Other names: GCTTS; Giant Cell Tumor of the Tendon Sheath; TSGCT
Tenosynovial giant cell tumors (TSGCTs) are rare, benign (not cancerous) tumors that involve the small or large joints causing pain, swelling and movement issues.
The parts within the joints that are affected include the tendon sheath (membrane that covers the tendon), the bursae (fluid-filled sacs that form a cushion between bones, tendons and muscles) and the synovial membrane (a thin membrane that is the inner layer of the joint capsule).
The growth of the tumor damages and disrupts the surrounding structures and tissues causing the pain, stiffness and reduced joint motility. If the tumor is left untreated or recurs after treatment the damage can cause significant joint issues and disability.
Types of tenosynovial giant cell tumors:
Tenosynovial giant cell tumors can be separated into four different subtypes depending exactly where in the joint it is located and whether it is localized to one area or diffuse.
Diffuse-type giant cell tumor
Pigmented villonodular synovitis (PVNS)
Intra-articular giant cell tumor of the tendon sheath
Extra-articular giant cell tumor of the tendon sheath
Cause of tenosynovial giant cell tumors:
The tumor is caused by a change within the cell chromosomes called a translocation. This results in these affected cells making too much of a protein called colony stimulating factor-1 (CSF-1). The CSF-1 protein attracts cells within the body that have a matching CSF-1 receptor including macrophages (a type of white cell) and several other types of cells. These cells that have the matching CSF-1 receptor accumulate and cause the bulk of the tumor, they are also thought to cause inflammatory changes in the affected area.
Diagnosis:
Diagnosis of TSGCT is often delayed as symptoms start off vague as pain and inflammation, and may mimic other joint injuries or conditions.
The Doctor will need to take a detailed patient history, and identify relevant symptoms. If TSGCT is suspected then specialized tests or imaging may be ordered including X-rays, MRI (magnetic resonance imaging), sampling of synovial fluid and a biopsy to check what type of cells make up the tumor.
Treatment Options:
Surgery is the standard treatment for TSGCT with the goal being removal of the tumor from the joint area, however complete removal is not always possible depending on the subtype, location and extent of the tumor. Surgery can be open surgery when the area is opened up with an incision so the surgeon has full access to the joint. Arthroscopic surgery which is done through a small incision may be an option in some cases of TSGCT.
Medications for TSGCT include Tyrosine Kinase inhibitors (TKI) which selectively blocks the CSF-1 receptor. This stops the accumulation of macrophages and other cells in the affected area.
Turalio (pexidartinib). Due to known side effects of Turalio it is only used in selected patients who can not have surgery, have significant disease morbidity and they must be enrolled in a Risk Evaluation and Mitigation Strategy program (REMS)
Drugs used to treat Tenosynovial Giant Cell Tumor
The medications listed below are related to or used in the treatment of this condition.
For ratings, users were asked how effective they found the medicine while considering positive/adverse effects and ease of use (1 = not effective, 10 = most effective).
Activity
Activity is based on recent site visitor activity relative to other medications in the list.
Rx
Prescription only.
OTC
Over-the-counter.
Rx/OTC
Prescription or Over-the-counter.
Off-label
This medication may not be approved by the FDA for the treatment of this condition.
EUA
An Emergency Use Authorization (EUA) allows the FDA to authorize unapproved medical products or unapproved uses of approved medical products to be used in a declared public health emergency when there are no adequate, approved, and available alternatives.
Expanded Access
Expanded Access is a potential pathway for a patient with a serious or immediately life-threatening disease or condition to gain access to an investigational medical product (drug, biologic, or medical device) for treatment outside of clinical trials when no comparable or satisfactory alternative therapy options are available.
Pregnancy Category
A
Adequate and well-controlled studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
B
Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
C
Animal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
D
There is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
X
Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risks involved in use in pregnant women clearly outweigh potential benefits.
N
FDA has not classified the drug.
Controlled Substances Act (CSA) Schedule
M
The drug has multiple schedules. The schedule may depend on the exact dosage form or strength of the medication.
U
CSA Schedule is unknown.
N
Is not subject to the Controlled Substances Act.
1
Has a high potential for abuse. Has no currently accepted medical use in treatment in the United States. There is a lack of accepted safety for use under medical supervision.
2
Has a high potential for abuse. Has a currently accepted medical use in treatment in the United States or a currently accepted medical use with severe restrictions. Abuse may lead to severe psychological or physical dependence.
3
Has a potential for abuse less than those in schedules 1 and 2. Has a currently accepted medical use in treatment in the United States. Abuse may lead to moderate or low physical dependence or high psychological dependence.
4
Has a low potential for abuse relative to those in schedule 3. It has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 3.
5
Has a low potential for abuse relative to those in schedule 4. Has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 4.
Alcohol
X
Interacts with Alcohol.
Further information
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