Sarcomas of the larynx are exceedingly uncommon, of which primary laryngeal osteosarcomas are the rarest. To date, 25 cases of primary laryngeal osteosarcomas have been reported in the literature. Laryngeal osteosarcomas may closely simulate sarcomatoid carcinomas, since both entities share clinical, histological and immunohistochemical features. Herein, we report a case of primary laryngeal osteosarcoma in a 55 year old male, focusing on the importance of light microscopic, immunohistochemical and ultrastructural features in accurately establishing the diagnosis. In addition, the current paper provides a review of the English language literature on the subject. Laryngeal osteosarcomas usually carry a dismal prognosis with no general consensus on the most effective mode of therapy. Similarly, the current patient developed bilateral lung metastases 8 months after the initial surgery.Keywords Laryngeal osteosarcoma Á Sarcomatoid carcinoma Á Immunohistochemistry Á Electron microscopy Case Report A 55 year old male presented at the Division of Otorhinolaryngology, Tygerberg Hospital, Cape Town, South Africa, with a 4 months history of dysphonia and mild dyspnoea on exertion. He elicited a smoking history of 2 years, a habit that ceased 28 years ago. His medical history was otherwise unremarkable and no palpable cervical lymph nodes were detected on clinical examination. Direct laryngoscopy demonstrated a solid ulcerating polypoid lesion at the level of the right vocal cord (Fig. 1). Computerized tomography (CT) scans revealed an ovoid soft tissue mass of the right glottis with increased peripheral density. The lesion occupied the laryngeal vestibule with supraglottic extension (Fig. 2a, b).Histological examination of an incisional biopsy performed at direct laryngoscopy revealed a malignant neoplasm composed predominantly of atypical spindled cells and osteoid. Due to the small size of the biopsy, it was not possible to offer an exact diagnosis. The microscopic features and the macroscopic finding of a polypoid glottic lesion were nevertheless highly suggestive of a sarcomatoid (spindle cell squamous cell) carcinoma; however, due to the lack of a squamous component, a primary laryngeal osteosarcoma could not be excluded. A chest radiograph was taken which showed no abnormalities. The patient was subsequently lost to follow up for several months. Eventually, 7 months after the initial laryngoscopy, the patient underwent a total laryngectomy with wide surgical margins.Macroscopic examination of the resected specimen disclosed a 60 9 45 9 45 mm ulcerating polypoid lesion

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