Low Grade Laryngeal Chondrosarcoma: Clinical Presentation, Management and Short Term Outcome

Abstract

Low grade laryngeal chondrosarcomas are rare, slow growing tumors. Surgical removal of the tumor along with preservation of laryngeal function is the preferred modality of treatment. We report a case of a large low grade chondrosarcoma removed by transoral CO₂ LASER surgery which had avoided an open surgery.

Introduction

Chondrosarcomas of the larynx are rare tumors and represent approximately 0.2% of all laryngeal neoplasms [1]. Histologically they are divided into low grade and high grade, with low grade tumors having a 5 year survival rate of 79.4% [1]. The definitive treatment of these tumors has been a topic of debate. Laryngeal preservation surgeries like CO₂ LASER resection are preferred for low grade tumors. However total laryngectomy is the standard care of treatment for large tumors involving more than 50% of posterior lamina of cricoid cartilage [2]. We report a case of large low grade laryngeal chondrosarcoma, which was removed using a transoral LASER surgery and short term outcome following it.

Case Details

A male patient of age 54 years, resident of Yemen presented with the complaints of hoarseness, difficulty in swallowing and gradual onset of breathlessness over the past 2 years. Clinical examination of ear, nose, oral cavity and oropharynx was normal. On videolaryngoscopy (Fig. 1a), a smooth surfaced swelling was seen in the posterior medial wall of left pyriform sinus. Superiorly it extends upto the level of left arytenoid cartilage, displacing it anteromedially. Also, there was narrowing of subglottic lumen due to mass extending into it. The vocal cords were normal and mobile. Contrast enhanced CT (Fig. 1b) neck revealed a heterogeneous enhancing lesion of size 2.7 × 2.9 × 4.2 cm (AP × TR × CC) seen in the region of left cricoid cartilage causing anterior superior and medial displacement of left arytenoid cartilage, with intralesional calcifications (Fig. 1c), with no significant cervical lymphadenopathy. The direct laryngoscopy guided biopsy was reported as a well circumscribed tumor with features of low grade chondrosarcoma.

Fig. 1.

a Videolaryngoscopy showing the smooth surface lesion in the left pyriform sinus. b CECT of neck in axial view showing lesion seen in the region of cricoid cartilage. c Soft tissue window showing intralesional calcifications

Following the biopsy report, microlaryngoscopic CO₂ LASER excision and debulking of the lesion was planned. An elective tracheostomy was performed prior to the procedure. During the procedure (done under general anaesthesia) the larynx was suspended using a Lindholm laryngoscope and visualised with the help of a microscope. Using microlaryngeal instruments and Ultrapulse CO₂ LASER (Lumenis Ultrapulse) set at 10 W power and in continuous mode, a mucosal flap was elevated over the lesion. The lesion was removed in piecemeal using cold instruments and CO₂ LASER. After significant debulking of the lesion, hemostasis was achieved and flap was repositioned and secured using laryngeal clips. The postoperative recovery was uneventful. Follow up endoscopy showed healing at the operative site and significant reduction in the size of the lesion along with improvement of subglottic airway. The final histopathology was reported as a low grade chondrosarcoma, which is mildly cellular and composed of lobules of neoplastic chondrocytes having hyperchromatic nuclei with areas of myxoid change and no evidence of high grade transformation or dedifferentiation (Fig. 2).

Fig. 2.

H&E (10 × magnified) image showing lobules of neoplastic chondrocytes

Patient was decannulated later and was in follow-up for 2 years with no increase in size of the residual lesion noted at the operative site.

Discussion

Laryngeal chondrosarcomas are the third most common tumor next to squamous cell carcinoma and adenocarcinoma of larynx [2]. It occurs in the age group above 50 years with male preponderance (M:F ratio 3:1). The reported average age for diagnosing these tumors is 62.5, which corresponds to the age at which hyaline cartilages in the larynx tend to ossify [2]. The exact risk factors and underlying genetic mutations for these tumors is not yet known [3]. It has been proposed that these tumors might arise as a result of disorders of ossification [4]. In a recent systematic review done by Chin et al., the most common cartilage involved was cricoid (72–75%) followed by thyroid cartilage, arytenoid cartilage and the epiglottis [2].

These slow growing tumors present with symptoms ranging from hoarseness/change in voice, difficulty in swallowing and dyspnoea/breathlessness on exertion depending on the site of involvement.

The single best modality of imaging study would be CT scan of the neck to know the site and extent of the tumor as well as presence of neck node involvement. Intralesional calcifications have been described as one of the characteristic CT findings of these tumors which can help in differentiating from other tumors [3].

Diagnosis is established by incisional/trucut biopsy [4]. Histologically they are characterised by nuclear atypia, increased cellularity and extension into surrounding tissues and classified into low, medium and high grade depending on the degree of differentiation [5]. Grade I or low grade tumors have very good prognosis whereas tumors with high grade, myxoid type, clear cell chondrosarcoma and with malignant mesenchymal components have got poor prognosis [2].

Treatment of laryngeal chondrosarcomas is done based on the grade, size and location of the tumor. High grade tumors need aggressive management strategies like total laryngectomy [6]. Whereas in case of low grade tumors preservation of laryngeal function by surgical excision should always be given a preference over radical approaches [5, 7, 8]. There are reports of transoral CO₂ LASER assisted excision of low grade chondrosarcoma of the larynx [9] and also LASER assisted removal is used for recurrences following total laryngectomy [10].

In our patient, there was involvement of cricoid cartilage with narrowing of subglottic lumen which led to a dilemma in choosing between radical and conservative approach. The possibility of total removal of the tumor by total laryngectomy or cricoid excision followed by thyrotracheal anastomosis was discussed. But since it is a low grade tumor with majority of the it seen in the hypopharynx and the patient being a middle aged male, we preferred a conservative approach by performing a transoral CO₂ LASER assisted excision and debulking of the tumor, thereby preserving the laryngeal function. We could debulk the majority of tumor with resolution of clinical symptoms. Patient is under follow-up for the past 2 years with no increase in size of the residual lesion and with no worsening of clinical symptoms.

The drawback of our procedure is that the tumor is not completely removed and there is always a chance of regrowth of the tumor.

Conclusion

Low grade laryngeal chondrosarcomas are slow growing tumors, which can be removed/debulked by using transoral CO₂ LASER procedure. Preservation of laryngeal function is to be given preference over total laryngectomy in these patients.

Funding

Department of Research, Deenanath Mangeshkar Hospital and Research Centre, Pune, India.

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Conflict of interest

No conflicts of interests.

Consent for Publication

I give my consent for publication of this article.

Ethical Approval

Obtained from Institutional ethical committee, Deenanath Mangeshkar Hospital and Research centre, Pune, India.

Human and Animal Rights

It is a retrospective study done on human subjects.

Informed Consent

Not applicable.