Clear cell chondrosarcoma of the larynx

Abstract

Chondrosarcomas represent a group of malignant tumours composed of cells producing cartilage, and clear cell chondrosarcomas (CCC) represent a variant of these. A rare case of a laryngeal CCC is presented. The patient was a 70-year-old man who presented with a 6-week history of throat pain, worsening dysphonia and a lump in the neck. Examination revealed a level 3 neck mass and positron emission tomography imaging revealed a metabolically active right laryngeal mass. Biopsies revealed a cartilaginous neoplasm. The patient underwent total laryngectomy, and the pathology confirmed CCC of the larynx. Chondrosarcomas are the most common type of sarcoma in the larynx but the CCC variant is rare. CCC are usually low-grade tumours affecting long bones, with a male predominance. Less than six cases have been described in the literature affecting the larynx. Management of these malignancies is complete surgical excision given the high risk of local recurrence.

Background

There have been <10 documented cases of clear cell chondrosarcomas (CCC) occurring in the head and neck region in the literature with only 5 of these occurring in the larynx. Although these tumours are low-grade and slow-growing tumour, they have a propensity to recur, hence optimal management involves surgical resection. Our case highlights a rare disease warranting prompt surgical treatment and appropriate long-term follow-up. This case also serves as an important reminder to consider the various types of malignant tumours of the larynx.

Case presentation

The patient was a 70-year-old man who presented to hospital with a 6-week history of throat pain, worsening dysphonia and a lump in the neck. He was an ex-smoker (40 pack-year history) and consumed six standard alcoholic drinks per day for the past 40 years. His medical history was significant for hypertension, haemochromatosis and bilateral total hip replacements. On examination the patient showed evidence of mild dysphonia, mild dyspnoea but no obvious stridor. Palpation of his neck revealed a right level 3 neck mass without any other cervical lymphadenopathy. Flexible nasoendoscopy demonstrated a fixed right arytenoid without visualisation of the vocal cord. There were no obvious mucosal abnormalities.

Investigations

CT imaging further characterised the lesion (see figure 1) and subsequent positron emission tomography imaging for the purposes of staging revealed a moderately metabolically active (standardised uptake value: 6.1) right laryngeal mass (see figure 2). The report demonstrated intense activity involving the cricoid cartilage with possible involvement of the right side of the hyoid; however, no obvious extra-laryngeal soft-tissue extension or evidence of locoregional nodal disease was noted. An ultrasound-guided fine needle aspiration (FNA) and core biopsy was performed. FNA smears and core imprints were moderately cellular and showed fragments of hyaline cartilage. Core biopsy demonstrated similarly to non-specific appearances of cartilage that may represent a cartilaginous neoplasm.

Figure 1.

CT of a large tumour mass arising from the right glottic region with extensive calcification causing compression and distortion of the midline structures.

Figure 2.

Positron emission tomography of a moderately metabolically active lesion centred on the right larynx. The intensely calcified component is not active but there is a rim of intense activity surrounding this and extending to involve the left cricoid cartilage and possibly the right hyoid.

Outcome and follow-up

The case was discussed at the hospital’s head and neck multidisciplinary team meeting (MDM), and as suggested by the board, the patient underwent an en-bloc total laryngectomy and right hemi-thyroidectomy, with an adequate resection including all the macroscopic tumour. Histopathology confirmed the diagnosis of a 4.5 cm CCC arising from the right cricoid cartilage with some extension into the soft tissues anteriorly, posteriorly and inferiorly. There were two margins involved, posterior and inferior, with the anterior margin clear by 1 mm. After discussion at the head and neck MDM, considering the resection and absence of further soft-tissue extension on the imaging, an active surveillance approach was indicated without adjuvant treatment. At 13 months follow-up with close clinical and imaging observation, there were no signs of recurrence.

Discussion

Although the incidence of chondrosarcomas in the head and neck region is low (1%–3%), they are the most common type of sarcoma in the larynx. This is not surprising given the cartilaginous framework of the larynx. They have also been reported to occur at varying sites in the head and neck including the mandible, maxilla, nasal cavity, skull base and larynx.¹ There are several histological variants of chondrosarcoma; however, the clear cell type is the rarest accounting for approximately 2% of these. CCC are typically low-grade tumours that demonstrate slow growth and overall low rates of recurrence and metastases.^2–4 They have a male preponderance, typically affecting the long bones of the femur and humerus in elderly patients.

Only five laryngeal CCC have been reported since it was initially described in 1976.^5–8 All of the published cases occurred in male patients aged between 46 and 61 years. They most commonly occurred in the thyroid or cricoid cartilages, and all patients underwent surgical resection with total laryngectomy the procedure of choice for most (four of the five). The case we present is similar to the reported cases in the literature, with no reported cases of recurrence at follow-up (range: 6 months to 10 years). Mokharti et al's study in 2012 reviewed the literature on CCC of the head and neck region.⁹ Less than 10 cases of CCC were described in a MEDLINE search using the keyword ‘clear cell chondrosarcoma’. Tumour sites included the nasal septum, maxilla, skull in addition to the larynx as described earlier. Interestingly, despite a male predominance, both cases of nasal septum CCC occurred in females. Management included surgical resection for all the tumours with only one recurrence reported.⁹ In contrast to our case, most of these patients presented with a long duration of symptoms which is in keeping with the indolent nature of these tumours.

Learning points.

• Chondrosarcomas, although rare, do occur in the head and neck region.

• The clear cell variants are exceptionally rare, especially in the larynx, but they have been described.

• Clear cell chondrosarcomas (CCC) are predominately low-grade, slow-growing tumours with minimal metastatic potential; however, complete surgical resection is necessary to prevent local recurrence.

• We have presented one of the few cases of CCC variant arising in the larynx of an elderly male, and it serves as an important reminder to consider the various types of malignant laryngeal tumours.