Head and Neck Low Grade Chondrosarcoma—A Rare Entity

Abstract

Chondrosarcoma represents approximately 0.1% of all neoplasms of the head and neck and is considered a rare disease with a relatively good prognosis. The 5-year overall survival (OS) rate is estimated at 70–80%, being considered a disease with a low growth rate. Approximately 13% of all cases of chondrosarcoma are located in the region of the head and neck. We present the case of a 30-year-old patient without a medical history who reported dysphagia, swallowing difficulty, neck mass sensation and dysphonia that started insidiously after an upper respiratory tract infection. Subsequently, the patient was diagnosed with a low-grade glosso-epiglottic region chondrosarcoma and was multimodally treated with surgery followed by chemotherapy and radiotherapy. The radiation treatment was delivered with a Rokus M40 former Soviet Union cobalt machine without any image guidance capabilities. The inability to obtain resection margin information justified an aggressive adjuvant treatment with chemotherapy and radiotherapy. The early loss from the oncological record without recurrence of the disease could be associated in this case with the consequence of a major complication, of which we could assume an aspiration pneumonia secondary to a dysphagia associated with an aggressive multidisciplinary treatment. Large tumor size and positive resection margins (R1 resection) are risk factors that support an intensive adjuvant approach in order to reduce the risk of recurrence, but the low grade of tumor associated with a lower risk of recurrence as well as the adverse events (AE) of adjuvant radiotherapy and chemotherapy justify a more reserved therapeutic approach. Taking into account the longer life expectancy of these patients, it is recommended to use a more conformal irradiation technique in order to reduce doses to radiosensitive structures as well as to omit elective neck irradiation, taking into account the lower risk of lymph node involvement. The lack of guidelines, which include very rare tumors including low grade chondrosarcoma of the head and neck, makes a unified approach difficult, but the data presented in case reports could contribute to choosing the regimen that offers the best therapeutic ratio.