Bone cancer xray

Chondrosarcoma of the larynx

Sarcomas are uncommon turnouts of the larynx, constituting less than one percent of laryngeal malignancies. Chondrosarcoma is the most common of these malignant mesenchymal neoplasms.

The relative rarity of this condition, combined with the insidious onset of symptoms can lead to a delay in diagnosis and treatment. Histopathological distinction between chondroma and chondrosarcoma may also prove difficult, further complicating management decisions.

Surgical removal is the mainstay of treatment, with conservative resection wherever possible. Complete tumour removal must not be compromised as these lesions do recur and can metastatize.. Total laryngectomy may be necessary for advanced tumours. Radiotherapy has traditionally been held to be ineffective in the treatment of chondrosarcoma but may be of benefit in certain situations.

Case Report

A 68 year old male presented to the emergency department with a two day history of worsening stridor. There was a two year history of "wheezing" diagnosed as asthma by his general practitioner but no treatment had been instituted. He had noticed increasing exertional dyspnoea and noisy breathing in the two months prior to presentation. He gave an associated history of hoarseness which was initially intermittent but had become permanent in the previous two months. He also complained of dysphagia to solids and liquids. He ceased smoking twenty five years prior, having smoked one to two cigarettes a day for ten years.

Past medical history included cerebrovascular disease, ischaemic heart disease (with coronary bypass grafting six years earlier), hypertension and type 2 diabetes mellitus.

Examination revealed an elderly man with obvious stridor. He was not cyanosed and oxygen saturation in room air was 98 percent. Indirect laryngoscopy revealed a large subglottic mass restricting the airway to approximately 25 percent of normal. The vocal cords appeared normal although the right cord was tented up by the mass. A small node was palpable in the left level 3 region.

Chest xray was clear. Haematology and biochemistry screening was normal apart from a raised blood glucose level of 9.4 millimoles per litre.

A tracheotomy was performed under local anaesthesia and having secured the airway, the patient was placed under general anaesthesia and direct laryngoscopy was performed. A large fleshy mass was found to arise from the posterior subglottis. The vocal cords were draped over the tumour but not directly invaded. Multiple biopsies were taken and sent for histopathology and microbiology.

Histopathology revealed a cartilaginous tumour. Well differentiated chondrosarcoma was the favoured diagnosis although chondroma was offered as a possible alternative.

Computerised tomography revealed a three centimetre diameter mass arising from the posterior aspect of the cricoid cartilage, consistent with a chondroid neoplasm. No significant cervical lymphadenopathy was noted (see Figures 1 and 2).

In view of the degree of cricoid involvement, total laryngectomy with primary tracheo-oesophageal puncture was performed. Pathology revealed a well differentiated chondrosarcoma with complete excision.

Post-operative course was smooth apart from erratic blood glucose levels. The patient refused placement of a Blom-Singer valve after discussion with his family although he was able to produce a reasonable voice with occlusion of his fistula.

Discussion

Laryngeal chondrosarcoma is an uncommon tumour, accounting for less than one percent of laryngeal malignancies (Ferlito et al 1984). It most commonly affects patients aged 50-70 years with a male predominance of 3-4:1 (Mishell et al 1990). The cricoid cartilage is the most common site of origin (70-80%), followed by the thyroid cartilage (20-30%), arytenoids, vocal cords and epiglottis (Nicolai et al 1990).

The tumour presents most commonly with hoarseness, dyspnoea and dysphagia. Onset of the symptoms may be insidious and misdiagnosed as other respiratory conditions as this case (asthma). Progression is usually slow but acute presentation with airway obstruction can occur (Cantrell et al 1980). Other modes of presentation include vocal cord palsy or a mass in the neck, particularly if the tumour arises from the thyroid cartilage (Bogdan et al 1994).

Computerised tomography will show characteristic calcific stippling of the mass in 70-80 percent of lesions (Mishell et al 1990). Magnetic resonance imaging may be required to give enhanced soft tissue resolution in selected cases.

Chondrosarcoma usually appears as a submucosal mass which is hard in consistency. These features can make biopsy difficult. It can be difficult to distinguish between a chondroma and a low grade chondrosarcoma histologically which may have led to an over-reporting of laryngeal chondromata in the literature (Nicolai et al 1990). Factors leading to a diagnosis of chondrosarcoma include cells with plump nucleii, cells with two such nucleii and giant cartilage cells with a single large nucleus, multiple nucleii and clumped chromatin (Bogdan et al 1994).

Classification is according to histological grade (from 1-3) or degree of differentiation (well, moderately or poor). Prognosis worsens with increasing grade or decreasing degree of differentiation. In our case the degree of cricoid involvement made conservation surgery impossible thereby simplifying management decisions despite an equivocal biopsy result.

Surgery remains the cornerstone of treatment for these lesions with conservation of the larynx wherever possible. Oncologic principles must be maintained however as these lesions can recur. Metastasis occurs in 8.5-14 percent of cases, usually in high grade lesions (Nicolai et al 1990, Bogdan et al 1994, Gorenstein et al 1980).

The major dilemma with respect to laryngeal conservation arises when the cricoid cartilage is involved. When less than 50 percent of the cricoid is involved, conservative resections may be safely attempted. A number of techniques have been described using rib cartilage, hyoid bone and local muscle flaps to reconstruct the cricoid Nicolai et al 1990, Bogdan et al 1994). The number of cases remains small however, precluding a careful analysis of results.

Chondrosarcoma has generally been held to be a radioresistant tumour and therefore radiotherapy has not been advocated routinely. Gripp et al (1998) have reviewed their experience along with that in the literature. These cases included patients treated with radiotherapy for local recurrence, residual tumour or who refused surgery or were deemed medically unfit. They contend that radiotherapy is useful in the above settings and can provide remission for up to ten years.

Prognosis of laryngeal chondrosarcoma is generally good as most tumours are low grade. If excision is complete, cure rate approaches 75-80 percent.

Conclusion

Laryngeal chondrosarcoma is an uncommon malignancy which can present difficulties in diagnosis and management. Surgery is the mainstay of treatment with laryngeal conservation being the aim wherever possible. Radiotherapy may have an important role in adjuvant treatment or for treatment of local recurrence. Prognosis is generally good although these lesions can recur and do have a propensity to metastatize..

References

BOGDAN C.J., MANIGLIA A.J. ELIACHAR I., KATZ R.L. (1994) Chondrosarcoma of the Larynx: Challenges in Diagnosis and Management. Head Neck 16, 127-134.

CANTRELL R.W., REIBEL J.F., JAHRSDOERFER R.A., JOHNS M.E. (1980) Conservative Surgical Treatment of Chondrosarcoma of the Larynx. Ann. Otol. Rhinol. Laryngol. 89, 567-571.

FERLITO A., NICOLAI P., MONTAGUTI A., CECCHETTO A., PENNELLI N. (1984) Chondrosarcoma of the Larynx: Review of

the Literature and Report of Three Cases. Am. J. Otolaryngol. 5, 350-359.

GORENSTEIN A., BRYAN NEEL H., WEILAND L.H., DEVINE K.D. (1980) Sarcomas of the Larynx. Arch. Otolaryngol. 106, 8-12. GRIPP S., PAPE H., SCHMITT G. (1998) Chondrosarcoma of the Larynx. The Role of Radiotherapy Revisited - A Case Report and Review of the Literature. Cancer 82, 108-115.

MISHELL J.H., SCHILD J.A., MAFEE M.F. (1990) Chondrosarcoma of the Larynx. Diagnosis with Magnetic Resonance Imaging and Computed Tomography. Arch. OtolaryngoL Head Neck Surg. 116, 1338-1341.

NICOLAI P., FERLITO A., SASAKI C.T., KIRCHNER J.A. (1990) Laryngeal Chondrosarcoma: Incidence, Pathology, Biological Behaviour, and Treatment. Ann. Otol. Rhino]. Laryngol. 99, 515-523.

Royal Perth Hospital Perth, Australia

Stephen Rodrigues M.B., B.S. Registrar, Department of Otolaryngology, Head and Neck Surgery Royal Perth Hospital Wellington Street, Perth, Western Australia 6000 Tel: (08) 9224 3039 Fax: (08) 9224 1779

Stuart Miller F.R.A.C.S. Head of Department Otolaryngology, Head and Neck Surgery Royal Perth Hospital

Andrew Whyte B.D.S., F.D.S.R.C.S., D.D.R.R.C.R., M.B. ChB., F.R.C.R., F.R.A.C.R. Visiting Radiologist Department of Imaging Royal Perth Hospital