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Chondroid tumors of the larynx: Computed tomography findings
Chondroid Computed
Tumors of the Larynx: Tomography Findings
Steven J. Wang, MD, * Alexandra Borges, MD,? Robert B. Lufkin, MD,+ Joel A. Sercarz, MD, * and Marilene B. Wang, MD* Purpose: Chondromas and chondrosarcomas of the larynx are rare cartilaginous tumors making up less than 1% of all laryngeal tumors. Patients typically present with symptoms of hoarseness, dysphagia, or dyspnea. The most common location in the larynx for these tumors is the cricoid cartilage. Radiographically, these lesions are typically hypodense, wellcircumscribed masses containing mottled calcifications with smooth walls centered within the cartilage. Materials and Methods: We present 6 cases of chondroid tumors of the larynx. Results: One patient had a chondroma, 4 patients had low-grade chondrosarcomas, and 1 patient had an intermediate-grade chondrosarcoma. Two partial laryngeal resections and 4 total laryngectomies were performed. Conclusions: In most cases of chondroma or chondrosarcoma of the larynx, conservative surgery should be attempted, but total laryngectomy may be required for large or recurrent lesions. (Am J Otolaryngoll999;20:379-382. Copyright 0 1999 by W.B. Saunders Company)
Chondrosarcoma is a malignant tumor of cartilaginous origin, which accounts for 20% of all bone tumors in the United States.’ However, only 10% of chondrosarcomas occur in the head and neck region.2 The most common sites of chondrosarcomas of the head and neck are the larynx, the maxilla, and the base of skull.3 Chondroma and chondrosarcoma of the larynx are rare cartilaginous tumors making up less than 1% of all laryngeal tumors, with only about 250 cases reported in the 1iterature.l In this study, we present 6 cases of chondroid tumors of the larynx seen at UCLA Medical Center. Characteristic histological and radiologic findings of these tumors are important to recognize so that optimal treatment may be planned. PATIENTS AND METHODS A search of medical records at UCLA Medical Center showed 6 patients with chondroid laryngeal
From the *Division of Head and Neck Surgery and tDepartment of Radiological Sciences, UCLA School of Medicine, Los Angeles, CA. Address reorint reauests to Marilene 8. Wana. MD. Division of iead anb Neck Surgery, UCLA ti’edicai Center, CHS 62-132, 10833 Le Conte Ave, Los Angeles, CA 900951624. Copyright 0 1999 by W.B. Saunders Company 0196-0709/99/2006-0004$10.00/0 American
Journal
of Otolaryngology,
Vol20,
tumors who were treated at our institution between 1988 and 1997 (Table 1). Five patients were men, and 1 was a woman. Their average age was 65 years (range, 41 to 79 years). The radiographic findings evaluated included cartilage involvement, center of mass, and the presence of expansion. Chondroid calcifications, adenopathy, and airway compromise were noted. Histologic diagnosis was reviewed in 5 of the 6 patients.
RESULTS
All 6 patients’ tumors were located in the cricoid cartilage, with 4 located in the posterior cricoid and 2 located in the lateral cricoid. The histopathologic diagnosis of the tumors included 1 chondroma, 4 low-grade chondrosarcomas, and 1 intermediate-grade chondrosarcoma. As initial treatment, 2 patients underwent an organ-sparing partial laryngeal resection with clear margins, 1 underwent tracheotomy followed by total laryngectomy, and 3 underwent total laryngectomy as initial treatment. Among those who underwent a total laryngectomy, there were no recurrences. Among the 2 who underwent a partial laryngeal resection, one is disease-free 6 years later, and 1 had a recurrence 2% years later in his right posterior cricoid cartilage. In the latter case, it was felt that this critical portion of cricoid could not be removed alone without No 6 (November-December),
1999:
pp 379-382
379
380
WANG
TABLE 1. Patient
Patients
With
Chondroid
Tumors
Age
Date
Location
69 41
74
7/97 5/94 7/94
Posterior cricoid Posterior cricoid Lateral cricoid
79
6/91
62 65
6/88 4/84
Posterior cricoid Lateral cricoid Posterior cricoid
NED,
no evidence
1
2 3 4 5 6 Abbreviations:
ET AL
Histologic
Diagnosis
Treatment
Low-grade chondrosarcoma Low-grade chondrosarcoma intermediate-grade chondrosarcoma Low-grade chondrosarcoma Chondroma Low-grade chondrosarcoma
of recurrent
disease;
Follow-up
Total laryngectomy Laryngeal fissure Trach, then laryngectomy
NED 12/97 Recurrence 5/96 Metastatic prostate
Total laryngectomy Laryngeal fissure Total laryngectomy
NED 1 O/97 NED 12/94 Death, unrelated
CA7/97
4/91
CA, cancer.
compromising the structure of the larynx. The patient declined to have a total laryngectomy but has instead undergone at least 2 debulking procedures to maintain his airway. All patients had computed tomography (CT) scans of the neck (Figs 1 through 6). The results of the imaging are summarized in Table 2. CT typically shows a large, wellcircumscribed lesion with speckled chondroid calcifications centered over the cricoid cartilage, with some degree of airway obstruction. Adenopathy on CT was not present, or was there documentation of metastatic disease in any of the patients in our series. DISCUSSION Chondroid tumors of the larynx are more common in men than women, as reflected in our series. The peak incidence is in the sixth and seventh decade of life.4 Within the larynx, the most common location is the cricoid carti-
Fig 1. Patient 1. Axial CT scan of the larynx showing a large, heavlly calcified lesion centered in the right posterlor crlcoid cartllage, crossing the midline. The airway Is narrowed and pushed anterlorly.
lage, usually arising from the posterior or posterolateral aspect of the cricoid. Other commonly encountered sites include the thyroid cartilage, the arytenoids, and more rarely, the corniculates and epiglottis5 The most common presenting symptom is hoarseness, followed by dyspnea or dysphagia.” Chondrosarcomas tend to be slow-growing tumors, and patients may have symptoms for years before diagnosis. On examination, the tumor may appear as a subglottic bulge with intact mucosa. Ulceration is rare. Biopsy may be difficult because of the firm nature of the lesion. Vocal cord paralysis may be seen and is usually an early sign of cricoid involvement.’ Vocal cord immobility may be related to recurrent laryngeal nerve injury or interference with cricoarytenoid joint functions The histologic diagnosis of cartilaginous tumors of the larynx varies from the well-
Fig 2. Pstlent 2. Axial CT scan showing an expansile mass centered in the right posterior cricoid cartilage. The lesion is homogeneously hypodense and shows only a small perlpheral calcification near the right lateral border of the lesion. The borders of the leslon appear well circumscribed. The airway is markedly narrowed.
CHONDROID
TUMORS
OF THE
LARYNX:
CT FINDINGS
381
Fig 5. Patient 5. Axial CT scan of the larynx showlng a lesion similar to Fig 3 with areas of speckled calcification. Fig 3. Patient 3. Axial CT scan showing a small mass expanding the right cricoid cartilage. Note the absence of calcification and minimal impingement on the airway.
differentiated chondroma or low-grade chondrosarcoma to the anaplastic features of a high-grade chondrosarcoma. Distinguishing between a chondroma and a low-grade chondrosarcoma is very difficult microscopically.g Histologically, chondromas show a homogeneous, monotonous pattern with low cellularity and small, dark nuclei. Low-grade chondrosarcomas are most common (50%) and are very similar in appearance to chondromas but differ by having more darkly staining cells and
Fig 4. Patient 4. Axial CT scan showing an expansile lesion centered in the right lateral aspect of the cricoid cartilage. Note the calcified matrix and impingement on the airway.
occasional binucleated or multinucleated cells. Medium and high-grade tumors have increased cellularity and mitotic figures.3 Some investigators advocate that all neoplasms containing hyaline cartilage should be considered chondrosarcoma until proven otherwise.3 The only proof of the benign nature of the lesion is its failure to metastasize or recur after several years of follow-up or its pathological appearance on examination of the entire lesion. For this reason, a new classification has emerged, which divides the cartilaginous
Fig 6. Patient 6. Axial enhanced CT scan of the larynx: there is a large expansile lesion centered in the left cricoid cartilage. The lesion is hypodense and shows speckled calcification in its left anterior margin. The airway is narrowed and pushed to the right.
382
WANG ET AL
TABLE 2.
CT Findings
Location Pa- (Cartilage tient or Mucosa) 1 2 3 4 5 6
Cartilage Cartilage Cartilage Cartilage Cartilage Cartilage
Cartilage
Expansivity
Cricoid Cricoid Cricoid Cricoid Cricoid Cricoid
++ ++ + ++ + ++
Chon- Airway droid Corn- AdenoCa+ + promise pathy ++ + + +
40% >90%90%
-
tumors into low, intermediate, and high grade in a progressive spectrum of malignancy.10 Chondromas and chondrosarcomas cannot be distinguished on the basis of radiologic features.7 CT is the imaging study of choice for evaluating chondroid tumors of the larynx. These lesions present as hypodense, wellcircumscribed masses, with smooth walls centered within the cartilage rather than the mucosal surface. Coarse, mottled, chondroid calcifications are seen in 75% to 80% of cases. CT images show focal expansion of the involved laryngeal cartilage, causing displacement rather than invasion of adjacent structures. More aggressive imaging features, as well as nodal metastasis, are less common, but may be seen in high-grade chondrosarcomas. In our series, there was no correlation between radiographic appearance or degree of calcification and tumor histologic diagnosis. Imaging is also valuable to determine the degree of airway compromise. In our series, all patients had some degree of airway narrowing. Magnetic resonance imaging is less specific because of its insensitivity to chondroid calcifications. It can, however, aid in delineating the extent of the lesion due to its improved soft tissue contrast resolution.** Treatment is primarily surgical. Radiation therapy and chemotherapy do not appear to play a role as a primary or adjuvant therapy.7 Because chondrosarcomas are usually slowgrowing and of low metastatic potential, one should be conservative with surgical intervention. Excision of involved cartilage segments
with adequate free margins is sufficient. Although local recurrence does occur, considering the slow growth rate of the tumor, the mean age of diagnosis (63 years), and the high success rate of salvage laryngectomy (77%) v 86% for initial total laryngectomy), initial treatment should be directed towards preserving laryngeal function.7 However, in cases where gross removal of all tumor would compromise the skeletal support of the larynx, when more than half the cricoid is involved, or in high-grade lesions, a total laryngectomy may be the appropriate initial treatment. Prognosis for these slow-growing tumors is excellent, and chondrosarcoma of the larynx appears to have a better prognosis than cartilaginous tumors arising from other sites of the bodym8 REFERENCES 1. Bough ID, Chiles PJ, Fratalli MA, et al: Laryngeal chondrosarcoma: Two unusual cases. Am J Otolaryngol 16:126-131,1995 2. Mark RJ, Tran LM, Sercarz J, et al: Chondrosarcoma of the head and neck: The UCLA experience, 1955-1968. Am J Clin Oncol16:232-237,1993 3. Devaney KO, Ferlito A, Silver CE: Cartilaginous tumors of the larynx. Ann Otol Rhino1 Laryngol 194:251255,1995 4. Vangehuchten S, Dhooge I, De Potter C, et al: Chondrosarcoma of the larynx: A diagnostic and therapeutic dilemma. Acta Otorhinolaryngol Belg 49:19-23,1995 5. Koka VN, Veber F, Haguet JF, et al: Chondrosarcoma of the larynx. J Laryngol Otol109:168-170,1995 6. Browne JD: Management of nonepidermoid cancer of the larynx. Otolaryngol Clin North Am 39:215-229,1997 7. Sztern J, Sztern D, Fonseca R, et al: Chondrosarcoma of the larynx. Eur Arch Otorhinolaryngol 250:173-176, 1993 6. Nicolai P, Ferlito A, Sasaki C, et al: Laryngeal chondrosarcoma: Incidence, pathology, biological behavior, and treatment. Ann Otol Rhino1 Laryngol 99:515-523, 1990 9. Lewis JE, Olsen KD, Inwards CY: Cartilaginous tumors of the larynx: Clinicopathologic Review of 47 Cases. Ann Otol Rhino1 Laryngol io6:94-ioo,l997 10. Frobere MK. Meschrer SC. Brown RE. et al: Cartilaeinous tumors”of the larynx: A report of two cases w& definitive diagnosis by fine needle aspiration and computed tomography. Acta Cytol40:761-764,1996 11. Hediaer R, McEniff N. Karmodv C. et al: Recurrent chondrosarcoma. of the hyoid bone. Clin Imaging 21:6972,1997
Tumors of the Larynx: Tomography Findings
Steven J. Wang, MD, * Alexandra Borges, MD,? Robert B. Lufkin, MD,+ Joel A. Sercarz, MD, * and Marilene B. Wang, MD* Purpose: Chondromas and chondrosarcomas of the larynx are rare cartilaginous tumors making up less than 1% of all laryngeal tumors. Patients typically present with symptoms of hoarseness, dysphagia, or dyspnea. The most common location in the larynx for these tumors is the cricoid cartilage. Radiographically, these lesions are typically hypodense, wellcircumscribed masses containing mottled calcifications with smooth walls centered within the cartilage. Materials and Methods: We present 6 cases of chondroid tumors of the larynx. Results: One patient had a chondroma, 4 patients had low-grade chondrosarcomas, and 1 patient had an intermediate-grade chondrosarcoma. Two partial laryngeal resections and 4 total laryngectomies were performed. Conclusions: In most cases of chondroma or chondrosarcoma of the larynx, conservative surgery should be attempted, but total laryngectomy may be required for large or recurrent lesions. (Am J Otolaryngoll999;20:379-382. Copyright 0 1999 by W.B. Saunders Company)
Chondrosarcoma is a malignant tumor of cartilaginous origin, which accounts for 20% of all bone tumors in the United States.’ However, only 10% of chondrosarcomas occur in the head and neck region.2 The most common sites of chondrosarcomas of the head and neck are the larynx, the maxilla, and the base of skull.3 Chondroma and chondrosarcoma of the larynx are rare cartilaginous tumors making up less than 1% of all laryngeal tumors, with only about 250 cases reported in the 1iterature.l In this study, we present 6 cases of chondroid tumors of the larynx seen at UCLA Medical Center. Characteristic histological and radiologic findings of these tumors are important to recognize so that optimal treatment may be planned. PATIENTS AND METHODS A search of medical records at UCLA Medical Center showed 6 patients with chondroid laryngeal
From the *Division of Head and Neck Surgery and tDepartment of Radiological Sciences, UCLA School of Medicine, Los Angeles, CA. Address reorint reauests to Marilene 8. Wana. MD. Division of iead anb Neck Surgery, UCLA ti’edicai Center, CHS 62-132, 10833 Le Conte Ave, Los Angeles, CA 900951624. Copyright 0 1999 by W.B. Saunders Company 0196-0709/99/2006-0004$10.00/0 American
Journal
of Otolaryngology,
Vol20,
tumors who were treated at our institution between 1988 and 1997 (Table 1). Five patients were men, and 1 was a woman. Their average age was 65 years (range, 41 to 79 years). The radiographic findings evaluated included cartilage involvement, center of mass, and the presence of expansion. Chondroid calcifications, adenopathy, and airway compromise were noted. Histologic diagnosis was reviewed in 5 of the 6 patients.
RESULTS
All 6 patients’ tumors were located in the cricoid cartilage, with 4 located in the posterior cricoid and 2 located in the lateral cricoid. The histopathologic diagnosis of the tumors included 1 chondroma, 4 low-grade chondrosarcomas, and 1 intermediate-grade chondrosarcoma. As initial treatment, 2 patients underwent an organ-sparing partial laryngeal resection with clear margins, 1 underwent tracheotomy followed by total laryngectomy, and 3 underwent total laryngectomy as initial treatment. Among those who underwent a total laryngectomy, there were no recurrences. Among the 2 who underwent a partial laryngeal resection, one is disease-free 6 years later, and 1 had a recurrence 2% years later in his right posterior cricoid cartilage. In the latter case, it was felt that this critical portion of cricoid could not be removed alone without No 6 (November-December),
1999:
pp 379-382
379
380
WANG
TABLE 1. Patient
Patients
With
Chondroid
Tumors
Age
Date
Location
69 41
74
7/97 5/94 7/94
Posterior cricoid Posterior cricoid Lateral cricoid
79
6/91
62 65
6/88 4/84
Posterior cricoid Lateral cricoid Posterior cricoid
NED,
no evidence
1
2 3 4 5 6 Abbreviations:
ET AL
Histologic
Diagnosis
Treatment
Low-grade chondrosarcoma Low-grade chondrosarcoma intermediate-grade chondrosarcoma Low-grade chondrosarcoma Chondroma Low-grade chondrosarcoma
of recurrent
disease;
Follow-up
Total laryngectomy Laryngeal fissure Trach, then laryngectomy
NED 12/97 Recurrence 5/96 Metastatic prostate
Total laryngectomy Laryngeal fissure Total laryngectomy
NED 1 O/97 NED 12/94 Death, unrelated
CA7/97
4/91
CA, cancer.
compromising the structure of the larynx. The patient declined to have a total laryngectomy but has instead undergone at least 2 debulking procedures to maintain his airway. All patients had computed tomography (CT) scans of the neck (Figs 1 through 6). The results of the imaging are summarized in Table 2. CT typically shows a large, wellcircumscribed lesion with speckled chondroid calcifications centered over the cricoid cartilage, with some degree of airway obstruction. Adenopathy on CT was not present, or was there documentation of metastatic disease in any of the patients in our series. DISCUSSION Chondroid tumors of the larynx are more common in men than women, as reflected in our series. The peak incidence is in the sixth and seventh decade of life.4 Within the larynx, the most common location is the cricoid carti-
Fig 1. Patient 1. Axial CT scan of the larynx showing a large, heavlly calcified lesion centered in the right posterlor crlcoid cartllage, crossing the midline. The airway Is narrowed and pushed anterlorly.
lage, usually arising from the posterior or posterolateral aspect of the cricoid. Other commonly encountered sites include the thyroid cartilage, the arytenoids, and more rarely, the corniculates and epiglottis5 The most common presenting symptom is hoarseness, followed by dyspnea or dysphagia.” Chondrosarcomas tend to be slow-growing tumors, and patients may have symptoms for years before diagnosis. On examination, the tumor may appear as a subglottic bulge with intact mucosa. Ulceration is rare. Biopsy may be difficult because of the firm nature of the lesion. Vocal cord paralysis may be seen and is usually an early sign of cricoid involvement.’ Vocal cord immobility may be related to recurrent laryngeal nerve injury or interference with cricoarytenoid joint functions The histologic diagnosis of cartilaginous tumors of the larynx varies from the well-
Fig 2. Pstlent 2. Axial CT scan showing an expansile mass centered in the right posterior cricoid cartilage. The lesion is homogeneously hypodense and shows only a small perlpheral calcification near the right lateral border of the lesion. The borders of the leslon appear well circumscribed. The airway is markedly narrowed.
CHONDROID
TUMORS
OF THE
LARYNX:
CT FINDINGS
381
Fig 5. Patient 5. Axial CT scan of the larynx showlng a lesion similar to Fig 3 with areas of speckled calcification. Fig 3. Patient 3. Axial CT scan showing a small mass expanding the right cricoid cartilage. Note the absence of calcification and minimal impingement on the airway.
differentiated chondroma or low-grade chondrosarcoma to the anaplastic features of a high-grade chondrosarcoma. Distinguishing between a chondroma and a low-grade chondrosarcoma is very difficult microscopically.g Histologically, chondromas show a homogeneous, monotonous pattern with low cellularity and small, dark nuclei. Low-grade chondrosarcomas are most common (50%) and are very similar in appearance to chondromas but differ by having more darkly staining cells and
Fig 4. Patient 4. Axial CT scan showing an expansile lesion centered in the right lateral aspect of the cricoid cartilage. Note the calcified matrix and impingement on the airway.
occasional binucleated or multinucleated cells. Medium and high-grade tumors have increased cellularity and mitotic figures.3 Some investigators advocate that all neoplasms containing hyaline cartilage should be considered chondrosarcoma until proven otherwise.3 The only proof of the benign nature of the lesion is its failure to metastasize or recur after several years of follow-up or its pathological appearance on examination of the entire lesion. For this reason, a new classification has emerged, which divides the cartilaginous
Fig 6. Patient 6. Axial enhanced CT scan of the larynx: there is a large expansile lesion centered in the left cricoid cartilage. The lesion is hypodense and shows speckled calcification in its left anterior margin. The airway is narrowed and pushed to the right.
382
WANG ET AL
TABLE 2.
CT Findings
Location Pa- (Cartilage tient or Mucosa) 1 2 3 4 5 6
Cartilage Cartilage Cartilage Cartilage Cartilage Cartilage
Cartilage
Expansivity
Cricoid Cricoid Cricoid Cricoid Cricoid Cricoid
++ ++ + ++ + ++
Chon- Airway droid Corn- AdenoCa+ + promise pathy ++ + + +
40% >90%
-
tumors into low, intermediate, and high grade in a progressive spectrum of malignancy.10 Chondromas and chondrosarcomas cannot be distinguished on the basis of radiologic features.7 CT is the imaging study of choice for evaluating chondroid tumors of the larynx. These lesions present as hypodense, wellcircumscribed masses, with smooth walls centered within the cartilage rather than the mucosal surface. Coarse, mottled, chondroid calcifications are seen in 75% to 80% of cases. CT images show focal expansion of the involved laryngeal cartilage, causing displacement rather than invasion of adjacent structures. More aggressive imaging features, as well as nodal metastasis, are less common, but may be seen in high-grade chondrosarcomas. In our series, there was no correlation between radiographic appearance or degree of calcification and tumor histologic diagnosis. Imaging is also valuable to determine the degree of airway compromise. In our series, all patients had some degree of airway narrowing. Magnetic resonance imaging is less specific because of its insensitivity to chondroid calcifications. It can, however, aid in delineating the extent of the lesion due to its improved soft tissue contrast resolution.** Treatment is primarily surgical. Radiation therapy and chemotherapy do not appear to play a role as a primary or adjuvant therapy.7 Because chondrosarcomas are usually slowgrowing and of low metastatic potential, one should be conservative with surgical intervention. Excision of involved cartilage segments
with adequate free margins is sufficient. Although local recurrence does occur, considering the slow growth rate of the tumor, the mean age of diagnosis (63 years), and the high success rate of salvage laryngectomy (77%) v 86% for initial total laryngectomy), initial treatment should be directed towards preserving laryngeal function.7 However, in cases where gross removal of all tumor would compromise the skeletal support of the larynx, when more than half the cricoid is involved, or in high-grade lesions, a total laryngectomy may be the appropriate initial treatment. Prognosis for these slow-growing tumors is excellent, and chondrosarcoma of the larynx appears to have a better prognosis than cartilaginous tumors arising from other sites of the bodym8 REFERENCES 1. Bough ID, Chiles PJ, Fratalli MA, et al: Laryngeal chondrosarcoma: Two unusual cases. Am J Otolaryngol 16:126-131,1995 2. Mark RJ, Tran LM, Sercarz J, et al: Chondrosarcoma of the head and neck: The UCLA experience, 1955-1968. Am J Clin Oncol16:232-237,1993 3. Devaney KO, Ferlito A, Silver CE: Cartilaginous tumors of the larynx. Ann Otol Rhino1 Laryngol 194:251255,1995 4. Vangehuchten S, Dhooge I, De Potter C, et al: Chondrosarcoma of the larynx: A diagnostic and therapeutic dilemma. Acta Otorhinolaryngol Belg 49:19-23,1995 5. Koka VN, Veber F, Haguet JF, et al: Chondrosarcoma of the larynx. J Laryngol Otol109:168-170,1995 6. Browne JD: Management of nonepidermoid cancer of the larynx. Otolaryngol Clin North Am 39:215-229,1997 7. Sztern J, Sztern D, Fonseca R, et al: Chondrosarcoma of the larynx. Eur Arch Otorhinolaryngol 250:173-176, 1993 6. Nicolai P, Ferlito A, Sasaki C, et al: Laryngeal chondrosarcoma: Incidence, pathology, biological behavior, and treatment. Ann Otol Rhino1 Laryngol 99:515-523, 1990 9. Lewis JE, Olsen KD, Inwards CY: Cartilaginous tumors of the larynx: Clinicopathologic Review of 47 Cases. Ann Otol Rhino1 Laryngol io6:94-ioo,l997 10. Frobere MK. Meschrer SC. Brown RE. et al: Cartilaeinous tumors”of the larynx: A report of two cases w& definitive diagnosis by fine needle aspiration and computed tomography. Acta Cytol40:761-764,1996 11. Hediaer R, McEniff N. Karmodv C. et al: Recurrent chondrosarcoma. of the hyoid bone. Clin Imaging 21:6972,1997