Synovial sarcoma of the maxillary sinus: an extremely rare case with excellent response to chemotherapy

Abstract

This paper presents an extremely rare case of synovial sarcoma arising from the maxillary sinus, which resulted in a clinically complete response to chemotherapy. Synovial sarcoma is a rare soft tissue malignant tumor, most commonly affecting the extremities. While ~10% occur in the head and neck region, synovial sarcoma of the sinonasal tract is extremely rare, with only 11 cases having been reported previously. As with other sarcomas, the standard treatment is complete resection while allowing for a safe margin, but this is often difficult in the head and neck area due to the complicated anatomy there. This makes the treatment of head and neck sarcoma challenging and leads to the need for a multimodal approach in advanced cases. However, the exact efficacy of chemotherapy is not well understood. In this report, we present a case of unresectable maxillary sinus synovial sarcoma that was successfully treated by chemotherapy followed by radiation therapy. A 53-year-old Japanese man was referred to our hospital with a history of left nose obstruction over the previous couple of years. Computed tomography/magnetic resonance imaging revealed a tumor arising from the maxillary sinus that extended to adjacent tissues. A biopsy was performed, and the tumor was diagnosed as synovial sarcoma. Since the tumor was unresectable, neoadjuvant chemotherapy was administered. The response was excellent, and the tumor became undetectable under endoscopy and radiological imaging. This provided us with a clinical evaluation of “complete response”. The treatment was concluded with definitive radiotherapy and two more cycles of adjuvant chemotherapy. The patient remains free of disease 12 months after treatment. Synovial sarcoma of the head and neck is a rare entity; complete resection is the treatment of choice but (neo)adjuvant chemotherapy can be considered in unresectable cases, as we show here in the present case.

Efficacy of combination-chemotherapy with pirarubicin, ifosfamide, and etoposide for soft tissue sarcoma: a single-institution retrospective analysis

“…However, because most patients with soft tissue sarcoma were pretreated with other chemotherapies, including anthracyclines, the definite evaluation in soft tissue sarcoma was suspended [ 15 ]. Since then, there have been various case reports or case series analysis that indicated preferable outcomes with THP-based combination chemotherapy [ 16 – 19 ]. Thus, a randomized controlled trial for the usage of THP-based chemotherapy will be needed to gain future approval for soft tissue sarcoma.…”

Efficacy of combination-chemotherapy with pirarubicin, ifosfamide, and etoposide for soft tissue sarcoma: A single-institution retrospective analysis

“…However, because most patients with soft tissue sarcoma were pretreated with other chemotherapies, including anthracyclines, the de nite evaluation in soft tissue sarcoma was suspended (14). Since then, there have been various case reports or case series analysis that indicated preferable outcomes with THP-based combination chemotherapy (15,16,17,18). Thus, a randomized controlled trial for the usage of THP-based chemotherapy will be needed to gain future approval for soft tissue sarcoma.…”

Synovial sarcoma of the maxillary sinus and chest wall: An exceptional association of two synchronous rare locations, Case report

“…Synovial sarcoma is a high-grade histological variety of sarcomas [ 1 , 12 ]. It's a rare soft tissue malignant tumor accounting for 5%–10% of all adult soft-tissue sarcomas [ 3 , 4 , 6 , 9 ]. it can occur at any age and everywhere in the body [ 3 , 9 ].…”

“…It's a rare soft tissue malignant tumor accounting for 5%–10% of all adult soft-tissue sarcomas [ 3 , 4 , 6 , 9 ]. it can occur at any age and everywhere in the body [ 3 , 9 ]. the extremities in young adults are the most commonly affected sites [ 1 , 5 , 6 ], the cervico-facial localization is rare and represents less than 10% of malignant mesenchymal tumours [ 5 , 8 ].…”

“…the extremities in young adults are the most commonly affected sites [ 1 , 5 , 6 ], the cervico-facial localization is rare and represents less than 10% of malignant mesenchymal tumours [ 5 , 8 ]. Therapeutic management is multidisciplinary, based on surgery and radiotherapy for localised forms and chemotherapy for extensive forms [ 8 , 9 ].…”