Chondrosarcoma of the larynx: A therapeutic challenge.

Abstract

The diagnosis of laryngeal chondrosarcoma is likely to be missed because of its infrequent occurrence and its indolent pattern of growth. A 53-year-old woman came to our service with an 18-year history of hoarseness and increasing dyspnea. She had been previously documented as having left vocal fold paralysis and a bulging laryngeal mass. Computed tomography revealed the presence of a large calcified tumor that had caused a deformity of the larynx and an erosion of the arytenoid and cricoid cartilages. Direct laryngoscopy detected a large supraglottic mass with a normal-appearing mucosa. Total excision of the tumor was achieved through a lateral neck incision that spared the larynx. This case emphasizes the importance of a high index of suspicion for laryngeal chondrosarcoma in a patient who has unexplained vocal fold paralysis and a submucosal subglottic mass. Every effort should be made to take a conservative surgical approach that preserves laryngeal function when possible.

Introduction

As only approximately 200 cases have been reported, chondrosarcoma of the larynx is a rare tumor. [1] Even so, it is by far the most common laryngeal sarcoma. [2] The diagnosis of chondrosarcoma of the larynx is likely to be missed because of its infrequent occurrence and its indolent pattern of growth. [3] Complicating this clinical diagnostic challenge is the fact that it is difficult to differentiate between benign chondromas and the various degrees of malignant chondrosarcomas. [4] Conservative surgery is the mainstay of treatment, although sound oncologic principles must be applied. [3]

In this article, we describe the case of a patient who came to us with a large chondrosarcoma of the larynx, and we provide an updated review of the literature.

Case report

A 53-year-old woman was referred to our clinic with an 18-year history of hoarseness and increasing respiratory difficulty. Five years earlier, she had undergone direct laryngoscopy, which detected a paralysis of her left vocal fold and a bulging laryngeal mass. Otherwise, her medical history was unremarkable.

On clinical examination, no growths were palpable in the neck region. However, a very large supraglottic mass could be seen bulging from the posterior right larynx. The mass covered almost the entire right side of the larynx, obliterating the piriform sinus and causing a deformity of the laryngeal space. The left vocal fold was fixed and the right fold was mobile. The patient had a significant narrowing of the airway. The laryngeal mucosa appeared to be intact, smooth, and normal. A transoral biopsy obtained under local anesthesia revealed that the mucosa was indeed normal, although it was mildly inflamed.

Computed tomography (CT) of the neck revealed the presence of a very large neoplasm that had caused a deformity of the larynx. The tumor, which contained both soft tissue and calcifications, had eroded the arytenoid and cricoid cartilages and had spread as far as the vertebrae bilaterally (figure 1). On direct laryngoscopy, the extralaryngeal mass could be seen encroaching on the larynx. Again, the growth was covered with what appeared to be normal mucosa, the left vocal fold was fixed, and the airway was extremely narrow.

We suspected that the lesion was a chondrosarcoma, and we scheduled the patient for surgery. Because her airway was so narrow and distorted, she could not be intubated. Therefore, we first performed a tracheostomy under local anesthesia. Then we completely excised the neoplasm through an incision in the lateral neck. By using the inner mucosa of the larynx as the surgical margin, we were able to spare the entire larynx. Histopathologic findings were compatible with a low-grade chondrosarcoma of the larynx (figure 2).

The postoperative period was uneventful. Examination of the larynx revealed edema of the right arytenoid cartilage; the right wall of the larynx still contained a bulge, and the left vocal fold was still fixed. The patient was able to speak fluently and there were no aspirations, but the tracheostomy tube was left in place because of the narrow airway.

One year postoperatively, findings on direct laryngoscopy were similar to those noted previously. A followup CT 2 years postoperatively demonstrated no recurrence of the tumor (figure 3).

Discussion

Primary cartilaginous neoplasms of the larynx include chondromas and chondrosarcomas. [5] True chondromas are probably rare; the differential diagnosis includes chondrometaplasia and low-grade chondrosarcoma. [6] Indeed, many cases of cartilaginous tumors that were previously identified as laryngeal chondromas are now believed to represent chondrosarcomas. [6] Chondrosarcomas account for 10 to 20% of all primary malignant bone tumors, [3] and the head and neck are the primary sites in about 10% of these cases. [7] Although laryngeal sarcomas are uncommon, chondrosarcoma is the most common of them. [5] In a series of rare laryngeal tumors reported by the Mayo Clinic, investigators found that 75% of the laryngeal sarcomas were chondrosarcomas. [8]

Laryngeal chondrosarcomas usually arise from the hyaline cartilage of the posterior cricoid. They can also originate in the thyroid cartilage, the arytenoid, and the elastic cartilage of the epiglottis. As these tumors grow, symptoms are produced by mass effect. Rarely do these tumors invade contiguous structures. Vocal fold paralysis is primarily the result of tumor bulk rather than neural invasion.

One interesting aspect of our case was the fact the patient's vocal fold was paralyzed on the left even though the main bulk of the tumor was on the right. Her vocal fold paralysis continued even after the tumor had been surgically removed.

Chondrosarcomas are most common among men between 50 and 80 years of age. [5] Their signs and symptoms are variable, depending on the location of the tumor within the larynx. Hoarseness, dyspnea, dysphagia, and wheezing are common and are often attributed to other causes. [3] A delay in reaching the diagnosis can result in a situation in which a complete resection and preservation of laryngeal function become impossible. In our case, the patient had been symptomatic for 18 years before the diagnosis was established and surgical intervention was undertaken.

The diagnosis of laryngeal chondrosarcoma is based on the criteria devised by Lichtenstein and Joffe for malignant cartilaginous tumors of bone origin, which were published in their landmark article of 1943. [9] These criteria include the presence of (1) many cells with plump nuclei, (2) more than an occasional cell with two such nuclei, and (3) giant cartilage cells with large single or multiple nuclei or with clumps of chromatin. Most of the reported cases have involved low-grade tumors, but some high-grade lesions have been described in the larynx, [10] as have several cases of dedifferentiated chondrosarcomas. [11]

The natural history of chondrosarcoma of the larynx is characterized primarily by its indolent growth, which is often accompanied by a compensation of laryngeal function. There is a potential for local recurrence. [3,5,2] In fact, Hoffer et al reported a recurrence rate of 37% in the cases they studied. [12]

Metastases are uncommon, occurring in only 8.5 to 14% of cases reported; only about 20 cases have been documented in the literature. [13] In such cases, the lung is the most common site of metastasis, [2,14] and the incidence of high-grade malignancy is higher. [15] Death from the disease is uncommon and is usually attributable to uncontrolled local growth.

Referral to an otolaryngologist or head and neck surgeon does not always assure that the correct diagnosis will be suspected. These tumors are often hidden in the sub-glottic area and are covered by smooth, intact mucosa. Attempts at biopsy can be frustrating because the lesions are hard and difficult to penetrate. Failure to obtain a proper histologic diagnosis can result in a further delay in management. Our case illustrates this very point; although our patient was followed by an otolaryngologist and even underwent direct laryngoscopy (which identifled the vocal fold paralysis), a correct diagnosis was not made. Biopsies taken from the larynx also proved to be unfruitful.

CT of the neck is the most effective diagnostic modality. [3] Some 80% of chondrosarcomas have been found to exhibit calcific stippling, which can be diagnostic for the tumor and at least should be regarded with a high index of suspicion. [14]

Traditionally, neither radiotherapy nor chemotherapy has played a significant role in either the primary or adjuvant treatment of chondrosarcomas. [5] This tumor is generally considered to be radio-resistant, although one study reported a good response to radiotherapy in chondrosarcoma patients who were treated with a curative intent. [7] Therefore, a trial of radiotherapy can be considered for a patient who refuses surgery. No benefit of chemotherapy has been documented in the literature. [3] Thus, surgery remains the only treatment. The prognosis depends on the adequacy of the resection, the extent of the tumor, and the histologic grade. [16] Five-year cure rates are of little value in making a prognosis because recurrences can occur as long as 20 to 30 years after the completion of adequate primary treatment. [2,14]

Our case clearly demonstrates the diagnostic difficulties and therapeutic challenges involved in the management of chondrosarcomas of the larynx. During the many years that had elapsed before our patient was diagnosed correctly, even direct laryngoscopy with a laryngeal biopsy did not contribute to the diagnostic process. Chondrosarcoma was suspected only at a late stage, after the tumor had grown to very large proportions.

By following the generally recommended external approach, we were able to successfully resect the mass in its entirety while preserving the larynx. Two years following surgery, the patient was in excellent condition. She was able to speak fluently, she was not experiencing aspirations, and she was leading a fully normal life.

We conclude that despite its infrequency, chondrosarcoma of the larynx must be kept in mind in the differential diagnosis of patients who come to the office complaining of hoarseness, dyspnea, and dysphagia. The otolaryngologist should maintain a high index of suspicion, especially when there is an unexplained vocal fold paralysis and a submucosal subglottic mass. CT is of paramount importance in establishing the correct diagnosis. Early recognition will facilitate management and improve the chance that laryngeal function will be preserved.

We believe that laryngectomy is rarely necessary as an initial treatment for laryngeal chondrosarcoma. If laryn-geal function stands to be compromised by a complete resection of the tumor, a subtotal removal is appropriate, as long as the clinician maintains close clinical followup. Further surgery can always be performed if necessary. In our opinion, it is preferable to remove the tumor only to the extent that allows for preservation of some laryngeal function (with a tracheostomy if necessary) rather than to perform a total laryngectomy. With this approach, patients can expect to enjoy reasonable speech and near-normal swallowing.

Because of the indolent growth of chondrosarcomas, recurrences can often be treated by additional conservative surgery. Radical surgery should be reserved for very extensive or high-grade tumors and for patients who experience multiple recurrences.

References

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