Managing one of the rarest: Recurrent parachordoma

Parachordoma is one of the rarest tumors identified, with around fifty cases reported to date. It is reported to have a benign behavior, yet metastatic and fatal cases have been reported. A 63-year-old female patient presented with a subcutaneous mass in the right deltoideal region. After two excisional biopsies with tumor-free surgical margins and 33 cycles of radiotherapy, the case presented with a second recurrence. Due to malignant features in the second histopathological examination, she was treated with excision of the deltoid muscle, and the defect was reconstructed with latissimus dorsi myocutaneous flap. There is no standardized treatment protocol for parachordoma. Yet, increased mitotic activity and atypical mitotic figures arise suspicion for recurrent and malignant behavior. In the presence of these features in histopathological examination, tumor should be considered as low-grade sarcoma and be treated accordingly.