What is Low-Grade Fibromyxoid Sarcoma?
Low-Grade Fibromyxoid Sarcoma is a low grade, a rare tumor of soft tissues. Soft tissues are those tissues of the body which connect, protect, or lined other organs or structures of the body. Soft tissues of the body include muscles, tendons, ligaments, skin, fascia, nerves, blood vessels, fibrous sheaths, and synovial membranes of the joints. Low-grade tumors are those, which are nearly similar to the normal cells concerning their function and appearance and have a lower ability to grow and spread to other cells or parts of the body. Low-grade fibromyxoid sarcoma mostly affects soft tissues of the chest, abdomen, and extremities (shoulder, arms, hips, and legs) especially deep soft tissues of the thighs.
Disease Epidemiology
In most of the reported cases, low-grade fibromyxiod sarcoma was found in the younger and middle-aged population, the mean age is 34 and it ranges from 3-78 years. It is slightly more common in males as compared to females. Recent clinical research studies revealed that its recurrence rate is 65%, metastatic (spread of the tumor to the adjacent cells and the distant parts of the body) rate is 45% and the death rate is 42%. Metastasis of the tumor mostly involves lungs, pleura (outer protective layer of the lungs), and chest wall.
Disease Etiology (cause)
There is no exact well-known cause is found. Certain genetic mutations are considered responsible for the development of the Tumor.
Signs and symptoms
The patient may face the following signs and symptoms.
- The patient of low-grade fibromyxiod sarcoma remains symptoms free for a prolonged period after the development of the tumor.
- The tumor grows slowly over a prolonged period.
- Tumor lump /mass is painless in most of the cases. Tumor size varies from 1-8cm.
Diagnosis
Definite diagnostic tests to diagnose the low-grade fibromyxoid sarcoma are tumor biopsy and histopathology, Fine needle aspiration cytology, and cytogenetic study of the specimen. Radiological techniques like CT scan, MRI, USG, and X-ray also recommended locating the size and site of the tumor.
Treatment
For the tumor which is not widespread, wide local surgical excision of the tumor is the most effective treatment option. Chemotherapy and radiotherapy are the treatment options for tumors that spread to the distant parts of the body and not confined to its site of origin. Concerned about the potential of very late recurrence or metastasis (even decades after initial diagnosis) long term clinical follow up is advisable.
What Support can we Give for Low-Grade Fibromyxoid Sarcoma?
Low-Grade Fibromyxoid Sarcoma is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Low-Grade Fibromyxoid Sarcoma we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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