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Fibromatosis: Causes, Symptoms and Treatment

Fibromatosis refers to a group of benign but locally aggressive connective tissue disorders characterized by uncontrolled proliferation of fibroblasts. It can affect various regions of the body.

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Things worth knowing about "Fibromatosis"

Fibromatosis refers to a group of benign but locally aggressive connective tissue disorders characterized by uncontrolled proliferation of fibroblasts. It can affect various regions of the body.

What is Fibromatosis?

Fibromatosis is an umbrella term for a heterogeneous group of conditions characterized by excessive proliferation of fibroblasts (connective tissue-forming cells). Although the resulting tissue changes are histologically benign (non-cancerous), they can grow in a locally aggressive manner and infiltrate adjacent structures such as muscles, nerves, and blood vessels. Fibromatoses frequently recur after surgical removal but generally do not form distant metastases.

Types of Fibromatosis

There are numerous forms of fibromatosis, classified according to location and clinical behavior:

Superficial (Fascial) Fibromatoses

Dupuytren contracture: Thickened cords in the palm of the hand leading to flexion contractures of the fingers.
Ledderhose disease (plantar fibromatosis): Nodule formation on the sole of the foot.
Peyronie disease: Fibrous plaques in the penile shaft that can cause curvature.
Knuckle pads: Fibrous nodules over the knuckles.

Deep (Musculoaponeurotic) Fibromatoses

Desmoid tumor (aggressive fibromatosis): Often occurs intra-abdominally or on the abdominal wall; may be associated with familial adenomatous polyposis (FAP).
Infantile and juvenile fibromatosis: Occurs in children and can affect various soft tissue regions.

Causes and Risk Factors

The exact causes of fibromatosis are not fully understood. Contributing factors include:

Genetic predisposition: Mutations in the APC gene or the beta-catenin gene (CTNNB1) are known in desmoid tumors.
Trauma and injury: Mechanical injury or prior surgery can predispose to fibromatosis development.
Hormonal influences: Estrogen appears to play a role, particularly in desmoid tumors; incidence is higher in women of childbearing age.
Family history: Familial adenomatous polyposis (FAP) significantly increases the risk of intra-abdominal desmoid tumors.
Inflammatory processes: Chronic inflammation of connective tissue may be involved.

Symptoms

Symptoms vary considerably depending on the location and extent of the fibromatosis:

Palpable, firm nodules or cords under the skin
Pain or a sense of pressure in the affected area
Restricted mobility (e.g., finger contractures in Dupuytren disease)
Compression of adjacent structures (nerves, blood vessels, organs) in deep fibromatoses
Abdominal pain or bowel problems in intra-abdominal desmoid tumors

Diagnosis

Diagnosis is established through a combination of clinical examination and imaging:

Clinical examination: Palpation, assessment of mobility and functional impairment.
Ultrasound (sonography): First-line imaging for superficial lesions.
Magnetic resonance imaging (MRI): Gold standard for detailed assessment of extent and relationship to adjacent structures, especially in deep fibromatoses.
Biopsy and histology: Tissue sampling for microscopic examination to confirm the diagnosis and exclude malignant tumors.
Genetic testing: Recommended when FAP-associated desmoid tumors are suspected.

Treatment

Treatment depends on the type, location, extent, and clinical course of the fibromatosis:

Active Surveillance

For asymptomatic or slowly growing fibromatoses, a watch-and-wait approach may be adopted initially, as some lesions remain stable or even regress spontaneously.

Surgical Treatment

Surgical removal is often the first choice for symptomatic or growing fibromatoses. However, recurrence rates after surgery can be substantial, so complete resection is the goal whenever possible.

Medical Therapy

Nonsteroidal anti-inflammatory drugs (NSAIDs): e.g., sulindac, may inhibit desmoid tumor growth.
Anti-hormonal therapy: Tamoxifen or other anti-estrogens are used for hormone-dependent fibromatoses.
Tyrosine kinase inhibitors: e.g., imatinib or sorafenib, have shown efficacy in aggressive or recurrent desmoid tumors.
Chemotherapy: Considered in particularly aggressive or inoperable cases.

Radiation Therapy

Radiation therapy may be used as an adjunct after incomplete resection or for inoperable fibromatoses.

Physiotherapy and Occupational Therapy

To improve mobility and function, particularly in superficial fibromatoses such as Dupuytren contracture.

Prognosis

The prognosis for most superficial fibromatoses is favorable. Deep fibromatoses, especially desmoid tumors, can be challenging due to their local aggressiveness and high recurrence rates. As metastasis does not occur, life expectancy is generally not reduced; however, quality of life may be affected by functional impairment and repeated treatments.

References

Gronchi A. et al. - Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 2021.
Kasper D. et al. - Harrison's Principles of Internal Medicine, 20th edition, McGraw-Hill, 2018.
World Health Organization (WHO) Classification of Tumours Editorial Board - Soft Tissue and Bone Tumours, 5th edition, IARC Press, Lyon, 2020.

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