Chondrosarcoma of the larynx By A. COYAS, O. ANASTASSIADES, M. TZAGARAKJS and G. KONTOGEORGOS
(Athens) CARTILAGINOUS tumours of the larynx are uncommon and most of the reported cases are chondromas. From all cases presented to date only 37 have been chondrosarcomas (Chambers and Friedei, 1976). The first cartilaginous tumour was described by Mensinger in 1822 and nearly 160 cases of such tumours have been published since then. We felt it interesting to report our case because of the rarity of chondrosarcomas of the larynx and because of the unusual size of our tumour.
Case report Male patient, K.K., aged 76, was admitted to the ENT Department of the State General Hospital of Athens, complaining of hoarseness and progressive difficulty in breathing for the last two months. Dyspnoea had increased during the last five days and the patient had developed acute upper respiratory obstruction for which an emergency tracheostomy was performed on admittance. The laryngeal cavity was almost completely obstructed by a subglottic tumour. On direct laryngoscopy the tumour was found to be quite extensive, with a smooth surface, located on the left side and probably originating from the cricoid cartilage. The vocal cords were normal but almost fixed, with a small rima glottidis and firm arytenoid area. No palpable cervical lymph nodes were found. A total laryngectomy was performed ten days after the tracheostomy, with excision of the first tracheal ring, The final pathology report was as follows: 'The larynx 5-5x4-8x3 cm. in dimensions, showed marked oedema of the epiglottis, the aryepiglottic folds and the pharyngeal wall. The vocal cords and the region above them were intact. The subglottic area was nearly obstructed by a tumour 3-5 cm. in diameter, which originated from the left lateral wall and extended towards the laryngeal cavity. The tumour was also occupying a small part of the anterior and posterior fossa extending a short distance along the right lateral wall below the glottis. The surface of the tumour was ulcerated because of the former biopsy (Fig. 1). In cross section the tumour tissue was hard or elastic in consistency and whitish or brown in colour. Microscopically the tumour consisted of immature cartilaginous tissue separated in lobules by thin connective tissue septa. The neoplastic chondroid cells were found in small groups or, exceptionally, singly in the chondroid cavities. The nuclei were large and dense with irregularities in shape and size and showed a moderate number of mitoses. The ground substance was generally slight because of the great cellularity of the tumour. The tumour cells infiltrated the arytenoid cartilage and penetrated the underlying muscles to a small depth.' The histologic diagnosis was: 'Chondrosarcoma of the larynx with infiltration of the underlying tissues. Lymph nodes were not found.' (Fig. 2.) 529
530
A. COYAS et
al.
Discussion Although the aetiology of cartilaginous tumours of the larynx is unknown, some suggestions have been put forward by Moore (1925): — chondroplastic predisposition of bronchial tissue to chronic inflammation; — abnormalities in ossification. As far as sex and age incidence are concerned, all authors maintain that these tumours occur more frequently in males than females and in middle age. Also, a few cases have been reported at the age of 15 as well as 91 (Barsocchini and McCoy, 1968). The site of these tumours seems to be predominantly the cricoid cartilage, at a rate of 70 per cent (De, 1972), although other cartilaginous structures of the larynx may be involved, such as the thyroid, the epiglottis and the arytenoids. The diagnosis is easily made histologically, but a suggestion of cartilaginous tumour should be clinically based on the long duration of the symptoms in relation to the laiyngoscopic findings. The most common and characteristic symptoms of the lesion, which are of course related to the location of the tumour, are hoarseness and progressive difficulty in breathing and swallowing. Indirect laryngoscopy is very important for establishment of the diagnosis and reveals the site and the size of the tumour, which is usually subglottically and submucosally located. Microlaryngoscopy gives definitely more details about the exact extent of the tumour, whether it is sessile or not, and also whether other cartilaginous structures are involved. The treatment of chondrosarcomas of the larynx is definitely surgical and all authors agree that radiotherapy has little effect. The high rate of local recurrence does not seem to favour conservative surgical procedures, although several
FIG. 1 A large neoplastic tumour at the left subglottic area extending to the posterior fossa and the lateral right wall.
CLINICAL RECORDS
531
FIG. 2 Histology of the chondrosarcomatous tissue. Note the high cellularity and the marked atypia of the neoplastic chondroid cells.
authors (Barsocchini and McCoy, 1968) agree on a complex surgical scheme which is a combination of tracheostomy, laryngofissure and excision of the tumour with preservation of the laryngeal skeleton. An exception may be noted in our decision as to the extent of the operation and this is the age of the patiens and the size of the tumour. Small and sessile chondiosarcomas in young patientt may indicate a conservative surgical operation as a first stage, following up the patient's condition very closely. Adenopathy and metastasis, although considered extremely unusual, must also be closely followed. Summary
We report one case of chondrosarcoma of the larynx, which is considered to be rather uncommon. Clinical signs and symptoms have been noted, as well as some suggestions on the aetiology, incidence and site of cartliaginous tumours of the larynx. REFERENCES BARSOCCHINI, M. L. and McCoy, G. (1968) Annals of Otology, Rhinology and Laryngology, 77, 146. CHAMBERS, G. R. and FRIEDEL, W. (1976) The Laryngoscope, 86, 713.
DE, R. P. (1972) 'Chondrosarcoma of the larynx', Journal of Laryngology and Otology, 86, 1261. MOORE, J. (1925) Journal of Laryngology and Otology, 40:9, 84, 145. A. Coyas, 20 Kanari Street, Athens 138, Greece.
(Athens) CARTILAGINOUS tumours of the larynx are uncommon and most of the reported cases are chondromas. From all cases presented to date only 37 have been chondrosarcomas (Chambers and Friedei, 1976). The first cartilaginous tumour was described by Mensinger in 1822 and nearly 160 cases of such tumours have been published since then. We felt it interesting to report our case because of the rarity of chondrosarcomas of the larynx and because of the unusual size of our tumour.
Case report Male patient, K.K., aged 76, was admitted to the ENT Department of the State General Hospital of Athens, complaining of hoarseness and progressive difficulty in breathing for the last two months. Dyspnoea had increased during the last five days and the patient had developed acute upper respiratory obstruction for which an emergency tracheostomy was performed on admittance. The laryngeal cavity was almost completely obstructed by a subglottic tumour. On direct laryngoscopy the tumour was found to be quite extensive, with a smooth surface, located on the left side and probably originating from the cricoid cartilage. The vocal cords were normal but almost fixed, with a small rima glottidis and firm arytenoid area. No palpable cervical lymph nodes were found. A total laryngectomy was performed ten days after the tracheostomy, with excision of the first tracheal ring, The final pathology report was as follows: 'The larynx 5-5x4-8x3 cm. in dimensions, showed marked oedema of the epiglottis, the aryepiglottic folds and the pharyngeal wall. The vocal cords and the region above them were intact. The subglottic area was nearly obstructed by a tumour 3-5 cm. in diameter, which originated from the left lateral wall and extended towards the laryngeal cavity. The tumour was also occupying a small part of the anterior and posterior fossa extending a short distance along the right lateral wall below the glottis. The surface of the tumour was ulcerated because of the former biopsy (Fig. 1). In cross section the tumour tissue was hard or elastic in consistency and whitish or brown in colour. Microscopically the tumour consisted of immature cartilaginous tissue separated in lobules by thin connective tissue septa. The neoplastic chondroid cells were found in small groups or, exceptionally, singly in the chondroid cavities. The nuclei were large and dense with irregularities in shape and size and showed a moderate number of mitoses. The ground substance was generally slight because of the great cellularity of the tumour. The tumour cells infiltrated the arytenoid cartilage and penetrated the underlying muscles to a small depth.' The histologic diagnosis was: 'Chondrosarcoma of the larynx with infiltration of the underlying tissues. Lymph nodes were not found.' (Fig. 2.) 529
530
A. COYAS et
al.
Discussion Although the aetiology of cartilaginous tumours of the larynx is unknown, some suggestions have been put forward by Moore (1925): — chondroplastic predisposition of bronchial tissue to chronic inflammation; — abnormalities in ossification. As far as sex and age incidence are concerned, all authors maintain that these tumours occur more frequently in males than females and in middle age. Also, a few cases have been reported at the age of 15 as well as 91 (Barsocchini and McCoy, 1968). The site of these tumours seems to be predominantly the cricoid cartilage, at a rate of 70 per cent (De, 1972), although other cartilaginous structures of the larynx may be involved, such as the thyroid, the epiglottis and the arytenoids. The diagnosis is easily made histologically, but a suggestion of cartilaginous tumour should be clinically based on the long duration of the symptoms in relation to the laiyngoscopic findings. The most common and characteristic symptoms of the lesion, which are of course related to the location of the tumour, are hoarseness and progressive difficulty in breathing and swallowing. Indirect laryngoscopy is very important for establishment of the diagnosis and reveals the site and the size of the tumour, which is usually subglottically and submucosally located. Microlaryngoscopy gives definitely more details about the exact extent of the tumour, whether it is sessile or not, and also whether other cartilaginous structures are involved. The treatment of chondrosarcomas of the larynx is definitely surgical and all authors agree that radiotherapy has little effect. The high rate of local recurrence does not seem to favour conservative surgical procedures, although several
FIG. 1 A large neoplastic tumour at the left subglottic area extending to the posterior fossa and the lateral right wall.
CLINICAL RECORDS
531
FIG. 2 Histology of the chondrosarcomatous tissue. Note the high cellularity and the marked atypia of the neoplastic chondroid cells.
authors (Barsocchini and McCoy, 1968) agree on a complex surgical scheme which is a combination of tracheostomy, laryngofissure and excision of the tumour with preservation of the laryngeal skeleton. An exception may be noted in our decision as to the extent of the operation and this is the age of the patiens and the size of the tumour. Small and sessile chondiosarcomas in young patientt may indicate a conservative surgical operation as a first stage, following up the patient's condition very closely. Adenopathy and metastasis, although considered extremely unusual, must also be closely followed. Summary
We report one case of chondrosarcoma of the larynx, which is considered to be rather uncommon. Clinical signs and symptoms have been noted, as well as some suggestions on the aetiology, incidence and site of cartliaginous tumours of the larynx. REFERENCES BARSOCCHINI, M. L. and McCoy, G. (1968) Annals of Otology, Rhinology and Laryngology, 77, 146. CHAMBERS, G. R. and FRIEDEL, W. (1976) The Laryngoscope, 86, 713.
DE, R. P. (1972) 'Chondrosarcoma of the larynx', Journal of Laryngology and Otology, 86, 1261. MOORE, J. (1925) Journal of Laryngology and Otology, 40:9, 84, 145. A. Coyas, 20 Kanari Street, Athens 138, Greece.
