There are several phrases that people live in dread hearing from their doctor. One such phrase is “We think you have a tumor”. Tumors are considered synonymous with cancer to many people. However there are many cases where the tumour is considered “benign” or non cancerous./ People diagnosed with tenosynovial giant cell tumors are one such group.
Since the name is quite the mouthful, tenosynovial giant cell tumors is often abbreviated to TSGCTs. These tumors occur in the tissues surrounding the vital aspects of the joints like the bones and tendons. Specifically, these tumors originate in the bursae, tendon sheath and the synovium that covers them. The tumors occur in this synovium and then can push against the tendons and joints. While non cancerous, TSGCTs can be very uncomfortable and cause degenerative damage to joints if it goes untreated. Since it’s a rare condition, it often goes misdiagnosed. Knowing more information about TSGCTs is a good start to getting a proper diagnosis.
Symptoms for people suffering from TSGCTs are going to depend on the location of the tumors. There’s actually two subsets of TSGCTs. The first is pigmented villonodular synovitis (PVNS) which used to be known as diffuse-type PVNS. In most cases, symptoms are going to show up around a persons’ hips and knees. Sometimes in the ankles, shoulders, jawbones or spine. Symptoms are typical of many joint issues. The area will swell and probably feel warm or tender. Pain is common and sometimes the joints seem like they lock together and don’t function correctly upon request. These symptoms start slowly and progress as the tumor gets worse.
The second type is giant cell tumors of the tendon sheath (GCTTS). These tumors are found in the small joints located in the hands and feet. While these can cause swelling, it’s often without pain. The joints do get that “locking” feeling of not moving sometimes, and the joint can become unstable.
The primary treatment option for tenosynovial giant cell tumors is surgical. Tumors that are located on the tendon sheath have the highest successful surgery rates and recur the least. How the surgery will progress depends on where the tumor(s) is located and how far along it is. The ideal surgical procedure will remove the tumor and part of the synovium lining. In some cases, partial removal is not possible and the entire synovium must be removed. Surgery can be performed as both open, or arthroscopic based on the comfort of the surgeon.
In some cases, radiation therapy is also used with tenosynovial giant cell tumors. This can be added to the surgical option if there is a fear of tumors returning to the joints after the surgery has been completed.
Tumors are caused when cells grow at a rapid rate for some reason. In this case, the cells within a TSGCT are found to have one specific issue. Some of the cells are found to have what’s known as chromosome translocation. To boil it down for easy understanding, some of the structure of the chromosome has rearranged. Typically it will cause unexpected results. For this rare disease, it’s found that the new cells end up over producing CSF-1. This is a protein that ends up attracting other cells from the body to the region, which grow together to form the tumor. It’s a slightly different methodology from how many other tumors are formed, but still has similar results.
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