Maxillary aggressive chondrosarcoma: A rare and challenging case

Abstract

Chondrosarcomas represent 20%-30% of primary malignant bone tumors, but only about 1% occur in the head and neck region. Maxillary chondrosarcomas constitute a mere 5.76% of head and neck cases, predominantly affecting adults between the second and sixth decades of life. Symptoms, including facial swelling, pain, and nasal obstruction, often lead to delayed diagnoses and increased tumor aggressiveness. This case report details a 45-year-old Moroccan male with an aggressive maxillary chondrosarcoma presenting as a progressive left cheek swelling accompanied by significant pain and vision impairment. Imaging studies revealed a large, lytic maxillary lesion, and biopsy confirmed a low-grade chondrosarcoma. Given the tumor's unresectability, the patient underwent radiotherapy, receiving a total dose of 70 Gy with modest reduction in tumor size but ultimately developed metastatic pulmonary lesions, leading to palliative chemotherapy. Despite aggressive management, the patient succumbed after the fourth cycle of chemotherapy. This case underscores the importance of timely diagnosis and multidisciplinary collaboration in managing maxillary chondrosarcomas, highlighting the challenges posed by their aggressive nature and the need for long-term surveillance to monitor for recurrence and metastasis.

Keywords: Chondrosarcoma; Maxilla; Multidisciplinary management; Radiotherapy.

Fig. 1

Left maxillary swelling with involvement of the left nostril and inflammatory signs in the adjacent regions.

Fig. 2

Axial (A), sagittal (B), and coronal (C) facial CT images demonstrating a large, Ill-defined invasive soft tissue mass centered on the left maxillary sinus with extensive infiltration into adjacent sinuses, orbital structures, and significant osteolysis of the left hemiface (blue arrow).

Fig. 3

Axial MRI (T1-weighted with contrast [A] and diffusion-weighted imaging [C]) and sagittal section (B) Demonstrating an invasive mass centered on the left maxillary sinus with extensive soft tissue and Osseous Involvement (yellow arrow).

Fig. 4

Representative micrograph of the tumor. Tumor proliferation presents diffuse growth (A). Myxoid changes (B) and chondroid matrix (C) are observed. Tumor cells presents moderate nuclear atypia and binucleation (D) (Hematoxylin-eosin, x100).

Fig. 5

Graphical representation of treatment planning using VMAT radiotherapy.