Abstract
Aim
Non-epithelial tumors of the larynx are rare and encompass a wide range of pathology. We present the decade-long experience of a single institution to define clinical presentations and outcomes.
Material and methods
This is a ten year retrospective chart review of a tertiary head and neck cancer center. Index patients were identified from a review of a pathology database, and patient demographics, presenting signs and symptoms, treatment modalities, and clinical outcomes were extracted from electronic medical records. Epithelial tumors (squamous cell carcinoma, spindle cell carcinoma, and salivary tumors), granulomas, sarcoidosis, papilloma, and amyloidosis were all excluded.
Results
Twenty-four patients with ages ranging from 2 months-old to 84 years were identified. Malignant lesions (11) included chondrosarcoma (6), Kaposi’s sarcoma (2), metastatic melanoma, synovial cell sarcoma, and T cell neoplasm. Six were operated upon endolaryngeally, but four required either upfront or salvage total laryngectomy. Two received adjuvant therapy. Benign lesions (13) included hemangioma (4), granular cell tumor (3), myofibroblastic tumor (2), schwannoma (2), chondroma, and ossifying fibromyxoid tumor. Nine underwent endolaryngeal operations, and four were managed medically or with observation. None have required aggressive open resection or total laryngectomy.
Conclusion
Treatment approach of non-epithelial tumors of the larynx depends on the site and extent of the tumor, histology, and sensitivity of adjuvant therapy. Benign tumors can be managed without need for aggressive resection thereby sparing laryngeal function.
1
Introduction
Benign and malignant laryngeal neoplasms are almost exclusively epithelial in origin. The most frequently occurring malignant (squamous cell carcinoma), premalignant (squamous dysplasia), and benign (papilloma) lesions of the larynx all arise from the squamous epithelium which covers the true vocal cords. Even rare forms of laryngeal malignancies such as spindle cell carcinoma and salivary gland tumors are epithelial in origin. However, there is a small subset of laryngeal tumors that are non-epithelial in origin, arising from a diversity of cell types, including cartilage, muscle, or blood vessels. These tumors are usually reported as small series or isolated cases, making it difficult to assess incidence and management strategies.
In this study, we aim to present our experience with non-epithelial neoplasms over a decade at a large cancer to provide data on the incidence, management, and outcomes for these rare tumors. We further aim to provide a guide for otolaryngologists in their approach to these rare tumors by reviewing the literature to discuss approaches and treatment strategies for rarely encountered pathologies.
2
Materials and methods
In an IRB-approval protocol, the pathology archives of the Johns Hopkins Hospital and clinical records of all patients with laryngeal lesions were reviewed to identify non-epithelial laryngeal tumors between January 2004 and December 2013. Age, gender, presenting symptoms, location of the tumor, pathology, treatment modality (surgery, radiation and/or chemotherapy), and disease status were then extracted from the patient charts. Epithelial tumors (squamous cell, spindle cell, and salivary tumors), granulomas, sarcoidosis, cystic lesions, papilloma, and amyloidosis were all excluded.
2
Materials and methods
In an IRB-approval protocol, the pathology archives of the Johns Hopkins Hospital and clinical records of all patients with laryngeal lesions were reviewed to identify non-epithelial laryngeal tumors between January 2004 and December 2013. Age, gender, presenting symptoms, location of the tumor, pathology, treatment modality (surgery, radiation and/or chemotherapy), and disease status were then extracted from the patient charts. Epithelial tumors (squamous cell, spindle cell, and salivary tumors), granulomas, sarcoidosis, cystic lesions, papilloma, and amyloidosis were all excluded.
3
Results
Seven hundred seventy eight (778) patient records were identified with laryngeal tumors over the decade-long study period. When all epithelial tumors (squamous cell carcinoma, spindle cell, and salivary tumors) were excluded, twenty-four patients were identified with non-epithelial tumors ( Table 1 ).
| Age | Gender | Histology | Tumor location | Tobacco | Clinical presentation | Initial treatment | Operative technique | Status | Follow-up period | |
|---|---|---|---|---|---|---|---|---|---|---|
| Malignant tumors | ||||||||||
| 1 | 79 | M | Chondrosarcoma | Cricoid | Y | Dysphonia | Surgery | TL | NED | 2 y |
| 2 | 84 | M | Chondrosarcoma | Cricoid | N | Dyspnea | Biopsy | Trach, declined Tx | LTF | LTF |
| 3 | 65 | M | Chondrosarcoma | Cricoid | N | Dysphonia | Surgery | Endo | Recurrence, salvage TL | 5 y |
| 4 | 54 | F | Chondrosarcoma | Cricoid | Y | Dysphonia | Surgery | Endo debulking | AWD | 4 mo |
| 5 | 78 | M | Chondrosarcoma | Cricoid | NI | Dyspnea | Surgery | TL | Recurrence, AWD | 4 y |
| 6 | 49 | M | Chondrosarcoma | Cricoid | N | Dysphonia | Surgery | PL | Recurrence, salvage TL | 7 y |
| 7 | 41 | M | Kaposi’s sarcoma | Subglottic | Y | Dyspnea | Surgery – RT/CT | Endo | DOC | 10 y |
| 8 | 43 | M | Kaposi’s sarcoma | Supraglottic | Y | Globus | Surgery | Endo | NED | 1 y |
| 9 | 48 | M | Synovial cell sarcoma | Thyroid cartilage | NI | Dysphonia | RT/CT | – | LTF | LTF |
| 10 | 49 | F | Metastatic Melanoma | Supraglottic | N | Dysphonia, dyspnea | Surgery | Endo | DOD | 3 mo |
| 11 | 60 | F | T cell neoplasm | Transglottic | N | Dysphonia, dyspnea | Biopsy | – | AWD | 1.5 y |
| Benign tumors | ||||||||||
| 12 | 2 mo | F | Hemangioma | Subglottic | N | Stridor | Observation | – | Stable | 5 y |
| 13 | 2 mo | F | Hemangioma | Subglottic | N | Stridor | Propranolol | – | Stable | 1 y |
| 14 | 51 | F | Hemangioma | Supraglottic | N | Dyspnea | Surgery | Endo | Stable | 7 y |
| 15 | 32 | M | Hemangioma | Transglottic | N | Dyspnea | Surgery | Endo, Trach | Stable | 7 y |
| 16 | 11 | F | Granular cell tumor | Glottic | N | Dysphonia | Surgery | Endo | NED | 3 y |
| 17 | 40 | F | Granular cell tumor | Glottic | Y | Incidental finding | Biopsy | – | LTF | LTF |
| 18 | 54 | F | Granular cell tumor | Glottic | NI | Dysphonia | Surgery | Endo | LTF | LTF |
| 19 | 15 | F | Myofibroblastic tumor | Glottic | N | Dysphonia | Surgery | Endo | NED | 3 y |
| 20 | 8 | M | Myofibroblastic tumor | Glottic | N | Dysphonia | Surgery | Endo | NED | 1.5 y |
| 21 | 29 | F | Schwannoma | Subglottic | NI | Dyspnea | Surgery | Endo | Recurrence, Serial Endo | 14 y |
| 22 | 47 | F | Schwannoma | Supraglottic | N | Dysphonia | Surgery | Endo | NED | 6 mo |
| 23 | 62 | F | Chondroma | Cricoid | N | Incidental finding | Biopsy | – | Stable | 1 y |
| 24 | 73 | M | Ossifying fibromyxoid tumor | Supraglottic | N | Dysphonia | Surgery | Endo | Recurrence | 6 mo |
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