized by plump blended almost imperceptibly into foci of malignant and hyperchromatic nuclei and lacunae in which multinucleated cells were present. Mitoses were not conspicuous (Fig. 2). X-ray examination at the time of the patient’s admission to the hospital revealed a bony defect in the anterior portion of the mandible in the region of the alveolar ridge, extending 1.5 cm. to each side of the symphysis (Fig. 3). On the third hospital day a partial mandibulectomy was performed. The lower lip was split in the midline and the diagastric, hypoglossus, and masseter muscles were incised, after which the first and second molars on each side were extracted. The mandible was then severed in front of the third molar bilaterally. A stainless steel prosthesis was inserted in the place of the resected mandible, and the floor of the mouth, cheek flap, and lower lip were reconstructed. The patient has been followed for 7 years and continues to do well, with no evidence of recurrence or metastasis. Examination of the surgical specimen revealed a portion of a mandible, resected 3 cm. on either side of the midline. A 3 by 2 cm. operative defect replaced the midline dentition and extended 1.5 cm. deep into the bone. Microscopic examination failed to reveal rest of fibrocartilaginous tissue the presence of residual tumor, but a 5 mm. embryonic was demonstrated in the superior lingual margin of the mandible.
DISCUSSION
We believe that this is only the fourth reported case of chondrosarcoma of the mandibular symphysis. Of fifty-five reported chondrosarcomas of the jaws, thirty-four cases originated in the maxilla1-31 6l 7l g and twenty-one in the mandiblc3, 4, lo As compared with other bony tumors of the maxilla or mandible, chondrosarcoma is relatively rare. In a survey of jaw sarcomas in Denmark, only one of forty-three various sarcomas was a chondrosarcoma.10 Kragh and cartilaginous tumors of the jaw and facial area, and associates,G reviewing Henderson and Dahlin5 in a study of 288 cases of chondrosarcoma of bone,
Volume 25 Number 5
Chondrosarcoma
of
mandibular
symphysis
667
Fj,q. 8. Malignant cartilage characterized by binucleated, plump, and hyperchromatic nuclei. (Magnification, x450.)
.:, Hc rrport,ed only two cases of chondrosarcoma of the maxilla and none in the ~r~andihlc. In an extensive review of the English likrature, Chaudhr?; and colleagues3 reported twentp-one and fifteen chondrosarcomas of the maxilla and mandible, respectively. Only eighteen eases of benign chondroma or its variants were documented, indicating that the malignant tumor was more common than its benign counterpart. The pathogcnesis of cartilaginous tumors of the mandible is obscure, inas-
O.S., O.M. & OP. May, 1968
much as cartilage normally is not present after the first year of life. The mandible develops bilaterally- in the fetus as a thin plate of bone. Associated with this development is a, round rod of cartilage (Meckel’s cartilage) which extends from the area of the middle car to the midline, where it is in contact with the cartilage of the other side. Most of this cartilage disappears without contributing to the formation of the bone of the mandible. The two halves of the mandible are joined in the midline by fibrocartilage which is derived from differentiation of connective tissue in the midline. By the end of the first year the t,wo halves of the mandible unite by ossification of the symphyseal fibrocartilage.8 It has been postulated that the chief source of cartilaginous cells are vestigial rests. In the mandibular symphysis, where cartilaginous elements should disappear well before the first year of life, remnants of Meckel’s cartilage may persist. Other sources of cartilage cells include chondroid bone, differentiation of mesenchymal cells into chondroblasts, and metaplasia of connective tissue into c*artilage.3 In the case presented here, the embryonic fibrocartilaginous rest tissue, intimately associated with the tumor and in the lingual margin, is rcgarded as strong evidence of the chondrosarcoma’s origin in either a residuum of Meckcl’s cartilage or the fibrocartilage of the developing mandibular symphysis. Because experience with these lesions has been limited, little is known concerning the prognosis of chondrosarcoma of the jaws. 9 survey of the Iiterature revealed thirty-five cases of chondrosarcoma of the mandible or maxilla which were followed for 2 to 5 years, l-43 6, 7 Twenty-one patients (60 per cent) died of the disease within 5 years. Death was due to either extensive local destruction or distant meta.stasis, most commonly t.o the lungs and bones. In nine patients (26 per cent) there were one or more recurrences. Five year follow-up records after the last recurrence were available for only two patients, in whom no evidence of disease was found. Five patients (14 per cent) were alive and well, without recurrence, more than 5 years after the initial diagnosis was made. Five other patients are reported to have had no recurrence, but these had been followed for less than 5 years. Radical surgical intervention appears to be the treatment of choice. Radiation therapy is indicated only for the treatment of recurrent tumors. There appears to be a good correlation between the prognosis and the histologic grade of the tumor.g Low-grade chondrosarcomas are associated with the more favorable prognosis, but when the tumor is less well differentiated the outlook is poor. In the case presented here, the patient is alive and well, with no recurrence of the tumor, 7 years after removal of a low-grade, well-differentiated chondrosarcoma of the mandibular symphysis. SUMMARY
AND
CONCLUSIONS
1. The fourth known ease of ehondrosareoma arising in the area of the mandibular symphysis is reported. Seven years after resection of a portion of the mandible, there is no evidence of disease, 2. A survey of the literature reveals only thirty-four cases of chondrosarcoma
Volume 25 Number 5
Chontlrosarcomn
of mandibular
synaphysis
669
of the maxilla and twent.y-one cases invol\-in g the mandible. As compared with the incidence of chondrosarcomas elsewhere and various sarcomas of the jaws, c+ondrosarcoma arising in the maxilla or mandible is a, very uncommon lesion. It is, however, more common than the benign chondromas of the jaws. of th 3. Evidence is presented to support the concept that chondrosartoma mandibular symphysis may arise from a, vestigial rest ( AIeckrl’s cartilage or the fibrocartilagc of the developing mandibular symphysis) . 4. %t,y per cent ot’ thirt.y-five patients with ~ltontlrosa~rolna of thP maxilla or mandible died of the tumor within 5 years. Tn 26 per cent of the c.ases there were one or more recurrences. The more favorable prognosis is associated wit,11 the well-differentiated, low-grade tumors. The authors gratefully acknowledge the histologic consultations with Drs. H. L. Jaffe and A. 1). Johnston, Hospital for *Joint Diseases, lVew York, N. Y., and General .J. 31. Blumberg, USA, Armed Forces Institute of Pathology, \Yashington, D. C. REFERENCES
1. Batsakis, J. G., and Dito, W. K.: Chondrosarcoma of the Maxilla, &4rch. Otolarpng. 75: 69-75, 1962. 3. Botstein, Chondrosarcoma of the Maxilla C., and Marcus, N.: A Case of Recurrent Treated Unsuccessfully With Sulfur-35, Am. ,T. Roentgenol. 89: 555558, 1963. A. P., Robinovitch, M. R., Mitchell, D. I?., and Vickers, R. A.: Chondrogenic 3. Chaudhry, Tumors of the Jaws? Am. J. Surg. 102: 403-411, 1961. 4. Cohen, B., and Srnlth, C. J.: Chondrosarcoma of the Mandible, Bnn. Roy. Coll. Surg. Eng. 32: 303-313, 1963. E. I)., and Dahlin, 1). C.: Chondrosarcoma of Bone-A Survey of 358 Cases, 5. Henderson, .T. Bone & Joint Surg. 45A: 1450-1458, 1963. ti. Kragh, L. V., Dahlin, D. C., and Erich, J. B.: Cartilaginous Tumors of the Jaws and Facial Regions, Am. J. Surg. 99: 852-856, 1960. A., Ramm, C., and Fani, IX.: Chondrosarcoma of Maxilla (Case Report), J. 7. T,apitlot, T,aryng. 80: 743-747, 1966. : Oral Ilistology and Embryology, St. Louis, 19-11, The C’. V. Moshy 8. Orban, B. (editor) Companp, pp. 190-196. 9. Phelan, J. T., and Cabrera, A.: (.~hollllro~arc~ollla of Bone. Sing. Gynec. & Obst. 119: 42, 1964. .T. .T.: The Tncitlence Rale of Jaw Sarcomas in T)rnmark, ORAL ST-RO., ORAL 1n. Pindborg, MEI).
R- ORAL PATH.
14:
L"i6-"if? -
> 1961.