Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Sarcomas of the Larynx Sarcomas of the larynx are rare. Of all cancers, 2% are located in the larynx and less than 1% are of sarcomatous or mesenchymal origin. Chondrosarcoma is the most common of these laryngeal sarcomas and differs from the other sarcomas with respect to its origin and length of survival free of disease. Recent literature reviews on sarcomas of the larynx have found the prevalence of chondrosarcoma to surpass that of fibrosarcoma, which was previously thought to be the most common mesenchymal lesion of the larynx. Chondrosarcoma will be reviewed in detail, followed by a brief discussion of fibrosarcoma. Chondrosarcoma accounts for 10% to 20% of malignant primary bony-cartilaginous tumors. Chondrosarcoma is rarely encountered in the head and neck; in a series by Burkey et al, only 12% of the cases arose in the head and neck. This tumor is commonly encountered in the pelvis (22%), the long bones of the upper limb (17%) and lower limb (20%), as well as the sternum, ribs and clavicle (24%). Chondrosarcoma of the larynx has a different biologic behavior from those lesions found elsewhere in the body. They are usually more well differentiated and clinically less aggressive than lesions found at other sites and for these reasons should be treated differently. Chondrosarcoma originates from cartilage, of which there are three types: hyaline, elastic, and fibrocartilage. Almost all chondrosarcomas arise from the hyaline cartilage which is located in the cricoid, thyroid and arytenoids. Elastic cartilage can be found in the epiglottis, corniculate and cuneiform cartilages and is less frequently associated with this tumor. Fibrocartilage is not found within the larynx. With respect to the site of origin, 75% of the tumors originate in the cricoid cartilage while only 15% to 20% are located in the thyroid cartilage. Epiglottic tumors are rare because the epiglottis is made up of elastic cartilage. Heusinger in 1822 is credited with the first description of a cartilaginous tumor of the larynx, not otherwise specified. New, in 1935, reported the first malignant cartilaginous lesion of the larynx. Since that time there have been fewer than 200 case reports of laryngeal chondrosarcoma. The true incidence is unknown and is difficult to assess secondary to low grade chondrosarcoma being misinterpreted as chondroma. Thus the incidence of chondroma tends to be overestimated. Since 1984, when Alfio Ferlito of Italy reviewed over 149 cases in the world literature, there have been less than 50 case reports. Epidemiologically, age and sex distributions do not differ much from those lesions of long bones. Chondrosarcoma of the larynx tends to occur in the sixth through seventh decade and is more common in males than in females. These tumors do not seem to be related to smoking or alcohol intake and in general arise spontaneously. The signs and symptoms are related to the location of the tumor. Numerous case reports in the literature note a history of idiopathic vocal cord paralysis. In general, patients with chondrosarcoma note a slow advancement of symptoms. Chambers et al reported the average duration from the time of onset to the time of diagnosis to be 1.8 to 3 years, with some going for greater than 9 years with symptoms prior to diagnosis. Patients tend to note progressive hoarseness and dyspnea with lesions of the posterior lamina of the cricoid. Dysphagia may also be present, with the extension of the lesion into the hypopharynx. Occasionally patients may note a hard mass in the neck when the growth is emanating from the thyroid cartilage. Acute respiratory insult combined with the slow growing lesion may lead to acute airway obstruction necessitating emergent tracheotomy. Vocal cord paralysis present with a submucosal mass are the usual findings on indirect laryngoscopy. Endoscopic findings may be vocal cord paralysis that is not associated with any other laryngeal lesions or damage to the recurrent laryngeal nerve. Other findings include a submucosal mass typically located in the posterolateral or posterior subglottic region. Rarely is there seen any ulceration or fungating mass. Radiologic evaluation may begin with soft tissue x-rays which show mottled calcification within the mass in 80% of patients. This is pathognomonic for chondrosarcoma or chondroma. CT scan of the neck with contrast is the best diagnostic imaging study to be performed. In general, laryngeal chondrosarcoma appears as a lesion with calcified areas extensively involving one or more cartilages with moderate enhancement after injection of contrast. Often seen is the displacement of surrounding structures rather than invasion, secondary to the slow growing nature of this tumor. The radiographic differentiation of chondroma vs. chondrosarcoma is not possible. MRI adds little information. Lesions with this mode of imaging show T1 images with homogenous and intermediate signal intensity and hyperintense T2 weighted images. Diagnosis is made upon histologic examination of tissue. Biopsy is frequently difficult secondary to submucosal location and hard bony consistency of the tumor, as in the case of the patient presented. Generous biopsy sample is necessary due to varying degrees of differentiation. Tracheotomy may be necessary to obtain better exposure to the lesion. Some have advocated the use of laryngofissure to perform an excisional biopsy. Differentiating chondroma vs. chondrosarcoma is difficult. Chondroma, a benign tumor of cartilage is often indistinguishable form chondrosarcoma, particularly with well differentiated chondrosarcoma. According to Thomas et al, there is no evidence of malignant transformation of chondroma to chondrosarcoma. Chondromas resemble normal cartilage but are slightly more cellular and are composed of chondrocytes, one per lacunae, with uniform hyperchromatic nuclei. Histologically, chondrosarcoma can be classified as grade 1, grade 2, or grade 3. The criteria for malignancy were devised in 1943 by Lichtenstein and Jaffe. The histologic grades vary with respect to the cellularity, matrix, and presence of mitoses. Ninety-five percent of tumors are of low grade nature. There is a very small percentage of high grade tumors which carry a poor prognosis. Immunohistochemistry has allowed more defined classification of these tumors. Immunohistochemical studies show marked positivity for the s-100 protein and vimentin. Low grade tumors are very similar to chondroma. The histologic section is hypercellular with abundant basophilic and metachromatic cartilaginous matrices, also known as chondroid, in which the cells are small with densely staining nuclei. Vesicular nuclei and prominent nucleoli indicate malignancy. Mitoses are usually absent in low grade tumors. Medium grade tumors show increased cellularity, particularly in the periphery of the lobules. Also noted are plump binucleated cells with scarce mitoses, less than 2 per 10 high power fields. In comparison, high grade chondrosarcoma reveals pronounced cellularity and poorly developed matrix with frequent mitoses, greater than 2 per 10 high power fields. Surgical excision is the treatment of choice. The tumor should be excised widely with special attention to excision of the external perichondrium, along with a margin of normal tissue, in order to ensure eradication of the tumor. Since the tumor is not locally aggressive, conservative voice preserving procedures are preferred despite high recurrence rates. Total laryngectomy should be performed for lesions involving greater than 50% of the cricoid and those with local invasion. Neck dissection is indicated in the rare cases of cervical lymph node involvement. Radiation and chemotherapy have not proven to be beneficial in cases of primary disease. The management of cricoid tumors depends on the extent of cricoid involvement. The cricoid is the cornerstone of the larynx and removal of greater than half interrupts the laryngeal support. Tumors of the cricoid can be excised through laryngofissure only if less than half of the cricoid is involved, in order to prevent the risk of permanent laryngeal stenosis. Reconstruction of the laryngeal lumen can be performed via placement of an interposed rib graft or through the use of a composite epiglottic flap. Total laryngectomy is performed for the majority of tumors involving greater than 50% of the cricoid. Batsakis et al determined factors that affect the prognosis of chondrosarcoma to include the degree of differentiation, tumor size, tumor location and adequacy of initial surgical resection. Five-year survival has been quoted as 76% to 91% for low grade tumors. High grade tumors carry a considerably worse prognosis with survival quoted at 43% by Finn et al. Recurrence rates following conservative resection are high, 40% to 80%. Recurrence is probably secondary to piecemeal excision sparing the external perichondrium and persistence of the tumor. Metastases are rare but are more frequent with the higher grade lesions and usually occur to the lung. Fibrosarcoma was thought to be the most common malignant mesenchymal tumor but, as previously stated, has been surpassed by chondrosarcoma. Many of these "spindle cell" lesions reported in the literature as fibrosarcomas, after further review, have turned out to be other fibrous tumors. With the advent of immunohistochemical staining and ultrastructural investigations, there has been a notable decrease in the number of case reports of fibrosarcoma over the recent past. A true fibrosarcoma is a marker negative tumor, with positive staining for vimentin only. Ultrastructural cell composition is fibroblastic. Fibrosarcoma has similar epidemiology and clinical presentation to that of chondrosarcoma. The tumor is usually located on the anterior portion of the vocal cord, anterior commissure or both. The tumors appears as a nodular and pedunculated mass and is rarely ulcerated. Histologically, uniform spindle shaped cells are seen arranged in an interlacing bundle, forming a herringbone pattern. Well differentiated tumors have well developed collagen production and few mitoses. Poorly differentiated tumors have plump spindle shaped cells with frequent mitoses and little chromatin. The biologic behavior is related to the histopathologic differentiation. Well differentiated fibrosarcoma is three times more common than poorly differentiated tumors and usually has no metastases. There is rarely spread to regional lymph nodes. Treatment depends on the size of the tumor and extent of the lesion. All lesions should be widely excised. Histologic grade is also a significant factor with conservative excision for only the well differentiated tumors. The five-year survival is 50% for well differentiated and 5% for poorly differentiated and there is a 40% local recurrence rate. In conclusion, chondrosarcoma and fibrosarcoma are the most common laryngeal sarcomas. The diagnosis of these lesions is difficult but has been aided by the advent of immunohistochemical staining. Surgical resection is the treatment of choice for chondrosarcoma and fibrosarcoma and should be tailored based on the tumor location and histologic grade. Conservative surgical treatment is the mainstay as the lesions are slow growing with a low rate of metastases. Case Presentation A 65-year-old white female s/p lumpectomy and radiation therapy for stage I cancer of the left breast in 1986, presented with a right true vocal cord paralysis in 1988. Evaluation at that time was negative. She was lost to follow-up until January 1993, when she was readmitted for a coronary artery bypass procedure. During induction of anesthesia, the patient was unable to be intubated and an emergent tracheotomy was performed. The otolaryngology service was consulted postoperatively and evaluation revealed a large amount of laryngeal edema and right submucosal hemorrhage obscuring the glottis. Subsequent persistent asymmetry of the glottis was noted and attributed to traumatic injury. She was decannulated at the end of her hospital stay and discharged home. On follow-up as an outpatient, the edema slowly resolved but the asymmetry persisted. CT scan was performed which showed a large right-sided laryngeal mass which appeared submucosal in nature, extending from the aryepiglottic fold superiorly down to the subglottic region. Irregular ringlike calcifications were noted in the mass suggestive of cartilaginous origin. The patient was admitted to The Methodist Hospital in September 1993 and underwent tracheostomy and direct laryngoscopy with biopsy. 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Arch Otolaryngol 1975;101:232-234. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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