Total Cricoidectomy in the Treatment of Laryngeal Chondrosarcomas

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1 The Laryngoscope VC 2011 The American Laryngological, Rhinological and Otological Society, Inc. Total Cricoidectomy in the Treatment of Laryngeal Chondrosarcomas Marco de Vincentiis, MD; Antonio Greco, MD; Massimo Fusconi, MD; Giulio Pagliuca, MD, PhD; Salvatore Martellucci, MD; Andrea Gallo, MD, PhD Objectives/Hypothesis: Our goal was to describe a total cricoidectomy, a laryngeal-preserving procedure for the treatment of low-grade chondrosarcomas of the larynx. These extremely rare cartilaginous tumors arise in the cricoid cartilage in most cases. Although these are slow-growing and rarely metastasizing tumors, large chondrosarcomas of cricoid cartilage are generally treated with total laryngectomy. An oncologically radical but function-preserving approach would therefore be preferable. Study Design: Case series. Methods: Three consecutive patients with low-grade chondrosarcomas of the larynx underwent total cricoidectomy from 1996 to An end-to-end anastomosis between the remaining larynx and the trachea was performed to restore the continuity of airways. In all cases the laryngeal lumen was stented using a Montgomery T-tube. Oncologic and functional results were observed during at least 6 years of follow-up. Results: No evidence of tumor recurrence was detected during follow-up. One month after surgery, all patients were able to tolerate a soft diet and to speak satisfactorily. One patient was ultimately decannulated, and two patients still have a tracheostomy. However, even nondecannulated patients were able to keep the tracheostoma closed for most of the time, maintaining good phonatory and swallowing functions. Conclusions: Total cricoidectomy with thyrotracheopexy may avoid the need for total laryngectomy in low-grade chondrosarcomas of the cricoid cartilage. Key Words: Cricoidectomy, thyrotracheopexy, chondrosarcoma. Level of Evidence: 4. Laryngoscope, 121: , 2011 INTRODUCTION Chondrosarcomas are rare tumors accounting for 0.2% of all head and neck malignancies and 1% of all laryngeal tumors. Low-grade chondrosarcomas are the most common, whereas moderate and high-grade tumors are less common, and the mesenchymal variety is extremely rare. Approximately 70% of chondrosarcomas of the larynx arise from the posterior lamina of the cricoid cartilage. 1 Low-grade chondrosarcomas grow slowly, rarely metastasize, and may show a clinical course that is similar to chondromas. However, chondrosarcomas are prone to local recurrence when not treated with radical surgical procedures. Because of their indolent behavior, these tumors may easily be overlooked and may reach considerable size before correct diagnosis. The most frequent From the Department of Sensorial Organs, Otorhinolaryngology Section (M.D.V., A.G., M.F.) and Department of Surgical Biotechnologies and Science, Otorhinolaryngology Section (G.P., S.M., A.G.), Sapienza University of Rome, Rome, Italy. Editor s Note: This Manuscript was accepted for publication July 25, This study was presented as a podium presentation at the American Laryngological Association Combined Otolaryngological Spring Meeting, Chicago, Illinois, U.S.A., April 27 28, The authors have no funding, financial relationships, or conflicts of interest to disclose. Send correspondence to Andrea Gallo, MD, PhD, Via Adolfo Venturi 19, Rome, Italy. andrea.gallo@uniroma1.it DOI: /lary symptom is dysphonia and, less often, dyspnea due to mechanical obstruction of the airway. 2 Surgery is the standard treatment for these cartilaginous tumors. Although there is general consensus toward a conservative surgical approach for chondrosarcomas of the thyroid, arytenoids, and epiglottis because these tumors are easily resectable, the treatment of cricoid chondrosarcomas has often been debated. 3 The cricoid ring plays a pivotal role in supporting the glottis plane, and complete removal of the entire tumor while maintaining the structural and functional integrity of the larynx is challenging. 3,4 For this reason, total laryngectomy remains the most practiced procedure for large chondrosarcomas involving cricoid cartilage. 5 The aim of this study was to describe the surgical technique of a laryngeal-preserving procedure as an alternative to total laryngectomy in the treatment of lowgrade chondrosarcomas localized in the posterior lamina of cricoid cartilage. The oncologic and functional results of such a procedure are reported. MATERIALS AND METHODS Total Cricoidectomy and Thyrotracheopexy: Surgical Technique The procedure was conducted with general anesthesia. The patient s neck was slightly extended and a shoulder roll was placed. A U-shaped incision of skin, subcutaneous tissue, and platysma muscle was made about 2 cm from the sternal 2375

2 Fig. 1. Total cricoidectomy and thyrotracheopexy: a schematic representation. (A) Normal anatomic relationships between laryngeal cartilage and trachea. (B) Cricothyroid and cricoarytenoid joints were transected and cricoid cartilage was removed. (C) The remaining larynx was approximated to the trachea. notch. To spare the vascularization of the tracheal rings irrigated by the subthyroid perforating arterioles, a subisthmic tracheotomy was performed. 6 A broad-based subplatysmal flap was then raised to about 2 cm above the hyoid bone and suspended. Sternohyoid and thyrohyoid muscle were dissected at the level of the hyoid bone and then moved down. The isthmus of the thyroid gland was identified, then the pretracheal fascia was identified and dissected to release the cervicomediastinal tract of the trachea. An important step was the preparation of the thyroid cartilage and the sectioning of the pharyngeal constrictor muscle from its posterior edge, from the superior cornu to the inferior cornu. After exposure of the cricothyroid joint, the inferior cornu of the thyroid cartilage was bilaterally disarticulated from the cricoid cartilage. Then, the articulations between the arytenoid cartilage and the cricoid were transected. Cricothyroid, posterior cricoarytenoid, and lateral cricoarytenoid muscles were sacrificed. After the complete release from the remaining larynx, cricoid cartilage was dissected by the first or the second tracheal ring and then removed (total cricoidectomy). The rest of the thyroid cartilage and arytenoid mucosa were approximated to the trachea with 2-0 absorbable sutures, and an end-to-end anastomosis was run (thyrotracheopexy) (Fig. 1). Finally, a Montgomery T-tube was inserted through the tracheostoma to stent the lumen in order to minimize airway stenosis at the thyrotracheopexy level. Patients In the Otorhinolaryngology Section of the Sensorial Organs Department of our institution, three patients with lowgrade chondrosarcoma of cricoid cartilage were treated from 1996 to The first two patients were males and the last one was a female, aged 60, 63, and 64 years, respectively. All the patients presented with slight dysphonia, which worsened, and mild dyspnea starting at least 6 months before. In all cases fiberoptic examination revealed a smooth subglottic swelling covered by intact mucosa. Cordal mobility was normal in the first two cases, whereas the examination showed impaired mobility of the left vocal cord in the last one. In all cases, computed tomography (CT) imaging showed the presence of round lytic lesions on the posterior lamina of cricoid cartilage with coarse calcifications (Fig. 2). The maximum diameters of the tumors were 3.2, 3.5, and 2.8 cm. None of the patients had laterocervical adenopathies or involvement of other laryngeal cartilage at CT imaging. Magnetic resonance imaging (MRI) showed a very high signal intensity in T2-weighted sequences owing to a high water content. Both CT and MRI revealed features that were indicative of cartilaginous neoplasms and suggestive of laryngeal chondrosarcomas. A low-grade chondrosarcoma diagnosis was made through a biopsy performed during direct laryngoscopy with general anaesthesia. During hospitalization, patients underwent thorough evaluation of risk factors and comorbidities before surgical treatment. Only one patient (patient 1) was experiencing slight hypertension and type 2 diabetes (with a good glycemic control). After appropriate counseling, the patients underwent total cricoidectomy with thyrotracheopexy. In all cases the Montgomery T-tube was removed 90 days after surgery, and a fenestrated tracheal cannula was left in place. Patient respiration was evaluated after plugging the tracheotomy tube to monitor changes in the inner diameter of the airways at the pexy level. The plug occluded the proximal end of the outer cannula, forcing the patients to breathe through the fenestrations and the upper airway tract during the weaning process, which lasted 10 days during everyday life and nighttime rest. A CT scan and a flexible fiberoptic examination were performed in this phase to evaluate airway patency. When the patient was unable to tolerate the plugged cannula, a revision of anastomosis with CO 2 laser removal of the scar tissue under direct laryngoscopy, followed by T-tube insertion, was planned. Assessment of laryngeal motility and evaluation of swallowing and voice were regularly performed during follow-up. Vocal cord motility was evaluated through laryngoscopy using rigid or flexible endoscopes. Assessment of swallowing was obtained according to our protocol for partial laryngectomy, 7 which includes: 1) a patient self-assessed questionnaire about difficulties during swallowing, 8,9 2) a standard fiberoptic endoscopic evaluation of swallowing (FEES), and 3) videofluoroscopy when laryngeal penetration or tracheal aspiration were detected at FEES. 2376

3 Fig. 2. (A, B) Preoperative computed tomography images (axial and coronal sections). The tumor arising in cricoid posterior lamina on the left side involves more than half the cricoid ring. (C) Surgical specimen. The arrow indicates the tumor in the posterior lamina of the cricoid cartilage. Clinical assessment of vocal function was based on subjective criteria and on the instrumental analysis of physical acoustic parameters. Computer analysis of the voice was made based on open and isolated phonation of the sustained vowel /a/ picked up by an AKG model C410 professional microphone (AKG, Vienna, Austria) at a standard 5-cm distance from the patient. The vocal signal was recorded and analyzed using the KAY Computer Speech Lab model 4300B (Kay Elemetrics Corp., Lincoln Park, NJ), assisted by a personal computer with CSL 5.0 basic software. All subjects were trained to voice a vocal sample of a sustained /a/, at a conversational voice intensity, as constantly as possible, for the maximum phonation time (MPT). The following acoustic measurement parameters were recorded in this study: fundamental frequency, the perturbation quotients jitter (variations of frequency [Jitt%]) and shimmer (variations of amplitude [Shim%]), and noise measurements (noise-to-harmonics ratio [NHR]). Regarding the subjective evaluation, each patient was invited to fill out the Italian translation of voice handicap index (VHI) form during the follow-up consultation to achieve self-assessment data on the perceived quality of life. 10 RESULTS In all cases the final histologic examination on the entire specimen confirmed the diagnosis of low-grade chondrosarcoma of cricoid cartilage. Presently all the patients are alive and in good health. No major peri- or postoperative complications occurred. No recurrence of disease was observed during follow-up. As far as the functional results are concerned, following Montgomery T-tube removal, only one patient reported being able to keep the tracheal cannula plugged night and day without experiencing breathing difficulties (Table I). The remaining two patients, although capable of tolerating tracheostoma closure for most of the day, experienced a slight dyspnea during nighttime rest. Postoperative CT and fiberoptic examination confirmed normal airway patency recovery in the patient without dyspnea, whereas a fair amount of scar tissue was detected at the thyrotracheal anastomosis level in patients with breathing difficulties (Fig. 3). Only the patient with normal respiration was permanently decannulated, whereas surgical revision of the stenotic tract was performed in the others. As described in the methods section, surgical revision consisted of scar tissue removal by means of CO 2 laser performed during direct laryngoscopy with general anaesthesia. Although an adequate size of respiratory tract was achieved, a Montgomery T-tube was reinserted at the end of the TABLE I. Demographic Features of the Sample. Patients Sex Age, yr Size of Chondrosarcoma, cm Definitive Decannulation Further Surgical Procedures Follow-Up, yr 1 Male No Male Yes No 13 3 Female No

4 Fig. 3. (A) Postoperative coronal computed tomography scan of the decannulated patient. Note the absence of the cricoid ring. (B) Postoperative axial computed tomography scan showing a reduction of the normal size of the airway at the glottic level. procedure and left in place for an additional 3 months. Both patients underwent several procedures without achieving appropriate airway size, thus preventing permanent decannulation (Table I). Nevertheless, they were able to breathe and speak with the tracheal cannula kept closed most of the time, removing the plug only while sleeping or during sporadic episodes of difficult respiration. One out of these two patients underwent three procedures of surgical revision and the other underwent four; however, both refused to continue to try reestablishing airway patency. The endoscopic examination of laryngeal motility performed approximately 10 days after Montgomery T-tube removal showed several peculiarities in the morphology and function of the larynx, similar in all patients. The vocal folds appeared normotrophic and in the paramedian position during breathing. However, in all cases it was possible to observe the shortening and the adduction (even if not complete) of the vocal folds during phonation. Although all the patients had sufficient breathing space at the glottic level, two of them (patients 1 and 2) showed a subglottic stenosis at the pexy level. The ventricular folds were symmetrically hypertrophic in all patients, and epiglottis motility turned out to be preserved. Assessment of swallowing features was achieved by means of the Leipzig and Pearson scale 8,9 and FEES. None of the patients reported aspiration after surgery nor did they experience aspiration-related problems, although all of the patients complained of occasional cough during meals, in absence of clinical problems, in the first 2 months after surgery. None of the subjects had FEES suggestive for aspiration. All the subjects were able to tolerate a normal diet. The mean value of the acoustic parameters and the single measures for every subject are reported in Table II. The speech ability was preserved in all cases, and the mean of VHI scores evaluated during follow-up was DISCUSSION Surgery is considered the treatment of choice for laryngeal cartilaginous tumors, and only in the last decade has exclusive radiotherapy been proposed as an alternative approach in patients for whom surgery is contraindicated due to clinical conditions. 11 Laryngeal chondrosarcomas do not respond to chemotherapy, which is not considered a treatment option. 2 Radical removal of these tumors through larynxpreserving surgery is a widely shared therapeutic strategy because local recurrence of chondrosarcomas is common and could be associated with a progressive dedifferentiation of the tumor. 2 Nevertheless, it is generally agreed that because of the benign evolution of the disease, preservation of laryngeal function is considered to be more important than the radical extirpation of the disease. To pursue this goal, some authors suggest endoscopic laser resection as the preferred method to maintain the airway passage in cricoid chondrosarcomas. Most patients remain symptom-free for years with the possibility of repeating resection in case of residual or recurrent tumor growth. 12 Total Patients TABLE II. Values of the Acoustic Parameters and Voice Handicap Index Score. MPT, sec Fo Jitt% Shim% NHR VHI Score Mean value MPT ¼ maximum phonation time; Fo ¼ fundamental frequency; Jitt% ¼ jitter percent; Shim% ¼ shimmer percent; NHR ¼ noise-to-harmonic ratio; VHI ¼ voice handicap index. 2378

5 cricoidectomy or total laryngectomy could be considered as salvage surgery in case of tumor recurrence after repeat laser resection. Although supracricoid partial laryngectomy allows complete, organ-preserving removal of chondrosarcomas arising in the thyroid, arytenoids, or epiglottis, the management of cricoid chondrosarcomas is still controversial and much debated. Although larynx-preserving surgery should be the treatment of choice, involvement of the cricoid ring makes the radical removal of these tumors without deranging laryngeal function rather difficult. In fact, the resection of a large part of the cricoid causes the interruption of laryngeal support and collapse of the glottic plane and consequently leads to laryngeal stenosis. 2 4,13 For this reason a large, even low-grade chondrosarcoma of cricoid lamina still represents an indication for total laryngectomy. Because this procedure appears to be too disabling for the treatment of a tumor that displays such limited aggressive behavior, many authors have pursued less-invasive approaches. According to some authors, conservative treatment of cricoid chondrosarcomas through laryngofissure is considered possible when the tumor involves less than half the cartilage. 3,14,15 However, tumor debulking did not seem an appropriate treatment because it predisposes to tumor recurrence. It is a generally shared opinion that these tumors require wide excisions with removal of external perichondrium along with a margin of normal tissue to ensure complete eradication. 14 Some authors claim that an endolaryngeal thyrotomic approach allows complete removal of the chondrosarcoma, including internal and external perichondrium, and ensures the preservation of cricoarytenoid joints. Damiani and Tucker described an alternative external approach that permits tumor resection while preserving the internal perichondrium. 3 Despite the caution applied in the resection of the external perichondrium, recurrences are not rare in these cricoid-sparing techniques. To avoid tumor recurrence and multiple surgery, Cohen et al. 15 proposed a vertical hemicricoidectomy as primary treatment in patients affected by cricoid chondrosarcomas with involvement of less than half the cricoid and adequate contralateral subglottic airway. Satisfying functional and oncologic results have been reported; however, this technique seems to be indicated for limited tumors that involve only half the cricoid cartilage or less and spares the cricoarytenoid joints. Delaere et al. reported a case of cricoid chondrosarcoma involving the hemilarynx treated with a vertical hemicricoidectomy in association with a complex twostep tracheal autotransplantation procedure to restore the integrity of laryngeal support. 14 Hantzakos et al. 16 have recently described a case of a large chondrosarcoma treated with hemicricoidectomy in which reconstruction of the cricoid ring was performed using a composite hyoid-sternohyoid osteomuscular flap (Rethi- Ward technique). Although the use of autologous grafts undoubtedly makes this technique interesting, this procedure appears for the same reason to be technically insidious and difficult to repeat. The total removal of cricoid cartilage for the treatment of large chondromatous tumors was first described by Leroux-Robert 17 in 1956 and recently reproposed by Nakano et al. 4 and by Thomè et al. 18 Our series of three cases represents, to the best of our knowledge, the most extensive experience in total cricoidectomy. We performed this surgery on large chondrosarcomas arising in cricoid lamina and involving more than half of the cricoid ring with the aim of obtaining complete removal of the tumor while preserving the physiology of the larynx as much as possible. The size and location of chondrosarcomas observed in this series did not allow for CO 2 laser surgical removal of the tumor, allowing oncologic radicality. 12 Therefore, in our opinion, total cricoidectomy represented the only reasonable surgical option to avoid total laryngectomy. After cricoid removal, the reconstruction of airways was performed through a simple end-toend anastomosis between the thyroid cartilage and the tracheal rings (thyrotracheopexy) without the use of autologous grafts. The Montgomery T-tube was positioned to ensure adequate re-epithelization of the laryngeal and tracheal lumen and to attempt reducing the risk of stenosis. Our experience showed excellent results in terms of oncologic control and good functional results if compared to those obtained with total laryngectomy, which is still considered the surgery of choice by several authors in the treatment of large subglottic chondrosarcomas. Although decannulation was achieved in only one third of the cases, the remaining two patients kept their tracheostoma closed for most of the time. Many factors may be involved in decannulation failure. The formation of subglottic stenosis may be due not only to a perfectible surgical technique but also to the patient s intrinsic characteristics such as an individual tendency to form hypertrophic scars. Although comorbidities may also adversely affect the results of surgery, the patients in our series did not show collateral diseases significant enough to influence functional outcome. The endoscopic observation suggests interesting considerations about the dynamics of the residual larynx. In patients undergoing total cricoidectomy, the main support for the arytenoid cartilage, represented by cricoid lamina, is missing. Cricoarytenoid joints are also resected, and the adductor and abductor muscles of the vocal cords are disconnected from cricoidal support. As a result, the arytenoid cartilages are involved in a fibrotic process (whose extent is difficult to predict preoperatively) and are welded to the underlying tracheal rings, losing most of their mobility. However, because the recurrent laryngeal nerve is spared during surgery, the thyroarytenoid muscles maintained the ability to thicken the vocal folds and to narrow the laryngeal inlet. These changes in the physiology of the larynx, together with the hypertrophy of the ventricular folds, allowed the achievement of acceptable laryngeal functions. Despite the marked abnormalities observed during the movements of the true vocal folds, the remaining larynx was shown to have maintained good phonatory and swallowing skills. All our patients tolerated a normal oral diet without the need for supplemental tube feedings. They maintained their weight and did not complain of social impairment 2379

6 during meals. By means of FEES, none of the patients demonstrated relevant swallowing abnormality resulting in varying degrees of aspiration. In all subjects, voice analysis displayed remarkable reduction of MPT, high NHR, increase in fundamental frequency and increase in physical acoustic parameters (Jitt% and Shim%). Despite these abnormal parameters, the voice originated by the neolarynx had little influence on perceived quality of life and was considered satisfactory by the patients, who achieved comprehensible and socially acceptable communication. In our opinion the proposed technique preserved function and allowed us to achieve the same radicality and preferable functional results compared to those obtained with total laryngectomy. Although the inclusion of only three patients makes interpretation of our results difficult, we consider this procedure a useful option for patients selected for total laryngectomy. In fact, even the nondecannulated patients were able to keep the tracheal cannula closed most of the day, enabling them to experience a normal social life. CONCLUSION Total cricoidectomy with thyrotracheopexy represents a simple and viable treatment for large cricoid cartilaginous tumors. It allows the radical removal of the tumor through a larynx-preserving procedure, thus representing a valid alternative to total laryngectomy even in advanced neoplastic lesions. Acknowledgments We thank Maria Grazia Saladino for help in the manuscript preparation and Costantina Antonino for illustrations. BIBLIOGRAPHY 1. 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: Baatenburg de Jong RJ, van Lent S, Hogendoorn PC. Chondroma and chondrosarcoma of the larynx. Curr Opin Otolaryngol Head Neck Surg 2004;12: Tiwari R, Mahieu H, Snow G. Long-term results of organ preservation in chondrosarcoma of the cricoid. Eur Arch Otorhinolaryngol 1999;256: Nakano Y, Asakura K, Himi T, Kataura A. Chondrosarcoma of larynx: a case successfully reconstructed after total cricoidectomy. Auris Nasus Larynx 1999;26: Saleh HM, Guichard C, Russier M, Kemeny JL, Gilain L. Laryngeal chondrosarcoma: a report of five cases. Eur Arch Otorhinolaryngol 2002;259: de Vincentiis M, Minni A, Gallo A. Supracricoid laryngectomy with cricohyoidopexy (CHP) in the treatment of laryngeal cancer: a functional and oncologic experience. Laryngoscope 1996;106: Simonelli M, Ruoppolo G, de Vincentiis M, et al. Swallowing ability and chronic aspiration after supracricoid partial laryngectomy. Otolaryngol Head Neck Surg 2010;142: Leipzig B. Neoglottic reconstruction following total laryngectomy: a reappraisal. Ann Otol Rhinol Laryngol 1980;89: Pearson BW. Subtotal laryngectomy. Laryngoscope 1981;91: Schindler A, Ottaviani F, Mozzanica F, et al. Cross-cultural adaptation and validation of the Voice Handicap Index into Italian. J Voice 2010;24: 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: Merrot O, Gleizal A, Poupart M, Pignat JC. Cartilaginous tumors of the larynx: endoscopic laser management using YAG/KTP. Head Neck 2009; 31: Windfuhr JP. Pitfalls in the diagnosis and management of laryngeal chondrosarcoma. J Laryngol Otol 2003;117: Delaere PR, Vertriest R, Hermans R. Functional treatment of a large laryngeal chondrosarcoma by tracheal autotransplantation. Ann Otol Rhinol Laryngol 2003;112: Cohen JT, Postma GN, Gupta S, Koufman JA. Hemicricoidectomy as the primary diagnosis and treatment for cricoid chondrosarcomas. Laryngoscope 2003;113: Hantzakos A, Evrard AS, Lawson G, Remacle M. Posthemicricoidectomy reconstruction with a composite hyoid-sternohyoid osteomuscular flap: the Rethi-Ward technique. Eur Arch Otorhinolaryngol 2007;264: Leroux-Robert J. 3 cases of chondromatous tumors in lamina of cricoid cartilage treated by total or partial sub-perichondrial cricoidectomy. Ann Otolaryngol Chir Cervicofac 1956;73: Thome R, Thome DC, de la Cortina RA. Long-term follow-up of cartilaginous tumors of the larynx. Otolaryngol Head Neck Surg 2001;124: