The Journal of Laryngology and Otology March 1992, Vol. 106, pp. 273-276
Chondrosarcoma of the hyoid SUHEL HASAN, M.S., V. KANNAN, M.D., ASHOK M. SHENOY, M.S., NANJUNDAPPA, M.S., K. N. NARESH, M.D.
(Bangalore, India)
Abstract Two cases of chondrosarcoma of the hyoid bone are described. They were managed with surgical resection and postoperative radiotherapy. These patients are disease free at 26 months and 15 months respectively.
Introduction Chondrosarcoma is the second most common primary malignant spindle cell tumour of bone (Malawer et al., 1989). Approximately 10-12 per cent arise in head and neck sites where they commonly involve skull base, maxilla and larynx (Cantrell et al., 1980; Burkey et al., 1990). Involvement of hyoid bone is rare. We present two cases of chondrosarcoma of the hyoid, the report illustrates their management and literature review.
a salivary gland tumour with myxoid and calcific material. A wide excision of the tumour mass using a transcervical approach was carried out; this revealed an encapsulated tumour adherent to the left greater cornu of hyoid. Our resection included, the whole tumour, including greater cornu and part of body of hyoid. Tumour spill occurred during surgery owing to efforts directed to preserve the left superior laryngeal neurovascular bundle. Histopathology revealed a chondrosarcoma Grade I (Fig. 2b). Two weeks later she was started on radiotherapy to the tumour bed. A dose of 60 Gy in 30 fractions was delivered over three months. Review of the patient in March 1991 revealed no evidence of disease.
Case reports Case 1: A 30-year-old woman was seen in October 1988, with a history of a swelling in the front of the neck for three years. On examination, a 5 x 5 cm hard, fixed swelling was seen over the right carotid triangle of the neck with an overlying incisional scar. Indirect laryngoscopy revealed a smooth bulge in the right vallecula. A soft tissue X-ray of the neck showed a soft tissue mass in the upper neck with hyoid bone destruction and attenuation of normal air shadow at epiglottic region. A CT scan neck revealed irregular destruction of the hyoid bone on the right side with mixed density and nodular calcification. There was no significant contrast enhancement (Fig. 1). A wide excision of the tumour mass was performed via a transcervical approach. There was near total destruction of the hyoid bone except for the left greater cornu. The tumour was adherent to the thyrohyoid membrane. Exposure of the right jugular vein revealed no significant lymphadenopathy. The left superior laryngeal neurovascular bundle was preserved. Capsular rupture occurred when the tumour was stripped off the thyro-hyoid membrane. A breach in the vallecular mucosa occurred after total excision which was repaired with three-zero interrupted Vicryl sutures. The patient was fed via a Ryles tube and in four weeks time was able to swallow normally. Histopathology showed a chondrosarcoma grade II (Fig. 2a). Six weeks after surgery she was planned for radiotherapy to the tumour bed. A dose of 50 Gy in 20 fractions was given from December of that year over four weeks. The patient was free of disease during her follow-up in March 1991. Case 2: A 45-year-old female reported to our Institute in August 1989 with a seven-year history of swelling over the left side of the upper neck. On examination, a 3 x 3 cm hard mobile swelling was palpable over the left submandibular region with an incisional scar over the swelling (Fig. 3). Examination of the oral cavity and indirect laryngoscopy were normal. There were no palpable cervical nodes. A soft tissue X-ray of the neck showed a soft tissue swelling in the submandibular area (Fig. 4). Fine needle aspiration cytology of the swelling was suspicious of
FIG. 1 Tumour involving hyoid except for the left greater cornu.
Accepted for publication: 30 October 1991. 273
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FIG. 2a Chondrosarcoma Grade II: cellular tumour with cells showing pleomorphic nuclei. H & E x40.
FIG. 2b atic nuclei. Occasional lacunae contain Chondrosarcoma Grade I: tumour cells are placed in a chondroid matrix with monomorphic hyperchrom two nuclei. H & E x40.
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CLINICAL RECORDS
FIG. 3 Clinical photograph case 2.
Discussion Chondrosarcomas of bone commonly involve the pelvis, femur and the shoulder girdle (Malawer et al., 1989). The hyoid bone is a rare site for primary chondrosarcoma. Literature review reveals one case reported by Finn et al. (1984). The age incidence of chondrosarcoma has remained fairly constant, from the third to sixth decade (Evans et al., 1977) with a male:female ratio of approximately 2:1. (Henderson and Dahlin, 1963). In a review of chondrosarcoma of the head and neck region, an equal sex incidence was reported by Finn et al. (1984). Prognostically, females tended to have a better prognosis than males especially five years or more after treatment (Pritchard et al., 1980). The radiological diagnosis of chondrosarcoma is primarily dependent upon the tumour matrix mineralization and associated bony destruction. The mineralization is reflected radiographically as strips of calcium variously called crescentic, flocculent, stippled or punctate (Wilner, 1982). Calcification when present is pathognomonic for this type of tumour (Neis et al., 1989). CT images do not superimpose adjacent structures and allow calcification in the neck to be distinguished from bone or normal cartilage (Munoz etal.,\ 990). This also helps in defining the origin and extent of the tumour and the best operative approach. The prognosis of chondrosarcoma depends on histopathology, presenting symptoms, site of origin and the adequacy of local treatment. Histologically they are graded from Grade I—III (Evans et al., 1977) and this grading has been correlated with biological behaviour. Reporting on 71 cases from all body sites, Evans et al. (1977) observed that metastasis did not occur in Grade I lesions; they occurred in 10 per cent of Grade II and 71 percent of Grade III lesions. The five-year survival of Grades I, II, III were 90 per cent, 81 per cent and 43 per cent respectively. Chondrosarcoma which present as a mass tend to have a better prognosis than those which present with pain (Kaufman et al., 1977). Both of our patients presented with a mass only.
Surgery has been the mainstay of treatment of chondrosarcoma and complete resectability is an important prognostic factor. Chondrosarcoma arising in extremities tend to fare better than those arising in the trunk and head and neck (Harwood et al., 1980). This was probably related to adequacy of total resection in the extremities. Results from M.D. Anderson Hospital reveal local recurrence in 93 per cent of Grades I and II tumours treated by local excision or curettage, but it was only 18 per cent in those treated by amputation or complete resection (Evans et al., 1977). In chondrosarcoma of the cricoid, total laryngectomy was followed by no local recurrence but a conservative approach led to an 81 per cent local recurrence (Lavertu and Tucker, 1984). Local recurrence can adversely affect survival (Pritchard et al., 1980). Dahlin and Beabout (1971) reported that 10 per cent of low grade chondrosarcomas can be expected to evolve into dedifferentiated sarcomas and inadequate surgical removal was attributed as a cause for this transformation in recurrent tumours. In both of our cases we achieved a total resection of the tumour masses with no compromise in laryngeal function. In case 1, inability to preserve the right neurovascular laryngeal bundle, coupled with sub-total removal of the hyoid bone led to delayed resumption of oral feeds. By contrast, case 2 did not even require post-operative Ryles tube feeding. Therefore, attempts must be made to conserve supraglottic innervation whenever oncological principles are not compromised. The role of radiotherapy in the management of chondrosarcoma as primary or adjuvant modality has been questioned in the surgical literature (Nicolai etal., 1990; Evans et al., 1977). Harward et al. (1980) reported that 50 per cent of cases treated with radical radiotherapy have been found to be in complete remission over 3 to 16 years post-treatment. McNaney et al. (1982) in an analysis of 20 cases, showed a local control rate of 55 per cent over a median period of 30 months with radiotherapy and/or surgery. A five-year actuarial local control rate of 77 per cent has been reported with radiotherapy in chondrosarcoma of base of skull after subtotal surgical resection (Berson etal., 1988). With surgery being the main modality of treatment, the above documents the role of radiotherapy, especially where surgery is not feasible or when post-operative surgical margins are grossly or microscopically inadequate. Both our patients received local radiotherapy to the tumour bed to prevent local recurrence. In head and neck chondrosarcomas two large series reveal a favour-
FIG. 4 Irregular dense opacity, left submandibular region.
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able outcome. Burkey et al. (1990) report a 70 per cent overall survival in 14 patients and Finn et al. (1984) report an 81 per cent survival in 23 patients. These results are promising and support an en bloc surgical resection and post-operative radiotherapy. Both our patients, following surgery and radiotherapy, are disease free at 26 months and 15 months respectively. References Berson, A. M., Castro, J. R., Petti, P., Phillips, T. L., Gauger, G. E., Gutin, P., Collier, J. M, Henderson, S. H., Baken, K. (1988) Charged particle irradiation of chordoma and chondrosarcoma of the base of skull and cervical spine: the Lawrence Berkeley Laboratory experience. International Journal of Radiation Oncology, Biology, Physics, 15: 559-565. Burkey, B. B., Hoffman, H. T., Baker, S. R., Thronton, A. R, McMclatchey, K. D. (1990) Chondrosarcoma of the head and neck. Larynoscope, 100: 1301-1305. Cantrell, R. W., Jahrsdoerfer, R. A., Reibel, J. R, Johns, M. E. (1980) Conservative surgical treatment of chondrosarcoma of the larynx. Annals of Otology, Rhinology and Laryngology, 89: 567-571. Dahlin, D. C, Beabout, J. W. (1971) Dedifferentiation of low grade chondrosarcomas. Cancer, 28: 461^66. Evans, H. L., Ayala, A. G., Romsdahl, M. R. (1977) Prognostic factors in chondrosarcoma of bone. Cancer, 40: 818-831. Finn, D. G., Goepfert, H., Batsakis, J. G. (1984) Chondrosarcoma of the head and neck. Laryngoscope, 94: 1539-1544. Harwood, A. R., Krajbich, J. I., Fornasier, V. L. (1980) Radiotherapy for chondrosarcoma of bone. Cancer, 45: 2769—2777. Henderson, S. H., Dahlin, D. C. (1963) Chondrosarcoma of bone: a study of 288 cases. Journal of Bone and Joint Surgery, 45A: 1450-1458. Kaufman, J. H., Douglass, H. O. Jr., Blake, W., Moore, R., Rao, U. N. M. (1977) The importance of initial presentation and treatment upon the survival of patients with chondrosarcoma. Surgery, Gynaecology and Obstetrics, 145: 357—363.
Key words: Chondrosarcoma; Hyoid bone
Lavertu, P., Tucker, H. N. (1984) Chondrosarcoma of the larynx. Annals of Otology, Rhinology and Laryngology, 93: 452-456. Malawer, M. M, Link, M. P., Donaldson, S. S. (1989) Sarcomas of bone. In Cancer—Principles and Practice of Oncology (DeVita, V. T. Jr., Hellman, S., Rosenberg, S. A., eds.) J. B. Lippincott: Philadelphia, p. 1418-1468. McNaney, D., Lindberg, R. D., Ayala, A. G., Barkley, H. T, Hussey, D. H. (1982) Fifteen-year radiotherapy experience with chondrosarcoma of bone. International Journal of Radiation Oncology, Biology Physics, 8: 187-190. Munoz, A., Penarrocha, L., Gallego, F, Olmedilla, G., Pochbroto, J. (1990) Laryngeal chondrosarcoma, CTfindingsin three patients. American Journal of Roentgenology, 154: 997-998. Neis, P. R., McMohan, M. R, Norris, C. W. (1989) Cartilagenous tumours of the trachea and larynx. Annals of Otology, Rhinology and Laryngology, 98: 31-36. Nicolai, P., Sasaki, C. T, Ferlito, A., Kirchner, J. A. (1990) Laryngeal chondrosarcoma: incidence, pathology, biological behaviour and treatment. Annals of Otology, Rhinology and Laryngology, 99: 515-523. Pritchard, D. J., Lunke, R. J., Taylor, W. R, Dahlin, D. C, Medley, B. E. (1980) Chondrosarcoma: a clinico pathological and statistical analysis. Cancer, 45: 149-157. Wilner, D. (1982) Chondrosarcoma. In Radiology of bone tumours and allied disorders, Vol. 3, W. B. Saunders: Philadelphia, p. 2174. Address for correspondence: V. Kannan, M. D., Lecturer, Department of Radiation Oncology, Kidwai Memorial Institute of Oncology, Hosur Road, Bangalore—560 029, India.
Chondrosarcoma of the hyoid SUHEL HASAN, M.S., V. KANNAN, M.D., ASHOK M. SHENOY, M.S., NANJUNDAPPA, M.S., K. N. NARESH, M.D.
(Bangalore, India)
Abstract Two cases of chondrosarcoma of the hyoid bone are described. They were managed with surgical resection and postoperative radiotherapy. These patients are disease free at 26 months and 15 months respectively.
Introduction Chondrosarcoma is the second most common primary malignant spindle cell tumour of bone (Malawer et al., 1989). Approximately 10-12 per cent arise in head and neck sites where they commonly involve skull base, maxilla and larynx (Cantrell et al., 1980; Burkey et al., 1990). Involvement of hyoid bone is rare. We present two cases of chondrosarcoma of the hyoid, the report illustrates their management and literature review.
a salivary gland tumour with myxoid and calcific material. A wide excision of the tumour mass using a transcervical approach was carried out; this revealed an encapsulated tumour adherent to the left greater cornu of hyoid. Our resection included, the whole tumour, including greater cornu and part of body of hyoid. Tumour spill occurred during surgery owing to efforts directed to preserve the left superior laryngeal neurovascular bundle. Histopathology revealed a chondrosarcoma Grade I (Fig. 2b). Two weeks later she was started on radiotherapy to the tumour bed. A dose of 60 Gy in 30 fractions was delivered over three months. Review of the patient in March 1991 revealed no evidence of disease.
Case reports Case 1: A 30-year-old woman was seen in October 1988, with a history of a swelling in the front of the neck for three years. On examination, a 5 x 5 cm hard, fixed swelling was seen over the right carotid triangle of the neck with an overlying incisional scar. Indirect laryngoscopy revealed a smooth bulge in the right vallecula. A soft tissue X-ray of the neck showed a soft tissue mass in the upper neck with hyoid bone destruction and attenuation of normal air shadow at epiglottic region. A CT scan neck revealed irregular destruction of the hyoid bone on the right side with mixed density and nodular calcification. There was no significant contrast enhancement (Fig. 1). A wide excision of the tumour mass was performed via a transcervical approach. There was near total destruction of the hyoid bone except for the left greater cornu. The tumour was adherent to the thyrohyoid membrane. Exposure of the right jugular vein revealed no significant lymphadenopathy. The left superior laryngeal neurovascular bundle was preserved. Capsular rupture occurred when the tumour was stripped off the thyro-hyoid membrane. A breach in the vallecular mucosa occurred after total excision which was repaired with three-zero interrupted Vicryl sutures. The patient was fed via a Ryles tube and in four weeks time was able to swallow normally. Histopathology showed a chondrosarcoma grade II (Fig. 2a). Six weeks after surgery she was planned for radiotherapy to the tumour bed. A dose of 50 Gy in 20 fractions was given from December of that year over four weeks. The patient was free of disease during her follow-up in March 1991. Case 2: A 45-year-old female reported to our Institute in August 1989 with a seven-year history of swelling over the left side of the upper neck. On examination, a 3 x 3 cm hard mobile swelling was palpable over the left submandibular region with an incisional scar over the swelling (Fig. 3). Examination of the oral cavity and indirect laryngoscopy were normal. There were no palpable cervical nodes. A soft tissue X-ray of the neck showed a soft tissue swelling in the submandibular area (Fig. 4). Fine needle aspiration cytology of the swelling was suspicious of
FIG. 1 Tumour involving hyoid except for the left greater cornu.
Accepted for publication: 30 October 1991. 273
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SUHEL HASAN, V. KANNAN, ASHOK M. SHENOY, NANJUNDAPPA, K. N. NARESH
FIG. 2a Chondrosarcoma Grade II: cellular tumour with cells showing pleomorphic nuclei. H & E x40.
FIG. 2b atic nuclei. Occasional lacunae contain Chondrosarcoma Grade I: tumour cells are placed in a chondroid matrix with monomorphic hyperchrom two nuclei. H & E x40.
275
CLINICAL RECORDS
FIG. 3 Clinical photograph case 2.
Discussion Chondrosarcomas of bone commonly involve the pelvis, femur and the shoulder girdle (Malawer et al., 1989). The hyoid bone is a rare site for primary chondrosarcoma. Literature review reveals one case reported by Finn et al. (1984). The age incidence of chondrosarcoma has remained fairly constant, from the third to sixth decade (Evans et al., 1977) with a male:female ratio of approximately 2:1. (Henderson and Dahlin, 1963). In a review of chondrosarcoma of the head and neck region, an equal sex incidence was reported by Finn et al. (1984). Prognostically, females tended to have a better prognosis than males especially five years or more after treatment (Pritchard et al., 1980). The radiological diagnosis of chondrosarcoma is primarily dependent upon the tumour matrix mineralization and associated bony destruction. The mineralization is reflected radiographically as strips of calcium variously called crescentic, flocculent, stippled or punctate (Wilner, 1982). Calcification when present is pathognomonic for this type of tumour (Neis et al., 1989). CT images do not superimpose adjacent structures and allow calcification in the neck to be distinguished from bone or normal cartilage (Munoz etal.,\ 990). This also helps in defining the origin and extent of the tumour and the best operative approach. The prognosis of chondrosarcoma depends on histopathology, presenting symptoms, site of origin and the adequacy of local treatment. Histologically they are graded from Grade I—III (Evans et al., 1977) and this grading has been correlated with biological behaviour. Reporting on 71 cases from all body sites, Evans et al. (1977) observed that metastasis did not occur in Grade I lesions; they occurred in 10 per cent of Grade II and 71 percent of Grade III lesions. The five-year survival of Grades I, II, III were 90 per cent, 81 per cent and 43 per cent respectively. Chondrosarcoma which present as a mass tend to have a better prognosis than those which present with pain (Kaufman et al., 1977). Both of our patients presented with a mass only.
Surgery has been the mainstay of treatment of chondrosarcoma and complete resectability is an important prognostic factor. Chondrosarcoma arising in extremities tend to fare better than those arising in the trunk and head and neck (Harwood et al., 1980). This was probably related to adequacy of total resection in the extremities. Results from M.D. Anderson Hospital reveal local recurrence in 93 per cent of Grades I and II tumours treated by local excision or curettage, but it was only 18 per cent in those treated by amputation or complete resection (Evans et al., 1977). In chondrosarcoma of the cricoid, total laryngectomy was followed by no local recurrence but a conservative approach led to an 81 per cent local recurrence (Lavertu and Tucker, 1984). Local recurrence can adversely affect survival (Pritchard et al., 1980). Dahlin and Beabout (1971) reported that 10 per cent of low grade chondrosarcomas can be expected to evolve into dedifferentiated sarcomas and inadequate surgical removal was attributed as a cause for this transformation in recurrent tumours. In both of our cases we achieved a total resection of the tumour masses with no compromise in laryngeal function. In case 1, inability to preserve the right neurovascular laryngeal bundle, coupled with sub-total removal of the hyoid bone led to delayed resumption of oral feeds. By contrast, case 2 did not even require post-operative Ryles tube feeding. Therefore, attempts must be made to conserve supraglottic innervation whenever oncological principles are not compromised. The role of radiotherapy in the management of chondrosarcoma as primary or adjuvant modality has been questioned in the surgical literature (Nicolai etal., 1990; Evans et al., 1977). Harward et al. (1980) reported that 50 per cent of cases treated with radical radiotherapy have been found to be in complete remission over 3 to 16 years post-treatment. McNaney et al. (1982) in an analysis of 20 cases, showed a local control rate of 55 per cent over a median period of 30 months with radiotherapy and/or surgery. A five-year actuarial local control rate of 77 per cent has been reported with radiotherapy in chondrosarcoma of base of skull after subtotal surgical resection (Berson etal., 1988). With surgery being the main modality of treatment, the above documents the role of radiotherapy, especially where surgery is not feasible or when post-operative surgical margins are grossly or microscopically inadequate. Both our patients received local radiotherapy to the tumour bed to prevent local recurrence. In head and neck chondrosarcomas two large series reveal a favour-
FIG. 4 Irregular dense opacity, left submandibular region.
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SUHEL HASAN, V. KANNAN, ASHOK M. SHENOY, NANJUNDAPPA, K. N. NARESH
able outcome. Burkey et al. (1990) report a 70 per cent overall survival in 14 patients and Finn et al. (1984) report an 81 per cent survival in 23 patients. These results are promising and support an en bloc surgical resection and post-operative radiotherapy. Both our patients, following surgery and radiotherapy, are disease free at 26 months and 15 months respectively. References Berson, A. M., Castro, J. R., Petti, P., Phillips, T. L., Gauger, G. E., Gutin, P., Collier, J. M, Henderson, S. H., Baken, K. (1988) Charged particle irradiation of chordoma and chondrosarcoma of the base of skull and cervical spine: the Lawrence Berkeley Laboratory experience. International Journal of Radiation Oncology, Biology, Physics, 15: 559-565. Burkey, B. B., Hoffman, H. T., Baker, S. R., Thronton, A. R, McMclatchey, K. D. (1990) Chondrosarcoma of the head and neck. Larynoscope, 100: 1301-1305. Cantrell, R. W., Jahrsdoerfer, R. A., Reibel, J. R, Johns, M. E. (1980) Conservative surgical treatment of chondrosarcoma of the larynx. Annals of Otology, Rhinology and Laryngology, 89: 567-571. Dahlin, D. C, Beabout, J. W. (1971) Dedifferentiation of low grade chondrosarcomas. Cancer, 28: 461^66. Evans, H. L., Ayala, A. G., Romsdahl, M. R. (1977) Prognostic factors in chondrosarcoma of bone. Cancer, 40: 818-831. Finn, D. G., Goepfert, H., Batsakis, J. G. (1984) Chondrosarcoma of the head and neck. Laryngoscope, 94: 1539-1544. Harwood, A. R., Krajbich, J. I., Fornasier, V. L. (1980) Radiotherapy for chondrosarcoma of bone. Cancer, 45: 2769—2777. Henderson, S. H., Dahlin, D. C. (1963) Chondrosarcoma of bone: a study of 288 cases. Journal of Bone and Joint Surgery, 45A: 1450-1458. Kaufman, J. H., Douglass, H. O. Jr., Blake, W., Moore, R., Rao, U. N. M. (1977) The importance of initial presentation and treatment upon the survival of patients with chondrosarcoma. Surgery, Gynaecology and Obstetrics, 145: 357—363.
Key words: Chondrosarcoma; Hyoid bone
Lavertu, P., Tucker, H. N. (1984) Chondrosarcoma of the larynx. Annals of Otology, Rhinology and Laryngology, 93: 452-456. Malawer, M. M, Link, M. P., Donaldson, S. S. (1989) Sarcomas of bone. In Cancer—Principles and Practice of Oncology (DeVita, V. T. Jr., Hellman, S., Rosenberg, S. A., eds.) J. B. Lippincott: Philadelphia, p. 1418-1468. McNaney, D., Lindberg, R. D., Ayala, A. G., Barkley, H. T, Hussey, D. H. (1982) Fifteen-year radiotherapy experience with chondrosarcoma of bone. International Journal of Radiation Oncology, Biology Physics, 8: 187-190. Munoz, A., Penarrocha, L., Gallego, F, Olmedilla, G., Pochbroto, J. (1990) Laryngeal chondrosarcoma, CTfindingsin three patients. American Journal of Roentgenology, 154: 997-998. Neis, P. R., McMohan, M. R, Norris, C. W. (1989) Cartilagenous tumours of the trachea and larynx. Annals of Otology, Rhinology and Laryngology, 98: 31-36. Nicolai, P., Sasaki, C. T, Ferlito, A., Kirchner, J. A. (1990) Laryngeal chondrosarcoma: incidence, pathology, biological behaviour and treatment. Annals of Otology, Rhinology and Laryngology, 99: 515-523. Pritchard, D. J., Lunke, R. J., Taylor, W. R, Dahlin, D. C, Medley, B. E. (1980) Chondrosarcoma: a clinico pathological and statistical analysis. Cancer, 45: 149-157. Wilner, D. (1982) Chondrosarcoma. In Radiology of bone tumours and allied disorders, Vol. 3, W. B. Saunders: Philadelphia, p. 2174. Address for correspondence: V. Kannan, M. D., Lecturer, Department of Radiation Oncology, Kidwai Memorial Institute of Oncology, Hosur Road, Bangalore—560 029, India.
