The Journal of Laryngology and Otology December 1992, Vol. 106, pp. 1081-1083
A rare congenital intranasal polyp: mesenchymal chondrosarcoma of the nasal region N. J. R O L A N D , * M Y A T M O N K H I N E , * * R. C L A R K E , * D. V A N V E L Z E N * *
Abstract A mesenchymal chondrosarcoma of the nasal region in a neonate is described. Problems of histological interpretation and management are discussed.
Introduction
from multiple sites gave several fragments of tissue, in total measuring 30 x 1 0 x 4 mm. Histologically, a highly cellular tissue composed of spindle to round cells with small oval to oblong nuclei was present. Irregularly scattered cartilaginous tissue was present, merging with the undifferentiated cellular areas. The cartilage contained binucleate cells and very occasional quadrinucleate cells. There was variation in nuclear size with several plump nuclei. Nucleolar structures were recognizable in some of the nuclei. No giant cells or herring bone pattern was seen. Vascular channels were prominent in some areas, with formation of a haemangiopericytomalike pattern (Fig. 3). Reticulin fibre arrangement was mainly around small clusters of cells and occasionally around single cells. On immunohistochemical analysis there was a strong intracellularpositivity of the cartilaginous tissue, as well as some of the spindle to round cells adjacent to the cartilage, to monoclonal antibodies against the S 100 protein (Fig. 4).
Mesenchymal chondrosarcoma is a rare and distinctive cartilage neoplasm first described in 1959 by Lichtenstein and Bernstein. Relatively few well documented examples of this highly malignant type of chondrosarcoma have been reported. Compared with conventional chondrosarcoma, the mesenchymal variant is commoner in younger patients (Dabska and Huvos, 1983). Most patients are in their second or third decade of life when the diagnosis is made, with ages ranging from 5-72 years (Salvador et al., 1971). It may arise in both bone and soft tissue (Dowling, 1964; Goldman, 1967; Salvador et al., 1971; Guccion et al, 1973; Huvos et al., 1983). Skeletal distribution is wide; the femur and ribs are often involved but about 20 per cent occur in the craniofacial skeleton, a relatively rare site for ordinary chondrosarcoma (Reid and Catto, 1987). The principal locations of soft tissue mesenchymal chondrosarcomas are in the head and neck, especially the orbit and meninges (Scheithauer and Rubenstein, 1978). The reported case of a nasal mesenchymal chondrosarcoma in a neonate is distinguished by the uncommon site of the tumour and uniquely low age of the patient. The histological diagnosis and treatment are discussed.
A diagnosis of mesenchymal chondrosarcoma was made on the basis of the combination of the undifferentiated round to spindle cell component with moderately well differentiated cartilage. A second biopsy was taken at multiple sites to exclude a midline teratoma of mixed histological type. All of the blocks on the second set of biopsies confirmed the original appearance. To confirm the diagnosis, specimens of tumour were sent for a second opinion to the Royal Manchester Children' s Hospital Department of Pathology, who agreed with the original histological interpretation. With a working diagnosis of a mesenchymal chondrosarcoma, chemotherapy was commenced. This regime was cyclophosphamide, adriamycin and vincristine. It produced no improvement in his tumour which increased slightly in size. There was slight displacement and swelling of the right orbit and he developed an obstructed nasolacrimal duct. Apart from his tumour, the patient remained reasonably well, with no evidence of metastatic disease. In view of the progression of the disease, his chemotherapy was revised and alternative therapy using carboplatin was started. The tumour did not respond. This was confirmed by repeat CT scan which showed an increase in size of the tumour. It was then decided to attempt surgical resection of the tumour. Thirty weeks after his birth the patient underwent a craniofacial resection of the nasal tumour. A large bi-coronal skull flap and mid-facial split to the upper lip was used to expose the cranial vault and mid face. An exophytic tumour six cms in diameter was occupying the whole of the right nasal cavity with extension from the lateral nasal wall up to the right cribriform plate. A frontal orbital bar with a free bi-frontal craniotomy flap was taken and the nasion was dissected. The tumour was growing up through the nasion into the dural base. A large fragment of dura was excised
Case report An infant aged one day was transferred to the Neonatal Surgical Unit of Alder Hey Childrens Hospital because of cyanotic episodes and upper airway obstruction. He was born to a 33-year-old mother by an elective lower segment caesarean section for polyhydramnios and maternal hypertension. He was 38 weeks gestation and had poor Apgar's with cyanotic episodes immediately after birth. The respiratory distress was eased by insertion of an oral airway and it was in this condition that he arrived at Alder Hey. Initial examination revealed a right-sided nasal polyp and splaying of the right lateral nasal wall with a facial swelling extending to just below the right orbit (Fig. 1). CT scan of the head and neck (Fig. 2) showed a partly calcined locally invasive tumour occupying the right nasal cavity and nasopharynx. Isotope bone scan uptake was compatible with local infiltration; no other lesion was demonstrated. Liver function tests, chest x-ray, 24 h urinary VMA' s. HCG, AFP and bone marrow were all normal. He had normal arterial gases on 40 per cent oxygen. An ultrasound of his head and urinary tract were both normal. CSF was clear and blood cultures were negative. Routine haematology and biochemistry were normal. There was no abnormality in the heart and echocardiogram was normal. The tumour was examined under a general anaesthetic. It appeared to arise from the lateral nasal wall extending to fill the nasal cavity and nasopharynx. Intranasal biopsy of the tumour
From the Departments of Otorhinolaryngology * and Fetal and Infant Pathology**, Royal Liverpool Childrens Hospital, Alder Hey, Liverpool, UK. Accepted for publication: 23 May 1992. 1081
1082
N. J. ROLAND, M. MON KHINE, R. CLARK, D. VAN VELZEN
FIG. 2 CT scan showing tumour occupying the right nasal cavity and nasopharynx. Note the calcification within the tumour.
FIG. 1 The child at presentation. Note the right nasal polyp and swelling at the right side of the face.
and infra-frontal lobes were exposed back to the anterior clivus, to the cribriform plate and pterygoids. A dural xeroderm graft was placed over the large dural defect. The floor of the anterior fossa and mid-face were reconstructed with a cranial vault graft. The cavatron was used to dissect the tumour and the wound was closed in layers. The procedure took eight hours in all. The histology of the excised tissue revealed tumour. The specimen was fragmented with the largest tissue piece measuring 3 x 2 x 1.5 cm and had a friable red brown appearance. It was similar to the original biopsy confirming a mesenchymal chondrosarcoma. As the tumour was removed piecemeal it was not possible to assess the resection margin. Post-operatively he had a cereborspinal fluid leak and was electively ventilated on the Intensive Care Unit. His recovery from there on was unremarkable. On review in clinic his wounds have healed, there is no CSF leak, and there is no obstruction to the nasal airway. Discussion A congenital nasal swelling occurs approximately once in every 20,000-40,000 live births and all intranasal masses in children, especially unilateral, should be treated with suspicion and circumspection (Cinnamond, 1987). In the reported case, a diagnosis of mesenchymal chondrosarcoma was certainly not anticipated. Intranasal cartilaginous tumours are rare (Murthy et al., 1991) and to our knowledge this is thefirstreport of such a tumour in a neonate. The mesenchymal variety of chondrosarcoma is
even more unusual and raises some important points in diagnosis and management. There are several clinical and pathological features which set the mesenchymal variant apart from a conventional chondrosarcoma. The importance of distinguishing between the two lies in the exceptionally poor prognosis of mesenchymal chondrosarcoma (Harwood et al., 1981). The course of mesenchymal chondrosarcoma appears to be relentless and the grave prognosis contrasts with the more favourable outlook of the conventional chondrosarcoma (Fu and Perzin, 1974; Vernerera/., 1984). Light microscopic descriptions have been well detailed in the literature (Dahlin and Henderson, 1962; Salvador et al., 1971; Guccion etal, 1973; Bloch et al., 1979) as have electron micro-
FIG. 3 Irregular islands of well differentiated cartilage with ill-defined borders, mixed with a mass of round to spindle cells. H&E xlOO.
1083
CLINICAL RECORDS
Acknowledgements The authors wish to thank Dr J. Martin, Mr S. D. Singh, Mr Cook and Mr P. May (Liverpool) for permission to report a case under their joint care. We also wish to thank Dr A. Kelsey (Manchester) for the second opinion on the histological interpretation.
FIG. 4 Chondrocytes in the cartilage as well as several of immediately adjacent spindle cells show cytoplasmic immunoreactive staining for SI00 protein. SI00 protein immunoperoxidase x200.
scopic studies (Steiner et al., 1973; Fu and Kay, 1974; Bloch et al, 1979). The histological diagnosis is established by thefindingof a richly cellular neoplasm composed of undifferentiated mesenchymal cells in which islands of relatively well differentiated benign appearing cartilage is found. The transition between the two components may be abrupt with sharply defined islands of cartilage, but often it is imperceptible. The undifferentiated mesenchymal cells vary from round and small cells to spindle shaped cells. They have scanty cytoplasm, round or ovoid hyperchromatic nuclei with little pleomorphism, and usually few mitoses. The cells may form solid sheets, be broken up by branching strands of collagen into alveolar clusters or proliferate around vascular spaces to give a haemangiopericytomatous pattern. The histological differential diagnosis in soft tissues is with a malignant haemangiopericytoma, soft tissue Ewing's sarcoma, malignant lymphoma, synovial sarcoma and embryonal rhabdomyosarcoma; The identification of the cartilage component and the undifferentiated cells are the critical diagnostic features. We also confirmed the presence of cartilage by using the immunohistochemical marker S-100. S-100 is an acidic calcium binding protein which was initially isolated from neuroectodermal tissue and subsequently cartilage cells, both normal and neoplastic. It has been suggested that it may be a useful marker of tumours of cartilaginous derivation (Weiss and Dorfman, 1986). Attempting to assess the results overall it seems that the 10 year survival is probably less than 25 per cent (Reid and Catto, 1987). However, some authors have previously commented on the limited value in assessing overall treatment results (Salvador et al, 1971; Huvos et al., 1983). In general, it has been found that local recurrences characterized the clinical course preceding metastatic dissemination, emphasizing the significance of radical local therapy (Dahlin and Henderson, 1962; Dowling, 1964; Salvador e/a/.,1971;PittmanandKeller, 1974;Huvose/a/.,1983).Inview of the young age of our patient and the inherent foreseeable technical difficulties in resection, he was initially treated with chemotherapy alone. It has been suggested (though on experience with relatively few patients) that these tumours, and particularly their undifferentiated small cell component, may respond to irradiation and that this should be tried in combination with multi-agent chemotherapy at sites where the tumour is unresectable or inadequately excised. Adjuvant chemotherapy is advised in patients treated by radical surgical excision (Harwood et al., 1981; Huvos et al., 1983). The tumour in the reported case proved to be very chemoresistant and we would re-emphasize the view that wide surgical excision is the preferred treatment.
References Bloch, D. M., Bragoli, A. J., Collins, D. N., Batsakis, J. G. (1979) Mesenchymal chondrosarcoma of the head and neck. Journal of Laryngology and Otology, 93:405^412. Cinnamond, M. J. (1987) Congenital anomalies of the nose. In ScottBrown's Otolaryngology, 5th edition, Volume 6 (Kerr, A. G. and Evans, J. N. G., eds.) 222-224. Dabska, M., Huvos, A. G. (1983) Mesenchymal chondrosarcoma in the young: A clinicopathologic study with an explanation of histogenesis. Virchows Archivfur Pathologische Anatomie undPhysiologie undfiir Klinische Medizin, 399: 89-104. Dahlin, D. C., Henderson, E. D. (1962) Mesenchymal chondrosarcoma: further observations on a new entity. Cancer, 15:410-417. Dowling, E. A. (1964) Mesenchymal chondrosarcoma. Journal of Bone and Joint Surgery, 46: 747-754. Fu, Y. S., Perzin, K. H. (1974) Non epithelial tumours of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. Cancer, 34:453^63. Fu, Y. S., Kay, S. A. (1974) A comparative ultrastructural study of mesenchymal chondrosarcoma and myxoid chondrosarcoma. Cancer, 33: 1531-1542. Goldman, R. L. (1967) Mesenchymal chondrosarcoma a rare malignant chondroid tumour usually primary in bone: report of a case arising in extra skeletal soft tissue. Cancer, 20: 1494—1498. Guccion, J. G., Font, R. L., Enzinger, F. M., Zimmerman, L. E. (1973) Extraskeletal mesenchymal chondrosarcoma. Archives of Pathology, 95: 336-340. Harwood, A. R., Krajbich, J. I., Fornasier, V. L. (1981) Mesenchymal chondrosarcoma. A report of 17 cases. Clinical Orthopaedics and Related Research, 158: 144-148. Huvos, A. G., Rosen, G., Dabska, M., Marcove, R. C. (1983) Mesenchymal chondrosarcoma. A clinicopathologic analysis of 35 patients with an emphasis on treatment. Cancer, 51: 1230-1237. Lichtenstein, L., Bernstein, D. (1959) Unusual benign and malignant chondroid tumours of bone. Cancer, 12: 1142-1157. Murthy, D. P., Gupta, A. C, Sen Gupta, S. K., Duttar, T. K., Pulota, M. L. (1991) Nasal cartilaginous tumour. Journal ofLaryngology and Otology, 105:670-672. Pittman, M. R., Keller, E. E. (1974) Mesenchymal chondrosarcoma: report of a case. Journal of Oral Surgery, 32: 443^447. Reid, R. P., Catto, M. E. (1987) In Recent Advances in Histopathology, Volume 13, (Anthony, P. P., MacSween, R. N. M., Eds) Chapter 5, Churchill Livingstone, London. Salvador, A. G., Beabout, J. W, Dahlin, D. C. (1971) Mesenchymal chondrosarcoma observation on 30 new cases. Cancer, 28: 605-615. Scheithauer, B. W., Rubenstein, L. J. (1978) Meningeal mesenchymal chondrosarcoma. Report of 8 cases and review of the literature. Cancer, 42:2744-2752. Steiner, G. C, Mirra, J. M., Bullough, P. G. (1973) Mesenchymal chondrosarcoma: A study of the ultrastructure. Cancer, 32: 926-939. Verner, J., Rice, D. H., Newman, A. N. (1984) Osteosarcoma and chondrosarcoma of the head and neck. Laryngoscope, 94: 240-242. Weiss, A. P., Dorfman, H. D. (1986) S-100 protein in human cartilage lesions. Journal ofBone and Joint Surgery, Vol 68A:4: 521-526. Address for correspondence: N. J. Roland, Department of Otorhinolaryngology, Royal Liverpool University Hospital, Prescot Street, Liverpool.
Key words: Nasal neoplasm; Mesenchymal chondrosarcoma
A rare congenital intranasal polyp: mesenchymal chondrosarcoma of the nasal region N. J. R O L A N D , * M Y A T M O N K H I N E , * * R. C L A R K E , * D. V A N V E L Z E N * *
Abstract A mesenchymal chondrosarcoma of the nasal region in a neonate is described. Problems of histological interpretation and management are discussed.
Introduction
from multiple sites gave several fragments of tissue, in total measuring 30 x 1 0 x 4 mm. Histologically, a highly cellular tissue composed of spindle to round cells with small oval to oblong nuclei was present. Irregularly scattered cartilaginous tissue was present, merging with the undifferentiated cellular areas. The cartilage contained binucleate cells and very occasional quadrinucleate cells. There was variation in nuclear size with several plump nuclei. Nucleolar structures were recognizable in some of the nuclei. No giant cells or herring bone pattern was seen. Vascular channels were prominent in some areas, with formation of a haemangiopericytomalike pattern (Fig. 3). Reticulin fibre arrangement was mainly around small clusters of cells and occasionally around single cells. On immunohistochemical analysis there was a strong intracellularpositivity of the cartilaginous tissue, as well as some of the spindle to round cells adjacent to the cartilage, to monoclonal antibodies against the S 100 protein (Fig. 4).
Mesenchymal chondrosarcoma is a rare and distinctive cartilage neoplasm first described in 1959 by Lichtenstein and Bernstein. Relatively few well documented examples of this highly malignant type of chondrosarcoma have been reported. Compared with conventional chondrosarcoma, the mesenchymal variant is commoner in younger patients (Dabska and Huvos, 1983). Most patients are in their second or third decade of life when the diagnosis is made, with ages ranging from 5-72 years (Salvador et al., 1971). It may arise in both bone and soft tissue (Dowling, 1964; Goldman, 1967; Salvador et al., 1971; Guccion et al, 1973; Huvos et al., 1983). Skeletal distribution is wide; the femur and ribs are often involved but about 20 per cent occur in the craniofacial skeleton, a relatively rare site for ordinary chondrosarcoma (Reid and Catto, 1987). The principal locations of soft tissue mesenchymal chondrosarcomas are in the head and neck, especially the orbit and meninges (Scheithauer and Rubenstein, 1978). The reported case of a nasal mesenchymal chondrosarcoma in a neonate is distinguished by the uncommon site of the tumour and uniquely low age of the patient. The histological diagnosis and treatment are discussed.
A diagnosis of mesenchymal chondrosarcoma was made on the basis of the combination of the undifferentiated round to spindle cell component with moderately well differentiated cartilage. A second biopsy was taken at multiple sites to exclude a midline teratoma of mixed histological type. All of the blocks on the second set of biopsies confirmed the original appearance. To confirm the diagnosis, specimens of tumour were sent for a second opinion to the Royal Manchester Children' s Hospital Department of Pathology, who agreed with the original histological interpretation. With a working diagnosis of a mesenchymal chondrosarcoma, chemotherapy was commenced. This regime was cyclophosphamide, adriamycin and vincristine. It produced no improvement in his tumour which increased slightly in size. There was slight displacement and swelling of the right orbit and he developed an obstructed nasolacrimal duct. Apart from his tumour, the patient remained reasonably well, with no evidence of metastatic disease. In view of the progression of the disease, his chemotherapy was revised and alternative therapy using carboplatin was started. The tumour did not respond. This was confirmed by repeat CT scan which showed an increase in size of the tumour. It was then decided to attempt surgical resection of the tumour. Thirty weeks after his birth the patient underwent a craniofacial resection of the nasal tumour. A large bi-coronal skull flap and mid-facial split to the upper lip was used to expose the cranial vault and mid face. An exophytic tumour six cms in diameter was occupying the whole of the right nasal cavity with extension from the lateral nasal wall up to the right cribriform plate. A frontal orbital bar with a free bi-frontal craniotomy flap was taken and the nasion was dissected. The tumour was growing up through the nasion into the dural base. A large fragment of dura was excised
Case report An infant aged one day was transferred to the Neonatal Surgical Unit of Alder Hey Childrens Hospital because of cyanotic episodes and upper airway obstruction. He was born to a 33-year-old mother by an elective lower segment caesarean section for polyhydramnios and maternal hypertension. He was 38 weeks gestation and had poor Apgar's with cyanotic episodes immediately after birth. The respiratory distress was eased by insertion of an oral airway and it was in this condition that he arrived at Alder Hey. Initial examination revealed a right-sided nasal polyp and splaying of the right lateral nasal wall with a facial swelling extending to just below the right orbit (Fig. 1). CT scan of the head and neck (Fig. 2) showed a partly calcined locally invasive tumour occupying the right nasal cavity and nasopharynx. Isotope bone scan uptake was compatible with local infiltration; no other lesion was demonstrated. Liver function tests, chest x-ray, 24 h urinary VMA' s. HCG, AFP and bone marrow were all normal. He had normal arterial gases on 40 per cent oxygen. An ultrasound of his head and urinary tract were both normal. CSF was clear and blood cultures were negative. Routine haematology and biochemistry were normal. There was no abnormality in the heart and echocardiogram was normal. The tumour was examined under a general anaesthetic. It appeared to arise from the lateral nasal wall extending to fill the nasal cavity and nasopharynx. Intranasal biopsy of the tumour
From the Departments of Otorhinolaryngology * and Fetal and Infant Pathology**, Royal Liverpool Childrens Hospital, Alder Hey, Liverpool, UK. Accepted for publication: 23 May 1992. 1081
1082
N. J. ROLAND, M. MON KHINE, R. CLARK, D. VAN VELZEN
FIG. 2 CT scan showing tumour occupying the right nasal cavity and nasopharynx. Note the calcification within the tumour.
FIG. 1 The child at presentation. Note the right nasal polyp and swelling at the right side of the face.
and infra-frontal lobes were exposed back to the anterior clivus, to the cribriform plate and pterygoids. A dural xeroderm graft was placed over the large dural defect. The floor of the anterior fossa and mid-face were reconstructed with a cranial vault graft. The cavatron was used to dissect the tumour and the wound was closed in layers. The procedure took eight hours in all. The histology of the excised tissue revealed tumour. The specimen was fragmented with the largest tissue piece measuring 3 x 2 x 1.5 cm and had a friable red brown appearance. It was similar to the original biopsy confirming a mesenchymal chondrosarcoma. As the tumour was removed piecemeal it was not possible to assess the resection margin. Post-operatively he had a cereborspinal fluid leak and was electively ventilated on the Intensive Care Unit. His recovery from there on was unremarkable. On review in clinic his wounds have healed, there is no CSF leak, and there is no obstruction to the nasal airway. Discussion A congenital nasal swelling occurs approximately once in every 20,000-40,000 live births and all intranasal masses in children, especially unilateral, should be treated with suspicion and circumspection (Cinnamond, 1987). In the reported case, a diagnosis of mesenchymal chondrosarcoma was certainly not anticipated. Intranasal cartilaginous tumours are rare (Murthy et al., 1991) and to our knowledge this is thefirstreport of such a tumour in a neonate. The mesenchymal variety of chondrosarcoma is
even more unusual and raises some important points in diagnosis and management. There are several clinical and pathological features which set the mesenchymal variant apart from a conventional chondrosarcoma. The importance of distinguishing between the two lies in the exceptionally poor prognosis of mesenchymal chondrosarcoma (Harwood et al., 1981). The course of mesenchymal chondrosarcoma appears to be relentless and the grave prognosis contrasts with the more favourable outlook of the conventional chondrosarcoma (Fu and Perzin, 1974; Vernerera/., 1984). Light microscopic descriptions have been well detailed in the literature (Dahlin and Henderson, 1962; Salvador et al., 1971; Guccion etal, 1973; Bloch et al., 1979) as have electron micro-
FIG. 3 Irregular islands of well differentiated cartilage with ill-defined borders, mixed with a mass of round to spindle cells. H&E xlOO.
1083
CLINICAL RECORDS
Acknowledgements The authors wish to thank Dr J. Martin, Mr S. D. Singh, Mr Cook and Mr P. May (Liverpool) for permission to report a case under their joint care. We also wish to thank Dr A. Kelsey (Manchester) for the second opinion on the histological interpretation.
FIG. 4 Chondrocytes in the cartilage as well as several of immediately adjacent spindle cells show cytoplasmic immunoreactive staining for SI00 protein. SI00 protein immunoperoxidase x200.
scopic studies (Steiner et al., 1973; Fu and Kay, 1974; Bloch et al, 1979). The histological diagnosis is established by thefindingof a richly cellular neoplasm composed of undifferentiated mesenchymal cells in which islands of relatively well differentiated benign appearing cartilage is found. The transition between the two components may be abrupt with sharply defined islands of cartilage, but often it is imperceptible. The undifferentiated mesenchymal cells vary from round and small cells to spindle shaped cells. They have scanty cytoplasm, round or ovoid hyperchromatic nuclei with little pleomorphism, and usually few mitoses. The cells may form solid sheets, be broken up by branching strands of collagen into alveolar clusters or proliferate around vascular spaces to give a haemangiopericytomatous pattern. The histological differential diagnosis in soft tissues is with a malignant haemangiopericytoma, soft tissue Ewing's sarcoma, malignant lymphoma, synovial sarcoma and embryonal rhabdomyosarcoma; The identification of the cartilage component and the undifferentiated cells are the critical diagnostic features. We also confirmed the presence of cartilage by using the immunohistochemical marker S-100. S-100 is an acidic calcium binding protein which was initially isolated from neuroectodermal tissue and subsequently cartilage cells, both normal and neoplastic. It has been suggested that it may be a useful marker of tumours of cartilaginous derivation (Weiss and Dorfman, 1986). Attempting to assess the results overall it seems that the 10 year survival is probably less than 25 per cent (Reid and Catto, 1987). However, some authors have previously commented on the limited value in assessing overall treatment results (Salvador et al, 1971; Huvos et al., 1983). In general, it has been found that local recurrences characterized the clinical course preceding metastatic dissemination, emphasizing the significance of radical local therapy (Dahlin and Henderson, 1962; Dowling, 1964; Salvador e/a/.,1971;PittmanandKeller, 1974;Huvose/a/.,1983).Inview of the young age of our patient and the inherent foreseeable technical difficulties in resection, he was initially treated with chemotherapy alone. It has been suggested (though on experience with relatively few patients) that these tumours, and particularly their undifferentiated small cell component, may respond to irradiation and that this should be tried in combination with multi-agent chemotherapy at sites where the tumour is unresectable or inadequately excised. Adjuvant chemotherapy is advised in patients treated by radical surgical excision (Harwood et al., 1981; Huvos et al., 1983). The tumour in the reported case proved to be very chemoresistant and we would re-emphasize the view that wide surgical excision is the preferred treatment.
References Bloch, D. M., Bragoli, A. J., Collins, D. N., Batsakis, J. G. (1979) Mesenchymal chondrosarcoma of the head and neck. Journal of Laryngology and Otology, 93:405^412. Cinnamond, M. J. (1987) Congenital anomalies of the nose. In ScottBrown's Otolaryngology, 5th edition, Volume 6 (Kerr, A. G. and Evans, J. N. G., eds.) 222-224. Dabska, M., Huvos, A. G. (1983) Mesenchymal chondrosarcoma in the young: A clinicopathologic study with an explanation of histogenesis. Virchows Archivfur Pathologische Anatomie undPhysiologie undfiir Klinische Medizin, 399: 89-104. Dahlin, D. C., Henderson, E. D. (1962) Mesenchymal chondrosarcoma: further observations on a new entity. Cancer, 15:410-417. Dowling, E. A. (1964) Mesenchymal chondrosarcoma. Journal of Bone and Joint Surgery, 46: 747-754. Fu, Y. S., Perzin, K. H. (1974) Non epithelial tumours of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. Cancer, 34:453^63. Fu, Y. S., Kay, S. A. (1974) A comparative ultrastructural study of mesenchymal chondrosarcoma and myxoid chondrosarcoma. Cancer, 33: 1531-1542. Goldman, R. L. (1967) Mesenchymal chondrosarcoma a rare malignant chondroid tumour usually primary in bone: report of a case arising in extra skeletal soft tissue. Cancer, 20: 1494—1498. Guccion, J. G., Font, R. L., Enzinger, F. M., Zimmerman, L. E. (1973) Extraskeletal mesenchymal chondrosarcoma. Archives of Pathology, 95: 336-340. Harwood, A. R., Krajbich, J. I., Fornasier, V. L. (1981) Mesenchymal chondrosarcoma. A report of 17 cases. Clinical Orthopaedics and Related Research, 158: 144-148. Huvos, A. G., Rosen, G., Dabska, M., Marcove, R. C. (1983) Mesenchymal chondrosarcoma. A clinicopathologic analysis of 35 patients with an emphasis on treatment. Cancer, 51: 1230-1237. Lichtenstein, L., Bernstein, D. (1959) Unusual benign and malignant chondroid tumours of bone. Cancer, 12: 1142-1157. Murthy, D. P., Gupta, A. C, Sen Gupta, S. K., Duttar, T. K., Pulota, M. L. (1991) Nasal cartilaginous tumour. Journal ofLaryngology and Otology, 105:670-672. Pittman, M. R., Keller, E. E. (1974) Mesenchymal chondrosarcoma: report of a case. Journal of Oral Surgery, 32: 443^447. Reid, R. P., Catto, M. E. (1987) In Recent Advances in Histopathology, Volume 13, (Anthony, P. P., MacSween, R. N. M., Eds) Chapter 5, Churchill Livingstone, London. Salvador, A. G., Beabout, J. W, Dahlin, D. C. (1971) Mesenchymal chondrosarcoma observation on 30 new cases. Cancer, 28: 605-615. Scheithauer, B. W., Rubenstein, L. J. (1978) Meningeal mesenchymal chondrosarcoma. Report of 8 cases and review of the literature. Cancer, 42:2744-2752. Steiner, G. C, Mirra, J. M., Bullough, P. G. (1973) Mesenchymal chondrosarcoma: A study of the ultrastructure. Cancer, 32: 926-939. Verner, J., Rice, D. H., Newman, A. N. (1984) Osteosarcoma and chondrosarcoma of the head and neck. Laryngoscope, 94: 240-242. Weiss, A. P., Dorfman, H. D. (1986) S-100 protein in human cartilage lesions. Journal ofBone and Joint Surgery, Vol 68A:4: 521-526. Address for correspondence: N. J. Roland, Department of Otorhinolaryngology, Royal Liverpool University Hospital, Prescot Street, Liverpool.
Key words: Nasal neoplasm; Mesenchymal chondrosarcoma
