Sarcomas are a rare type of cancer. It mainly involves bones and soft tissue of the body. Soft tissue is a name given to connective tissue like muscles, tendons, nerves, blood vessels, fibrous tissue , and fat. Since they usually account for about 1% of cancer, they are often overlooked. However it is more common in children and adolescent in which it account for up to 20% of cancers. In this article , our consultant oncologist Dr Subhas Pandit discuss important points about management of bone and soft tissue sarcoma.
What are the symptoms ?
Sarcoma is not a single disease, but a cluster of diseases. The symptoms depend on location of tumor. They usually present as slow growing mass. They are usually painless in first but later become painful. The most frequent location are the arms and legs but can develop in any body part.
What are the main groups of sarcomas?
Bone sarcomas account for 0.2% of all new cancer cases in adults, making it a very rare form of cancer. However, not all bone cancers are sarcomas.
Soft-tissue sarcomas account for 1% of all new cancer cases in adults. These sarcomas can affect any part of the body, developing in supporting or connective tissue such as the muscle, nerves, fatty tissue, and blood vessels. Soft-tissue sarcomas include:
Alveolar Soft Part Sarcoma (ASPS)
Gastrointerstinal Stromal Tumor (GIST)
Malignant Fibro Histiocytoma (MFH)
Malignant Peripheral Nerve Sheath Tumor (MPNST)
Gastrointestinal stromal tumor (GIST). A GIST develops in the gastrointestinal tract, or digestive system, which includes the stomach and intestines.
Gynecological sarcomas. These sarcomas occur in the female reproductive system, which includes the uterus (womb), ovaries, vagina, vulva, and fallopian tubes. They can affect women of any age.
Retroperitoneal sarcomas. These sarcomas occur in the retroperitoneum. This is an area deep in the abdomen and pelvis, behind the abdominal lining (called the peritoneum), where organs such as the major blood vessels, kidneys, pancreas, and bladder are located.
How is sarcomas treated?
Sarcoma can involve different body part and depending on location and type of sarcoma, treatment plan is made. Therefore tumor board discussion in multi-disciplinary team (MDT) is done for best results.
The MDT will also discuss the best sequence of different treatments, such as surgery, radiation therapy, or chemotherapy, to create a a customized treatment plan for the individual. Typically, you’ll have surgery first to remove the tumor. Often, radiation and chemotherapy are the next step. These treatments are more likely to cure you if you got an early diagnosis. The MDT will continue to follow the progress of a patient and, together, make important and time-sensitive treatment decisions.
Now there are targeted therapy like Imatinib, pazopanib etc which has made sarcoma like GIST highly curable.
Cancer centers with sarcoma MDTs are also more likely to have better treatment results.
What progress have we made in treating sarcoma?
Although sarcoma is rare disease and progress has been slow, there are some significant development in last decade.
Our understanding of the molecular biology of this unique group of diseases allows us to better classify and group them. By understanding that sarcoma is not just 1 disease, but a large collection of distinct diseases, each with differing biologies, we’ve made significant treatment advances. They include new chemotherapies as well as targeted drugs for different sarcoma subtypes.
There has also been advances in method of radiotherapy delivery in sarcoma. With more focussed technique like IMRT, we have been able to improve local control and reduce side effects of radiotherapy.
New drugs like trabectidin has improved outcome in some catagory of sarcoma like liposarcoma and leiomyosarcoma.
Where can I get more information or support?
Our team of experts are ready to provide you best evidence-based management plan. We work together in a tumor board and make plans that give patients best chance of cure. You can contact us via whatsapp or call us at 01-5091629 / 9818226237 for consultation and more information.