About Sarcoma

According to the American Cancer Society A “Sarcoma” is a cancer of the connective or supportive tissue (bone, cartilage, fat, muscle, blood vessels) and soft tissue. Although sarcoma effects people of all ages, they are actually very rare, accounting for only 1% of all cases of cancer.

Malignant Fibrous Histiocytoma (muh-LIG-nunt FY-brus HIS-tee-oh-sy-TOH-muh) is a soft tissue sarcoma that usually occurs in the limbs, most commonly the legs, and may occur in the abdomen. MFH is also sometimes called malignant fibrous cytoma

Some basic facts on MFH:

• Patients with low-grade, intermediate-grade, and high-grade tumors have 10-year survival rates of 90%, 60%, and 20%, respectively.
• Patients with tumors smaller than 5 cm at presentation have survival rates of 79-82%.
• In the US: MFH accounts for 20-24% of soft tissue sarcomas, making it the most common soft tissue sarcoma occurring in late adult life.
• MFH most commonly occurs in white patients than in patients of African or Asian descent.
• Male-to-female ratio is approximately 2:1.
• MFH occurs most commonly in the extremities (70-75%, with lower extremities accounting for 59% of cases)
• MFH most commonly occurs in pateinets with the age between 50-70
• MFH is extremely rare, with just a few thousand cases diagnosed each year.

Metastatic cancer is cancer that has spread from the part of the body where it originated (called its primary site) to other parts of the body. When cells break away from a cancerous tumor, they can travel to other areas of the body through either the bloodstream or lymphatic channels. My pathology report suggests that metastasis from a bone “primary” can not be excluded. Some types of primary bone cancer are osteosarcoma, Ewing's sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).

MFH manifests a broad range of histologic appearances with four sub-types described:

1. Storiform-pleomorphic - is the most common type, accounting for up to 70% of most cases
2. Myxoid - the second most common type accounting for approximately 20% of cases In order for a tumor to be characterized as a myxoid variant, myxoid tissue must account for at least half of the tumor. The myxoid form tends to be less aggressive and as a result is associated with a better prognosis.
3. Giant cell – Least common, more aggressive (The kind that I had…;)
4. Inflammatory - tends to occur in the retroperitoneum

Summary

• MFH is a curable disease.
• The mainstay of treatment for MFH is complete surgical excision, most often supplemented with adjuvant radiation therapy.
• Chemotherapy is reserved for patients at the highest risk of disease recurrence or patients that already have recurrence.
• Patients with recurrent MFH can still be cured.
• Favorable prognostic factors that correspond to superior survival include small tumor size, low grade, extremity location, superficial location, and localized disease.