Primary angiosarcoma of the larynx: a rare entity

Abstract

Angiosarcomas are extremely rare malignant tumours of the larynx. We present a case of laryngeal angiosarcoma in a 58-yearold man who presented with hoarseness of voice, dysphagia and neck swelling. The patient underwent a total laryngectomy with a pre-operative and frozen section diagnosis of a poorly differentiated carcinoma of the larynx. Histopathological and immunohistochemical evaluation revealed features of a laryngeal angiosarcoma. The case is presented for its rarity and diagnostic difficulty. KEywORdSAngiosarcoma -Supraglottis -Angioendothelioma Angiosarcoma is an uncommon, high grade neoplasm of vascular endothelial origin that can arise in any anatomical site of the body. Despite the fact that nearly 50% of all angiosarcomas occur in the skin and superficial soft tissues of the head and neck, they account for less than 0.1% of all head and neck malignancies. 1Livingston and Klemperer were the first to report the clinical features and histopathological characteristics of angiosarcoma.2 Jones, however, was the first to recognise the lesion as a distinct clinical entity. 3Over the years, a variety of terms have been used to describe this neoplasm, including haemangioendothelioma, angiosarcoma, haemangiosarcoma, malignant angioendothelioma and lymphangiosarcoma. Mucosal or organ-based angiosarcomas have been reported to arise in the nasal cavity, paranasal sinuses, orbit, temporal bone, middle ear, thyroid and larynx. Very few cases, however, have been described about this disease involving the larynx.4-8 Here we present a case of angiosarcoma involving the larynx with its management details in order to enhance the literature on this rare disease and its histopathological evaluation. Case historyWe report a rare case of a laryngeal angiosarcoma (LAS) in a 58-year-old man who presented at the head and neck services at our centre with a history of hoarseness, dysphagia and neck nodes. On clinical examination, endoscopy and direct laryngoscopy, a submucosal disease was revealed involving the left false vocal cord and ventricle. On computed tomography a diffuse mass involving bilateral vocal cords with erosion of the thyroid cartilage and disease extension into strap muscles with no subglottic extension of the disease was documented. Biopsy was suggestive of squamous cell carcinoma and in view of cartilage erosion and gross exolaryngeal spread of the disease, a total laryngectomy was planned.A total laryngectomy with a primary tracheo-oesophageal puncture with bilateral selective neck dissection was performed. On frozen section, the diagnosis was a poorly differentiated carcinoma with glandular differentiation. Gross examination of the histopathological specimen revealed a reddish brown tumour measuring 3cm x 2.5cm x 2cm involving the left glottis and bilateral subglottis. Underlying thyroid cartilage was grossly involved by the tumour. Microscopically, multiple sections showed a malignant tumour comprising tumour cells arranged in a vasoformative growth pattern with complex anastomosing channels an...

Nasal angiosarcoma metastatic to the larynx: Case report and systematic review of the literature

“…Angiosarcoma is a rare malignancy constituting 1% to 2% of all soft tissue sarcomas . Wanebo et al reported the occurrence of head and neck sarcomas in 214 patients, in order of decreasing frequency, in the neck, parotid, face, maxilla, mandible, paranasal sinuses, oropharynx, and larynx.…”

“…The bibliographies of relevant articles were further reviewed for other references. The variables extracted are listed in Table . Data extraction was performed by 2 of our authors (H.Q.…”

“…The English‐language peer‐reviewed literature yields 17 cases of primary laryngeal angiosarcoma, and no cases of laryngeal metastasis . Data regarding patient demographics, presentation, treatment, and outcome are reported in Table .…”