Synovial sarcoma

Synonyms

2

Overview

A synovial sarcoma is a rare form of cancer which usually occurs near to the joints of the arm, neck or leg. It is one of the soft tissue sarcomas. It is one of the rarest forms of soft tissue cancer in the world.

The name "synovial sarcoma" was coined early in the 20th century, as some researchers thought that the microscopic similarity of some tumors to synovium, and its propensity to arise adjacent to joints, indicated a synovial origin; however, the actual cells from which the tumor develops are unknown and not necessarily synovial.

Primary synovial sarcomas are most common in the soft tissue near the large joints of the arm and leg but have been documented in most human tissues and organs, including the brain, prostate, and heart.

Synovial sarcoma occurs most commonly in the young, representing about 8% of all soft tissue sarcomas but about 15-20% of cases in adolescents and young adults. The peak of incidence is before the 30th birthday and males are affected more often than females (ratio around 1.2:1).

Symptoms

Synovial sarcoma usually presents with an otherwise asymptomatic swelling or mass, although general symptoms related to malignancies can be reported such as fatigue.

Treatment

Treatment is usually multimodal, involving surgery, chemotherapy and radiotherapy:

  • Surgery, to remove the tumor and a safety margin of healthy tissue. This is the mainstay of synovial sarcoma treatment and is curative in approximately 20-70% of patients, depending on the particular study being quoted.
  • Conventional chemotherapy, (for example, doxorubicin hydrochloride and ifosfamide), to reduce the number of remaining microscopic cancer cells. The benefit of chemotherapy in synovial sarcoma to overall survival remains unclear, although a recent study has shown that survival of patients with advanced, poorly differentiated disease marginally improves with doxorubicin/ifosfamide treatment.
  • Radiotherapy, to reduce the chance of local recurrence. The benefit of radiotherapy in this disease is less clear than for chemotherapy.