Desmoid-type fibromatosis (DTF) is the medical term for a rare soft tissue tumor. It is characterized by the uncontrolled growth of fibrous tissue.
Understanding Desmoid-Type Fibromatosis
DTF, also known as desmoid tumor or aggressive fibromatosis, is not a cancerous tumor in the sense that it doesn't metastasize (spread to distant organs). However, it can be locally aggressive, meaning it can invade surrounding tissues and cause significant problems.
Key Characteristics:
- Rare: DTF affects approximately 5-6 people per million each year.
- Benign but Aggressive: While not metastatic, DTF can grow and infiltrate nearby structures.
- Fibrous Tissue Growth: The tumor consists of uncontrolled growth of fibrous tissue cells (fibroblasts).
- Location: DTF can occur in various parts of the body, including the abdomen, chest wall, and extremities.
- Age Predilection: While it can occur at any age, it's most common between 20 and 40 years old.
- Gender Predilection: More often seen in females.
Symptoms:
Symptoms vary depending on the size and location of the tumor, and may include:
- Painless lump or swelling
- Pain or discomfort
- Restricted movement
- Compression of nearby structures (nerves, blood vessels)
Diagnosis:
Diagnosis typically involves:
- Physical Examination: Assessing the lump and surrounding area.
- Imaging Studies: MRI (Magnetic Resonance Imaging) is the preferred imaging modality to visualize the tumor, its size, and its relationship to surrounding tissues. CT scans may also be used.
- Biopsy: A tissue sample is taken and examined under a microscope to confirm the diagnosis and rule out other conditions.
Treatment:
Treatment options depend on the size, location, and growth rate of the tumor, and may include:
- Active Surveillance: Monitoring the tumor for growth, as some DTFs may remain stable or even regress spontaneously.
- Surgery: Removal of the tumor. However, recurrence is possible, especially if complete removal is not achieved.
- Radiation Therapy: Using high-energy rays to kill tumor cells.
- Systemic Therapies: Medications such as hormonal therapy, chemotherapy, or targeted therapies may be used to slow tumor growth.
The management of DTF is complex and often requires a multidisciplinary approach involving surgeons, oncologists, and radiation oncologists.
