Primary Sarcomas of the Larynx: A Clinicopathologic Study of 27 Cases

Abstract

Primary sarcomas of the larynx are rare and are associated with diagnostic and treatment challenges. Studies of these tumors are limited, and most examples have been reported as small series. To further increase our understanding of laryngeal sarcomas, we reviewed our experience of an adult cohort. A retrospective search for laryngeal sarcomas from our pathology archives and consultation files of one of the authors was performed. We studied 27 primary laryngeal sarcomas that included 25 males, and 2 females, with a mean age of 60 years (range 33-85). The cases included conventional chondrosarcoma (16), well-differentiated liposarcoma (2), clear cell chondrosarcoma (1), leiomyosarcoma (2), high grade myxofibrosarcoma (2), high grade myofibroblastic sarcoma (1), low-grade myofibroblastic sarcoma (1), malignant granular cell tumor (1), and Kaposi sarcoma (1). Data on treatment and follow-up was available in 17 and 16 cases, respectively. 12 patients underwent partial laryngeal resection; five had total laryngectomy, and the patient with Kaposi sarcoma received combined highly active antiretroviral therapy and chemotherapy. Three patients developed local recurrence, and two patients developed metastases. The remaining patients with follow up had a favorable outcome and were disease-free after treatment. The important differential diagnosis of spindle cell sarcoma is sarcomatoid squamous cell carcinoma, and their distinction often requires extensive sampling of the mucosal surface and immunohistochemical analysis. The mainstay of treatment for laryngeal sarcomas is surgical removal, with the extent dictated by tumor type and grade. Adjuvant therapy is reserved for high-grade sarcomas and may be given in a neoadjuvant or adjuvant setting.

Keywords: Chondrosarcoma; Kaposi sarcoma; Larynx; Liposarcoma; Malignant granular cell tumor; Myofibroblastic sarcoma; Myxofibrosarcoma.

Fig. 1

a Reformatted sagittal computed tomography image of the larynx demonstrates a focally calcified mass located posteriorly, causing stenosis of the lumen. b Excised chondrosarcoma is solid, lobular, and has an area that is blue-white and regions that are glistening pale tan-yellow. c Low-grade chondrosarcoma arising from chondroma. The chondrosarcoma shows increased cellularity and mild nuclear atypia juxtaposed to the chondroma that is less cellular and lacks atypia. (HES × 10). d Clear cell chondrosarcoma. Sheets of large polygonal tumor cells with abundant clear to pale eosinophilic cytoplasm closely admixed with trabeculae of metaplastic woven bone focally lined by osteoblasts (HES × 40)

Fig. 2

a Reformatted sagittal computed tomography. Fat density mass seen extends from the inferior oropharynx to the larynx just above the level of the false vocal cords. b Well differentiated liposarcoma is composed of lobules of white adipocytes that vary in size, and scattered cells had enlarged hyperchromatic nuclei. The septae are thick, collagenous, and also contain spindle cells, some of which had enlarged hyperchromatic nuclei (HES × 20)

Fig. 3

a Direct laryngoscopy shows a 1.5 cm pedunculated mass growing in the left false vocal cord. b Kaposi sarcoma is composed of hypercellular fascicles of spindle cells with intervening slit-like vascular spaces containing numerous red blood cells, scattered plasma cells, and hemosiderin deposits (HES × 40). c Immunohistochemistry shows that the tumor cells exhibit positive nuclear staining for HHV-8

Fig. 4

a Leiomyosarcoma is composed of spindle cells with elongate nuclei that have coarse chromatin and blunted ends and eosinophilic cytoplasm. The tumor cells are arranged in fascicles that intersected one another at right angles (HES × 10). b Scattered cells have enlarged pleomorphic hyperchromatic nuclei (HES × 40)

Fig. 5

a Myxofibrosarcoma is composed of malignant pleomorphic spindle cells enmeshed in a myxoid stroma that contains arching small-caliber blood vessels (HES × 20)

Fig. 6

a Reformatted coronal computed tomography demonstrates a large mass involving the left pharyngeal wall, the base of tongue, left true vocal cord, and subglottic region. b Excised malignant granular cell tumor shows glistening tan-yellow cut surface. c Sheets of spindle and polygonal cells with granular cytoplasm (HES × 40)