How aggressive is pleomorphic sarcoma?
Sarcomas account for less than 1% of all cancers. Undifferentiated Pleomorphic Sarcoma, formerly called Malignant Fibrous Histiocytoma, is a rare subtype identified by a lack specific immunohistochemical markers for a specific lineage of differentiation. These soft tissue tumors are aggressive and rapidly enlarge.
What does undifferentiated pleomorphic sarcoma mean?
Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. Soft tissues connect, support and surround other body structures. UPS usually occurs in the arms or legs. Less often it can happen in the area behind the abdominal organs (retroperitoneum).
Is pleomorphic sarcoma malignant?
Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) of Soft Tissue Imaging.
What is the survival rate of pleomorphic sarcoma?
Pleomorphic rhabdomyosarcoma More than 30 out of every 100 people (more than 30%) will survive their cancer for 5 years or more after diagnosis.
How common is pleomorphic sarcoma?
UPS ranks the 4th most common soft tissue sarcoma with a slight male preponderance. The incidence has been evaluated to be close to 0.8-1 new case per 100000 per year in one European series. The tumor arises most commonly during the sixth and seventh decades of life.
What does pleomorphic mean?
Listen to pronunciation. (PLEE-oh-MOR-fik) Occurring in various distinct forms. In terms of cells, having variation in the size and shape of cells or their nuclei.
Is pleomorphic sarcoma hereditary?
A risk of soft tissue sarcoma can be inherited from your parents. Genetic syndromes that increase your risk include hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, neurofibromatosis, tuberous sclerosis and Werner syndrome.
Can you have a sarcoma for years?
Some types of soft tissue sarcoma that develop in the limbs or axial skeleton grow slowly over several years, or remain the same size for years or even decades, and then suddenly start to grow.
What is undifferentiated pleomorphic sarcoma (UPS)?
The undifferentiated pleomorphic sarcoma (UPS) —formerly known as malignant fibrous histiocytoma, is a high-grade aggressive soft-tissue sarcoma (STS). Mesenchymal stem cells are the most likely origin of the tumor, instead of histiocytes as previously thought.
How are the different types of sarcoma classified?
Many of these subtypes of sarcoma are classified, at least in part, according to the apparent line of differentiation of the tumor (the kind of normal cell that the tumor resembles). One particularly confusing diagnosis for patients is Malignant Fibrous Histiocytoma (MFH) or Undifferentiated Pleomorphic Sarcoma (UPS).
What is the rate of incidence for soft tissue sarcoma?
As with all sarcomas of soft tissue and bone, MFH is rare, with just a few thousand cases diagnosed each year. MFH of soft tissue typically presents in a patient that is approximately 50 to 70 years of age though it can appear at any age. MFH is very rare in persons less than 20 years old.
What is the who classification of multiple myeloma (MFH)?
Despite the frequency of diagnosis, MFH has remained an enigma. No true cell of origin has ever been identified. In 2002, the World Health Organization (WHO) declassified MFH as a formal diagnostic entity and renamed it as an undifferentiated pleomorphic sarcoma not otherwise specified, NOS (Ref. 4).