Synovialosarcoma: a rare and difficult to diagnose tumor – A la une

2023-04-26 13:02:00

26 avril 2023

It accounts for less than 10% of soft tissue sarcomas. Synovialosarcoma is a cancer that most often affects young men and whose treatment is primarily surgical. Explanations.

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Sports journalist Matthieu Lartot has announced his withdrawal from the France Télévisions antenna. He explained on his Instagram account that he had to be treated, for the second time, for cancer in the knee. “26 years later, history unfortunately stutters. There was a 5% chance of it happening and it happened”he wrote on the social network.

Matthieu Lartot, 43, will have to have his right leg amputated. ” Some cells that had been irradiated did not disappear and a sarcoma three times larger than the first one appeared,” he said to our colleagues from Midi-Olympique.

The journalist suffers from synovialosarcoma“a rare and aggressive soft tissue tumour”, explains Orpha.net. Despite its name, the tumor does not originate in synovial tissue but is likely related to mesenchymal stem cells. According to Orpha.net, synovialosarcoma represents between 7 and 8% of malignant sarcomas. It often concerns young adults, from 15 to 45 years old, generally male.

A tumor near the large joints

This high-grade tumour, a classification due in particular to a high risk of spreading, can develop in any soft part of the body. However, it is most often found in the lower limbs, near the large joints.

“The tumor manifests as a slowly growing, deep-seated, painless mass”, continues Orpha.net. This is why the diagnosis of cancer is sometimes late. The tumor grows and eventually causes pain to patients, depending on the area where it is located.

Medical imaging examinations are essential to determine its size and precise position. A biopsy and examination of the tumor tissues are also necessary. Synovialosarcoma is also characterized by a translocation of chromosomes X and 18 which results in the fusion of genes present on these chromosomes. This specificity can help confirm the diagnosis.

A strong metastatic potential

Surgical treatment consists of removing the tumour. When the latter is deep, amputation is necessary. Chemotherapy or radiotherapy can be administered upstream to reduce the tumor and make it more accessible during resection. Surgery is usually followed by radiation therapy to treat microscopic tumor remnants.

The occurrence of metastases, most often pulmonary, is frequent in this disease. Thus, in 20% of cases, metastases are discovered in the lungs at the time of diagnosis, specifies a study in the Pan-African Medical Journal in 2014.

This strong metastatic potential coupled with the risk of recurrence makes the prognosis of this cancer difficult. This will also depend on the size of the tumor and the presence of any residual tumors despite the treatments.

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