What is a desmoplastic tumor?

What is a desmoplastic tumor?

Desmoplastic small round cell tumors, or DSRCT, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body.

What is a desmoplastic?

Desmoplasia (also known as a desmoplastic reaction) is the term used by pathologists to refer to the growth of fibrous tissue around disease, usually cancer. However in dermatopathology, desmoplasia may also be seen with benign, as well as malignant, conditions.

What is the meaning of desmoplastic stroma?

Desmoplasia refers to growth of dense connective tissue or stroma. This growth is characterized by low cellularity with hyalinized or sclerotic stroma and disorganized blood vessel infiltration.

What is a desmoplastic stromal response?

Desmoplasia is the result of increased synthesis of extracellular matrix proteins and collagen by stromal cells. It is considered to be a reaction and response of the host tissue against invasive cancer cells.[5] The biological relevance of the desmoplastic response to cancer is still unclear.

Is DSRCT genetic?

The genetic change involved in DSRCT is acquired throughout a person’s lifetime and is not inherited .

How many people have DSRCT?

DSRCT is extremely rare. Fewer than 200 cases have been reported, about 85 percent of them in Caucasian people.

What is desmoplastic fibroma?

Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm, that is, recognized as the intra-osseous counterpart of soft tissue fibromatosis in both gnathic and extra-gnathic sites. It has a propensity for locally aggressive behavior and local recurrence.

What is desmoplastic melanoma?

Desmoplastic melanoma is a rare form of the disease that accounts for around 4 percent of all skin melanomas. The main sign of desmoplastic melanoma is a spot that may look like a scar in texture and appearance. It tends to develop on the face, scalp, neck, and sun-exposed areas of the arms and legs.

Can you survive DSRCT?

The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection.

Is DSRCT curable?

DSRCT is unlikely to be cured in the majority of patients. However, complete surgical resection by an experienced sarcoma surgeon has been shown to improve time to relapse and potentially provide cure in a subset of patients.

Is DSRCT hereditary?