This information has been written for patients, their families and friends and the general public to help them understand more about a rare form of cancer known as spindle cell sarcoma. This page will detail what spindle cell sarcoma is and how this tumour type can be diagnosed and treated.

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What is Spindle Cell Sarcoma of the Bone?

Spindle cell sarcoma is a soft-tissue tumour which can start in the bone. Spindle cell sarcomas of the bone are often found in the arms, legs and pelvis. They most commonly arise in patients over the age of 40 and are extremely rare, making up just 2-5% of all primary bone cancer cases.

Spindle cell sarcoma is referred to in this way due to the appearance of its tumour cells when they are looked at under a microscope. The cells which make up a spindle cell sarcoma are long and narrow, which is known as a ‘spindle-cell shape’(1).

There are three subtypes of spindle cell sarcoma of the bone:

  • Pleomorphic Undifferentiated Sarcoma (previously known as Malignant Fibrous Histiocytoma)
  • Fibrosarcoma
  • Leiomyosarcoma

The majority of spindle cell sarcomas follow the same diagnosis and treatment methods as osteosarcoma, a more common type of primary bone cancer. (1,3)

Spindle cell sarcoma can arise in any bone, in any area of the body, though the majority of these tumours are found in the long bones of the body. These include the thigh bone (known as the femur), the area around the knee, the shin bone (known as the tibia) or the upper arm (known as the humerus)(1,2). Additionally, one type of spindle cell sarcoma, known as undifferentiated sarcoma of the bone, is quite commonly found in the pelvis and fibrosarcomas can develop in the head and neck area.