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Chondrosarcoma of the arytenoid cartilage: a case report and review of the literature.

Abstract

We report the case of a 56-year-old black woman with a locally aggressive chondrosarcoma at an unusual tumor site: the right arytenoid cartilage. The tumor extended from the superior pole of the right arytenoid cartilage to the soft-tissue areas of the right aryepiglottic fold, piriform sinus, and interarytenoid area. We undertook a conservative surgical approach by performing an endoscopic transoral C[O.sub.2] laser resection, a right arytenoidectomy with wide soft-tissue margins, and a tracheotomy in anticipation of postoperative edema. Surgical exploration revealed that the tumor extended into the cricoid cartilage. Histopathology demonstrated a low-grade chondrosarcoma of the conventional variant. Clear margins were obtained by staged procedures. Chondrosarcomas of the larynx typically exhibit low-grade histopathology, and affected patients have a low incidence of metastasis and a good prognosis. Even so, these tumors can present diagnostic and therapeutic challenges. Surgical resection provides adequate airway protection and sound oncologic safety while preserving speech and swallowing, and these should be the surgeon's goals in this setting. Options include open laryngofissure, thyrotomy, organ preservation with partial laryngectomy, and endoscopic laser resection.

Introduction

Chondrosarcomas are malignant tumors of cartilaginous origin that are usually found in the pelvis and extremities. Only 2 to 5% of all chondrosarcomas arise in the head and neck, (1,2) and they account for fewer than 1% (3) of all laryngeal malignancies. The male-to-female ratio of laryngeal chondrosarcomas is 4:1, and the mean age at presentation ranges between 60 and 64 years? Histologically, laryngeal chondrosarcomas are classified as low-, intermediate-, and high-grade lesions; most are low-grade. Regional metastasis is uncommon.

Chondrosarcomas often originate in the hyaline cartilage. When they do, the most common sites are the cricoid cartilage (70%) and the thyroid cartilage (20%); they rarely arise in the cartilages of the arytenoid, trachea, or epiglottis. (5) In fact, fewer than 10 cases of chondrosarcoma of the arytenoid cartilage have been reported in the literature. As a consequence, information on the presentation, diagnosis, management, and follow-up of these lesions is limited. In this article, we describe a case of chondrosarcoma of the arytenoid cartilage, and we briefly review the literature on these lesions.

Case report

A 56-year-old black woman presented to our clinic for a completion thyroidectomy. She had undergone a thyroid lobectomy for papillary cancer approximately 2 years earlier and had been lost to follow-up.

At presentation, the patient's voice was slightly low-pitched, but she had no laryngeal complaints and no history of laryngeal surgery. Flexible laryngoscopy incidentally identified a thick white plaque that measured 4 to 5 mm in its widest dimension on the right arytenoid cartilage (figure 1). Examination also revealed mild vocal fold edema secondary to her long history of cigarette smoking, but her vocal fold mobility was unimpaired. No additional laryngeal abnormalities were observed.

Under microdirect laryngoscopy, the lesion was removed in its entirety. Histologic analysis identified it as a chondroid mass with low cellularity, mild cytologic atypia, and focal infiltration of the adjacent soft tissue and minor salivary glands (figure 2). The final histopathologic diagnosis was a chondroid lesion that was highly suspicious for low-grade chondrosarcoma of the conventional variant.

At follow-up 2 weeks later, nasopharyngolaryngoscopy detected another white lesion in the right arytenoid cartilage at the same site where the original lesion had arisen (figure 3). Computed tomography (CT) of the neck confirmed a prominence in the right arytenoid cartilage and right aryepiglottic fold; no pathologic lymphadenopathy was evident. Based on the CT findings and the known histopathology of the original lesion, the patient was advised to undergo a definitive excision of the tumor with wide margins.

[FIGURE 1 OMITTED]

The second procedure involved endoscopic transoral C[O.sub.2] laser excision of the superior half of the right arytenoid cartilage and adjacent soft tissue. The mucosa was closed with a suture over the site of excision. Histopathologic review confirmed the diagnosis of a low-grade chondrosarcoma that had infiltrated the respiratory mucosa and exhibited sarcomatous features at the soft-tissue and cartilaginous-specimen margins (figure 4).

A third procedure became necessary to resect residual disease at the surgical margins. Microlaryngeal surgical instruments were used for this procedure to avoid introducing laser artifact into the frozen-section surgical margins. Frozen-section analysis of the margins indicated that the tumor had extended into the mucosa and soft tissues adjacent to the right arytenoid cartilage. These margins involved the medial piriform sinus mucosa, the aryepiglottic fold and corniculate cartilage, and the interarytenoid mucosa. A complete disarticulation of the arytenoid from the cricoarytenoid joint was performed. However, frozen-section margins of the joint were still positive, and therefore further surgery was required--curettage of the superior aspect of the cricoid cartilage until the deep margins showed no evidence of tumor on frozen section (figure 5). A large soft-tissue defect remained, and it was left to heal by secondary intention.

A tracheotomy was performed at the end of the endoscopic procedure to prevent airway compromise from anticipated postoperative laryngeal edema. The patient's postoperative course was uncomplicated, and she was able to swallow without aspiration; dysphonia was only mild. Six weeks postoperatively, the site of the resection had completely mucosalized. The right vocal fold was immobile, but glottic closure was good. Successful decannulation of the tracheotomy was achieved.

[FIGURE 2 OMITTED]

Three years postoperatively, the patient remained free of recurrent disease.

Discussion

The etiology of chondrosarcoma is unknown, but several theories have been proposed. According to one theory, disordered ossification of the laryngeal cartilages contributes to mechanical trauma and ischemia, which in turn lead to the development of pluripotential mesenchymal stem cells. (4) Another theory is that chondrosarcomas are caused by malignant change secondary to polytetrafluoroethylene (Teflon) injections (4,6) or exposure to radiation. (7)

Presentation. The presenting features of laryngeal chondrosarcoma depend on the tumor's specific location in the larynx. Because the tumor grows slowly, patients may experience subtle symptoms for several years before a diagnosis is made. In a study of 111 patients with chondrosarcoma of the larynx, Thome et al found that 65% of them presented with hoarseness and 25% with dyspnea, airway obstruction, or breathing difficulties; a small number presented with a mass lesion, dysphagia or dysphonia, voice changes, stridor, shortness of breath, or pain. (4) It is remarkable that our patient did not exhibit any symptoms; her vocal fold mobility was unimpaired, and there was no mass effect in her airway.

[FIGURE 3 OMITTED]

Physical examination of a patient with chondrosarcoma of the larynx usually reveals the presence of a subglottic or submucosal laryngeal mass, vocal fold paralysis, or displacement of the arytenoid cartilage by the tumor. (8,9) Metastasis rates range from 2 to 8%. (4,10) One report of bone metastasis of a dedifferentiated chondrosarcoma indicated that higher tumor grade is directly correlated with metastasis. (11) Endoscopy in conjunction with CT and magnetic resonance imaging (MRI) may be useful in tumor detection, preoperative planning, and follow-up surveillance. However, it is difficult to distinguish a chondrosarcoma from a benign chondroma on imaging, although a CT that shows coarse or stippled calcifications may raise the suspicion of a chondrosarcoma.

In our patient, no bony or cartilaginous lesions were identified on CT. The prominence on the aryepiglottic fold was the only indication of pathology. The lack of calcific stippling in this case may have been attributable to the limited degree of ossification seen in the arytenoid cartilage; as mentioned, some authors believe that disordered ossification of the laryngeal cartilages may be a cause of laryngeal chondrosarcoma. (4) MRI may be useful in determining the extension of the lesion into adjacent soft tissues. Extension notwithstanding, only conservative surgery with clear margins is indicated for both chondrosarcoma and chondroma.

Differential diagnosis. The differential diagnosis of laryngeal chondrosarcoma includes, in addition to chondroma, benign lesions such as chondrometaplasia and tracheopathia osteoplastica, as well as malignant tumors such as adenocarcinoma and squamous cell carcinoma. Chondromas are also uncommon in cartilaginous tissue; they lack the nuclear atypia, mitotic figures, and necrosis seen in chondrosarcomas. It is interesting that benign chondromas have been observed juxtaposed on chondrosarcomas in the larynx; it has been theorized that this juxtaposition represents a developmental precursor to malignant change. (12)

[FIGURE 4 OMITTED]

Chondrosarcomas of the larynx mainly fall into the low-grade category, arising from hyaline cartilage. In addition to nuclear atypia, the histologic diagnosis of chondrosarcomas is based on their increased cellularity and tendency to invade surrounding tissue. Nonepithelial tumors such as chondrosarcomas tend to arise in submucosal tissue; whether the overlying mucosa is involved is determined by the resistance of local tissues to tumor growth. The penetration of the mucosa in our patient is interesting because most chondrosarcomas present as a submucosal mass. It is possible that the arytenoid tumor eroded through the mucosa by following the path of least resistance superiorly rather than inferiorly through the cricoid cartilage.

[FIGURE 5 OMITTED]

The five major histologic variants of chondrosarcoma are conventional, myxoid, dedifferentiated, clear-cell, and mesenchymal. An analysis of 400 patients with chondrosarcoma of the head and neck in the American College of Surgeons' National Cancer Data Base revealed that 80.8% of the tumors were of the conventional variant, 10.5 % were myxoid, and 8.8% were of the mesenchymal variant. (13) The mesenchymal and myxoid variants were twice as common in blacks and Hispanics than in whites, a difference that the authors attributed to a genetic rather than socioeconomic cause. A poorer 5-year survival rate was associated with higher-grade tumors, regional or distant spread, and the myxoid and mesenchymal subtypes.

Treatment. Preservation of a patient's quality of life with voice- and function-sparing techniques is an important consideration when planning surgical resection of these laryngeal tumors. Therefore, current opinion indicates that a conservative surgical approach is the best option. Tumor resection with a margin of normal cartilage is possible by open laryngofissure, thyrotomy, organ preservation with partial laryngectomy, or endoscopic resection. Total laryngectomy is indicated for large primary tumors, such as those that involve more than half of the cricoid cartilage and for recurrent tumors not amenable to partial laryngectomy. (14)

Conservative surgical management is supported by the low-grade behavior of these tumors and by studies that show only a small difference in survival between patients treated with a total laryngectomy (86%) and patients with recurrent tumors treated with salvage surgery (77%). (14-16) Overall 5-year survival rates in two comprehensive studies of laryngeal chondrosarcoma, one involving 111 patients (4) and the other 47 patients, (17) were 96.3 and 90.1%, respectively. Another option for resection of primary and recurrent lesions is endoscopic C[O.sub.2] laser surgery. (18)

Data on adjuvant radiation and chemotherapy are limited. Chondrosarcomas are generally considered to be radioresistant; radiotherapy for low-grade and residual lesions has been reported successful in only 2 cases with long-term follow-up (3 years (19) and 10 years (20)). Evidence on the efficacy of chemotherapy is even more limited, and it is therefore not recommended.

Overall recurrence rates range from 18 to 40%, so follow-up for at least 5 years--and possibly longer for higher-grade lesions--is critical to ensuring that a patient remains tumor-free. (4,5,21)

In conclusion, chondrosarcomas of the larynx can be cured with minimal morbidity and mortality if they are detected early. Patients with chondrosarcoma of the arytenoid cartilage may not exhibit the characteristic symptoms or findings on CT, but laryngoscopic examination may reveal unusual plaque-like lesions. The diagnosis and management of these tumors presents a challenge because of their infrequency, indolent nature, and high rate of recurrence.

References

(1.) Burkey BB, Hoffman HT, Baker SR, et al. Chondrosarcoma of the head and neck. Laryngoscope 1990; 100(12): 1301-5.

(2.) Ruark DS, Schlehaider UK, Shah JP. Chondrosarcomas of the head and neck. World J Surg 1992;16(5):1010-15; discussion 1015-16.

(3.) Kozelsky TF, Bonner JA, Foote RL, et al. Laryngeal chondrosarcomas: The Mayo Clinic experience. J Surg Oncol 1997;65(4): 269-73.

(4.) Thorne R, Thorne DC, de la Cortina RA. Long-term follow-up of cartilaginous tumors of the larynx. Otolaryngol Head Neck Surg 2001; 124(6):634-40.

(5.) Thompson LD, Gannon FH. Chondrosarcoma of the larynx: A clinicopathologic study of 111 cases with a review of the literature. Am J Surg Pathol 2002;26(7):836-51.

(6.) Hakky M, Kolbusz R, Reyes CV. Chondrosarcoma of the larynx. Ear Nose Throat J 1989;68(1):60-2.

(7.) Glaubiger DL, Casler JD, Garrett WL, et al. Chondrosarcoma of the larynx after radiation treatment for vocal cord cancer. Cancer 1991;68(8): 1828-31.

(8.) Friedlander PL, Lyons GD. Chondrosarcoma of the larynx. Otolaryngol Head Neck Surg 2000;122(4):617.

(9.) Rinaldo A, Howard D J, Ferlito A. Laryngeal chondrosarcoma: A 24-year experience at the Royal National Throat, Nose and Ear Hospital. Acta Otolaryngol 2000; 120(6):680-8.

(10.) Nicolai P, Ferlito A, Sasaki CT, Kirchner JA. Laryngeal chondrosarcoma: Incidence, pathology, biological behavior, and treatment. Ann Otol Rhinol Laryngol 1990;99(7 Pt 1):515-23.

(11.) Nakayama M, Brandenburg JH, Hafez GR. Dedifferentiated chondrosarcoma of the larynx with regional and distant metastases. Ann Otol Rhinol Laryngol 1993;102(10):785-91.

(12.) Baatenburg de Jong R J, van Lent S, Hogendoorn PC. Chondroma and chondrosarcoma of the larynx. Curr Opin Otolaryngol Head Neck Surg 2004; 12(2):98-105.

(13.) Koch BB, Karnell LH, Hoffman HT, et al. National cancer database report on chondrosarcoma of the head and neck. Head Neck 2000;22(4):408-25.

(14.) Koka VN, Veber F, Haguet JF, et al. Chondrosarcoma of the larynx. J Laryngol Otol 1995; 109(2): 168-70.

(15.) Jones DA, Dillard SC, Bradford CR, et al. Cartilaginous turnouts of the larynx. J Otolaryngol 2003;32(5):332-7.

(16.) Lavertu P, Tucker HM. Chondrosarcoma of the larynx. Case report and management philosophy. Ann Otol Rhinol Laryngol 1984;93(5 Pt 1):452-6.

(17.) Lewis JE, Olsen KD, Inwards CY. Cartilaginous tumors of the larynx: Clinicopathologic review of 47 cases. Ann Otol Rhinol Laryngol 1997;106(2):94-100.

(18.) Cohen JT, Postma GN, Gupta S, Koufman JA. Hemicricoidectomy as the primary diagnosis and treatment for cricoid chondrosarcomas. Laryngoscope 2003; 113(10): 1817-19.

(19.) Gripp S, Pape H, Schmitt G. Chondrosarcoma of the larynx: The role of radiotherapy revisited--a case report and review of the literature. Cancer 1998;82(1): 108-15.

(20.) Dailiana T, Nomikos P, Kapranos N, et al. Chondrosarcoma of the larynx: Treatment with radiotherapy. Skeletal Radiol 2002;31(9):547-9.

(21.) Hoffer ME, Pribitkin E, Keane WM, Atkins JP. Laryngeal chondrosarcoma: Diagnosis and management. Ear Nose Throat J 1992;71(12):659-62.

Stella Lee, MD; Anthony Sparano, MD; Paul Zhang, MD; Natasha Mirza, MD

From the Department of Otorhinolaryngology-Head and Neck Surgery, Hospital of the University of Pennsylvania, Philadelphia.

Reprint requests: Natasha Mirza, MD, Department of Otorhinolaryngology-Head and Neck Surgery, Hospital of the University of Pennsylvania, 5 Ravdin, 3400 Spruce St.. Philadelphia, PA 19104. Phone: (215) 662-2979: fax: (215) 662-4182: e-mail: Natasha. Mirza@uphs.upenn.edu

Originally presented as a poster at the Eastern Section meeting of the Triological Society; Jan. 28, 2005: Washington, D.C.
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Author:Lee, Stella; Sparano, Anthony; Zhang, Paul; Mirza, Natasha
Publication:Ear, Nose and Throat Journal
Article Type:Clinical report
Date:Dec 1, 2007
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