Head and Neck Juxtacortical Chondrosarcoma: A Systematic Review

Abstract

Objective: To present a case and systematically evaluate trends in clinical presentation, imaging, histopathology, and management modalities and outcomes of all head and neck juxtacortical chondrosarcoma (HNJCS) cases reported in the existing literature. Methods: We describe a rare case of HNJCS from our tertiary referral center following which the PubMed, MEDLINE, Embase, and Web of Science databases were searched for all HNJCS reports. Relevant titles and abstracts were screened. Resulting full-text articles were assessed for eligibility, and remaining studies were included for data extraction, summarization, and analysis. Results: Potential studies were identified dating from May 1946 to February 2019. A total of nine cases were included in our systematic review, eight of which were identified from full-text articles and one recruited from our tertiary referral center. The median presentation age was 41 years with a 66.7% male preponderance. The commonest presenting sign was a painless, isolated swelling after a median symptom duration of 2.5 months. CT imaging revealed hypodense lesions with peripheral enhancement and micro-calcifications. T1-weighted MRI showed hypo- to iso-intense, lobulated masses with peripheral and/or septal enhancement. The masses were diffusely hyper-intense on T2-weighted MRI. Histopathology demonstrated septated lobules of malignant hyaline cartilage with a peripheral fibrous capsule. Most tumors were low- or intermediate-grade tumors with average diameter of 4.3 cm. Local recurrence was identified in only one case (four years after initial resection). No distal and/or nodal metastases were identified. All tumors were managed by wide- or narrow-margin surgical excision. Two reports employed adjuvant treatment. There was no evidence of disease at final follow-up (median of 1.5 years). Conclusion: To the best of our knowledge, only nine cases of HNJCS have been adequately described. HNJCS have relatively consistent clinical and diagnostic profile regardless of location in the body. Surgical management yields excellent outcomes with low recurrence rates.