The differential diagnosis of laryngeal skeletal or soft tissue lesions composed entirely or partly of cartilage can be broken down into 4 groups: chondrosarcoma, chondroma, chondrometaplasia, and cartilage within otherwise classified neoplasms. The differential diagnosis is of paramount importance and various pathologic and clinical aspects make accurate diagnosis difficult.

Cartilaginous lesions are either neoplastic or metaplastic in origin, chondroma and chondrosarcoma being neoplastic lesions and chondrometaplasia being metaplastic in origin. The cause of cartilaginous lesions is unknown. It is felt that these lesions originate from either perichondrial connective tissue or from the body of cartilage. The fact that the lesions tend to occur in older age has led to two theories of origin: inflammation and senile ossification. Inflammation is thought to trigger a metaplastic change in the branchial tissue while senile ossification is thought to bring about pluripotent cells that then undergo transformation to cartilaginous tumors. A relationship has also been described between radiation, asbestos, and Teflon exposure although this has not been adequately studied.

In brief review of laryngeal histology, one can recall that both hyaline and elastic cartilage exist in this organ. Hyaline cartilage is found is the cricoid, thyroid, and arytenoid cartilages. It is composed of a homogenous, avascular matrix produced by chondrocytes that are surrounded by a capsule. These chondrocytes often exist in isogenous clusters. Elastic cartilage is found in the epiglottis, corniculate and cuneiform cartilages as well as the arytenoid apices. The obvious difference in the appearance is the elastic fibrils in the matrix.

Chondrosarcoma comprises 0.5% of all laryngeal tumors and only 0.1% of all head and neck malignancies. Approximately 200 cases of chondroma and chondrosarcoma of the larynx have been reported in the literature. The exact incidence of chondrosarcoma is not known and earlier literature is likely to have underestimated the true incidence of chondrosarcomas because the distinction between chondroma and low - grade chondrosarcoma had not been well made. Males predominate at a ratio of 3:1. They are seven times as common in Caucasians as African Americans. Malignant lesions tend to increase with age, the average age of diagnosis at 66 years, ranging from the fifth to seventh decade, but they have been diagnosed over an age range of 33 - 91 years.

Chondrosarcoma occurs most frequently in the cricoid cartilage particularly in the posterior lamina (78%), followed by the thyroid cartilage (18%) and epiglottis or arytenoids. There are no reported cases in the cuneiform, corniculate, or tichea cartilages. Observation of chondrosarcoma in the epiglottis disproves the previously held notion that chondrosarcoma occurs only in hyaline and not elastic cartilage.

Chondrosarcomas are almost always symptomatic. The signs and symptoms at presentation depend on size and location. With cricoid lesions, there is progressive compromise of the subglottis producing hoarseness and dyspnea that can be abrupt in onset possibly requiring emergent tracheotomy. Dysphagia may also occur with cricoid lesions and is more likely to exist when the lesion arises from the posterior lateral cricoid. Lesions of the thyroid cartilage present as an asymptomatic neck mass. Interestingly, the first clinical sign of chondrosarcoma may be vocal cord paralysis that is not associated with other laryngeal or nonlaryngeal lesions. Some authors believe that idiopathic vocal cord paralysis is almost an exclusive sign of cricoid chondrosarcoma and may be related to involvement of the recurrent laryngeal nerve or cricoarytenoid joint fixation. Combining idiopathic vocal cord paralysis with symptoms of pseudoasthma and cough are a few reasons why this lesion presents a diagnostic dilemma. Primary care physicians may treat patients for prolonged periods with a referral to an otolaryngologist resulting at the time of airway distress.

CT scan of the neck with contrast is the radiographic gold standard for diagnosis. 80% of cartilaginous lesions will demonstrate calcification of a moderately enhancing lesion. It cannot, however, distinguish chondroma from chondrosarcoma. One may also be presented with plain films of the soft tissues of the neck of which 75 % of the lesions will show calcification. Direct laryngoscopy with biopsy is necessary to establish a tissue diagnosis and a therapeutic plan. Biopsy, however, may be difficult either due to the hard consistency of the lesion or due to cystic softening. A laryngofissure for biopsy with preparation for definitive surgical procedure based on frozen section by an experienced pathologist may be necessary. If obtaining a formal diagnosis is not possible at the time of frozen section, awaiting a formal pathologic consultation is not a detriment to the patient. Given the slow growing nature of the lesion and the desire to preserve laryngeal function if oncologically sound.

Lichtenstein and Jaffe in 1942 are credited with making the histopathologic distinction and grades of chondrosarcomas and chondromas. This is the currently followed classification scheme for all cartilaginous tumors throughout the body. They stated that "a cartilaginous tumor should be considered a malignant tumor if it shows, even in scattered fields: 1)many cells with plump nuclei, 2)more than an occasional cell with two such nuclei, and 3)giant cartilage cells with large or multiple nuclei or with clumps of chromatin." Chondrosarcomas are graded into three groups: low, intermediate, and high grades. The histopathology of a low - grade lesion reveals a lobular pattern which under higher magnification reveals a slight increase in cellularity with hyperchromatic nuclei, and a rare binucleate or multinucleate cell. Intermediate - grade shows a significant increase in cellularity with enlarged binucleate cells and rare mitotic figures. High - grade lesions have a pronounced hypercellularity with frequent binucleate/multinucleate cells and frequent mitoses.

Chondrosarcoma is cited to have a local recurrence rate of 60 - 80%. The general concern for local recurrence involves surgical technique and lesions of the cricoid cartilage. Metastases are rare, occurring at a rate of 8.7% with a 10% mortality. They occur most frequently in the lung and lymph nodes. They occur long after diagnosis, making long term follow-up of these lesions necessary. High-grade lesions are those most likely to metastasize.

It is important to differentiate low - grade chondrosarcomas from chondromas. Chondromas are more likely to occur at a younger age although adults are more commonly affected. The symptoms are the same but chondromas are more likely to be asymptomatic. The site of origin is the same but chondromas tend to be less than 2cm while chondrosarcomas are greater than 3cm. It is extremely rare to have recurrence of a chondroma following complete excision nor do they exhibit metastatic potential. Histologically, chondromas appear as normal cartilage with defined lobules with low cellularity and absence of nuclear atypia.

Chondrometaplasia is a common lesion that must be differentiated from a neoplastic cartilaginous lesion. They are nodules less than 1cm that occur on the ventricular bands and vocal cords. They may present with hoarseness but are often asymptomatic. Elastic cartilage is present versus hyaline cartilage. There is not a lobular pattern but a zone of transition from a fibroblast periphery to elastic cartilage is present.

Radiotherapy and chemotherapy are of little value in the treatment plan. The choice of initial surgery is often difficult but should be based on the site, size, and grade of the lesion. Small lesions of the arytenoid and epiglottis can be removed endoscopically. Moderate sized lesions involving less than 50% of the cricoid may be treated with laryngofissure, with or without partial laryngectomy. Thyroid cartilage lesions may be removed through an extralaryngeal approach. Massive lesions, involving more than 50% of the cricoid cartilage or high grade lesion should be managed with total laryngectomy, laryngofissure with or without partial laryngectomy followed by laryngeal reconstruction, the later treatment being controversial.

All major series recommend a conservative attitude because salvage laryngectomy provides a cure. Initial laryngectomy has shown a cure rate of 86% versus 77% for salvage laryngectomy, the difference obviously not being significant.

In conclusion, cartilaginous lesions of the larynx are rare lesions that pose many diagnostic and therapeutic challenges. Fortunately even the most aggressive lesions are rare and the lower grade lesions curable. As always, keeping the patient in mind as regards to age, lifestyle, and willingness to undergo multiple surgical procedures possibly emergent in the face of life threatening airway compromise is critical. Caution should be taken not to underestimate these lesions. Future research for furthering laryngeal preservation with laryngeal reconstruction following excision of massive lesions or lesions involving more than 50% of the cricoid cartilage is needed. Seven cases have been reported to date using stents, rib grafts and an epiglottic flap. These numbers are inadequate to draw any meaningful conclusions.

R.P. is a 66-year old male with a history of emphysema, 4 years status post laryngofissure for a laryngeal chondroma, who presented with a 6-month history of increasing dyspnea. He had last been seen 3 years prior without evidence of disease although his hoarseness had never improved postoperatively. His voice quality had deteriorated to a whisper over the last year. He was newly diagnosed with reactive airway disease due to stridor but had been unresponsive to medication. He complained that he could hear himself wheezing and it was getting so severe that his primary care physician had referred him to a pulmonologist for evaluation. He denied dysphagia, weight loss, nor had he noted any neck masses.

His past medical history was remarkable for multiple medications for reactive airway disease and socially he quit smoking 18 years ago and did not drink alcohol.

Physical examination revealed biphasic stridor with mild respiratory distress due to a large submucosal mass filling the right side of the larynx, which pushed the left vocal cord laterally. Both vocal cords were essentially immobilized.

The patient was taken to the operating room where he underwent a tracheotomy and total laryngectomy. This revealed a tan, hard lesion filling the entire larynx and appearing to arise from the posterior cricoid cartilage. Final pathology revealed a grade I chondrosarcoma. One pathologists review of the previous chondroma revealed no error in diagnosis. Recovery was uneventful and the patient was discharged home the eleventh postoperative

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