Dr. Francisco Bengoa
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Soft-tissue tumors

Sarcoma with BCOR alteration

Soft tissueMalignant / sarcomaUndifferentiated small round cell sarcomas

Epidemiology

A group of rare sarcomas defined by BCOR alterations, presenting in children, adolescents, or young adults. It is one of the round cell sarcomas defined by genetics, so the diagnosis depends on molecular studies.

Basic features

It can involve soft tissue or bone and requires molecular confirmation so it is not confused with other sarcomas.

Usual treatment

Management is usually multimodal and is tailored to the site, extent, and pediatric or adult sarcoma protocols. Before chemotherapy starts it is worth discussing fertility preservation with the team, because some of these treatments can affect it and the options have to be raised beforehand.

Outcomes and prognosis

It is an aggressive sarcoma that requires multimodal treatment; its prognosis is usually somewhat better than that of CIC-rearranged sarcoma and varies according to the subtype, the extent, and the response to treatment.

Warning signs

These signs do not necessarily mean cancer, but they warrant evaluation before operating on or needling a mass.

  • A soft-tissue mass that grows, is larger than 5 cm, or is deep.
  • Persistent, night-time or progressive bone pain, especially if it limits activity.
  • A fracture from minimal trauma, or an aggressive bone lesion on an X-ray.
  • A lump that comes back after previous surgery.
  • Neurological symptoms, rapid swelling, or pain associated with a mass.

This information is general and does not replace a medical evaluation. A musculoskeletal tumor must be interpreted together with imaging, physical examination, biopsy when appropriate and each patient's context.

Specialist evaluation

If you have a mass, a bone lesion or a report suggesting a tumor, book an evaluation.

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