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Fibromatosis is a condition where fibrous overgrowths of dermal and subcutaneous connective tissue develop tumours called fibromas. The term refers to a group of soft tissue tumours with certain characteristics in common, including the absence of malignant features, a histology of well-differentiated fibroblasts, and an infiltrative growth pattern. Fibromatosis colli, for instance, is a benign tumour that develops in infants within 8 weeks of delivery and generally responds well to physiotherapy. Fibromatosis can also manifest in the form of plantar fibromas, which are rare, benign growths that develop on the arch of the foot and are more common in males. While most fibromatoses are benign tumours, some forms like desmoid tumours can be locally aggressive.
| Characteristics | Values |
|---|---|
| Definition | A condition where fibrous overgrowths of dermal and subcutaneous connective tissue develop tumours called fibromas. |
| Classification | Based on age (i.e. juvenile vs adult fibromatoses) and localisation (i.e. superficial vs deep fibromatoses). |
| Types | Intra-abdominal fibromatosis, palmar fibromatosis, plantar fibromatosis, infantile myofibromatosis, head and neck desmoid fibromatosis, dermatofibrosis lenticularis, fibromatosis hyalinica multiplex, and more. |
| Tumour Characteristics | Absence of cytologic and clinical malignant features, histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressiveness. |
| Diagnosis | Special stains are of limited assistance. Vimentin reactivity is seen. Actin (smooth muscle and muscle-specific) staining may be present focally. |
| Treatment | Treatment depends on the individual disease. Surgery is often required, and curative excision has been reported. Physiotherapy may also be effective. |
| Demographics | Males are twice as likely to develop plantar fibromatosis. People between the ages of 30 and 50 are more likely to develop plantar fibromas. |
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What You'll Learn
Fibromatosis colli
Fibromatosis is a term used to refer to a group of soft tissue tumours that share certain characteristics, including the absence of malignant features, a histology consistent with well-differentiated fibroblasts, and an infiltrative growth pattern. One specific type of fibromatosis is fibromatosis colli (FMC), a rare benign tumour that occurs in the lower part of the sternocleidomastoid (SCM) muscle in infants. It is also known as a sternocleidomastoid tumour of infancy, pseudotumour of infancy, or infancy sternocleidomastoid pseudotumour. The term "tumour" in this context refers to a swelling rather than a cancerous growth.
FMC is an uncommon tumour, with an incidence rate of 0.4% to 1.3% of live births. It typically develops within the first eight weeks after delivery, with an average onset of 24 days. The tumour usually appears on the right side of the neck and is firm, measuring a couple of centimetres in diameter. It is more common in male infants and can cause the infant's head to tilt towards the side of the mass, with the chin tilting to the opposite side due to sternocleidomastoid muscle contracture.
FMC is typically diagnosed through clinical examination, ultrasound, and microscopic cytology analyses. Ultrasound is particularly useful in accurately diagnosing FMC and excluding more suspicious swellings in the neck and face. Fine-needle aspiration cytology (FNAC) is a minor surgical procedure that can be used to examine the cells and differentiate FMC from other conditions.
Most cases of FMC resolve without treatment. Well over 90% of these tumours disappear within six months by following a daily regimen of stretching exercises and repositioning procedures. Physiotherapy is often recommended, and in some cases, surgery may be considered if the tumour persists beyond a year or causes significant range-of-motion problems. The surgical techniques involve cutting, manipulating, lengthening, and then stitching the muscles to correct them.
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Intra-abdominal fibromatosis
Fibromatosis refers to a group of soft tissue tumours that share certain characteristics, including the absence of cytologic and clinical malignant features, a histology indicative of well-differentiated fibroblasts, and an infiltrative growth pattern. These lesions arise from fibroblasts and/or myofibroblasts and are often diagnosed in the pectoral or cervical muscles of young adult horses. In skeletal muscle, fibrous tissue infiltration can become markedly invasive in advanced stages. While fibromatosis lacks metastatic potential, it can be locally aggressive, particularly in the head and neck regions, where it is known as head and neck desmoid fibromatosis.
Of particular interest to orthopaedic surgeons is palmar fibromatosis (Dupuytren's contracture), which is a relatively common tumour seen in young to middle-aged adults. This tumour arises in the connective tissues of the muscles and aponeurosis, particularly in the shoulder, pelvic girdle, and thigh. Another form of fibromatosis, fibromatosis colli, develops in infants within eight weeks of delivery and typically responds well to physiotherapy.
In summary, intra-abdominal fibromatosis is a rare benign tumour that can arise from abdominal fibrous tissue and exhibit aggressive local infiltration. It is essential to distinguish IAF from GIST to select the appropriate treatment approach, which may include surgery or chemotherapy in some cases. Fibromatosis more broadly encompasses a range of soft tissue tumours characterised by fibroblastic tissue and infiltrative growth patterns, with specific types affecting skeletal muscle, orthopaedic structures, and infant sternocleidomastoid muscles.
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Infantile myofibromatosis
Fibromatosis refers to a group of soft tissue tumours that share certain characteristics, including the absence of malignant features. Infantile myofibromatosis (IMF) is a rare disorder characterised by the growth of non-cancerous tumours. These tumours affect the skin, bone, muscle, soft tissue, and, rarely, the internal organs. The specific symptoms and severity of IMF can vary greatly from person to person, and the condition can be classified into different types.
The solitary form of IMF is the most common, accounting for about 75% of cases. It is characterised by a single nodule or lesion that usually occurs in the skin, subcutaneous tissue, or muscle tissue in the head, neck, or trunk areas. The multicentric form of IMF is less common and is characterised by multiple nodules in the skin, subcutaneous tissue, or muscle. The multicentric form with visceral involvement is the most severe type of IMF, as it includes growths in internal organs such as the lungs and heart.
The tumours or lesions that characterise IMF are typically firm, flesh-coloured, or purple bumps on the skin or just underneath it. These lesions may be freely movable if they are superficial, while deeper lesions are generally immobile. Skin lesions may be crusted or hardened and can cause discolouration and ulceration of the overlying skin. While the tumours are benign, they can grow large enough to damage or compress nearby structures, leading to pain and potential bone destruction, disfigurement, and physical limitations.
IMF is typically found at birth in about one out of 150,000 babies, and most cases occur in children under two years old. It is often discovered during the baby's examination at birth, but additional tests, such as ultrasound, MRI, or CT scans, may be performed. While IMF tumours do not spread (metastasise), they can cause symptoms by their size and compression of surrounding structures. Treatment options for IMF are varied and depend on the specific presentation of the tumour(s). Solitary IMF tumours often regress spontaneously within 18 to 24 months of diagnosis, so a watchful waiting strategy is commonly employed. More severe cases, such as those with visceral involvement, may require surgical excision or treatment with drugs and/or radiation therapy.
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Head and neck desmoid fibromatosis
Fibromatosis refers to a group of soft tissue tumours that are related to the sarcoma family. They are characterised by the absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggression.
Desmoid tumours, or desmoid-type fibromatosis, are rare, locally invasive benign tumours with high recurrence rates, causing significant morbidity. They are locally aggressive (myo)fibroblastic lesions that arise from musculoaponeurotic structures throughout the body. The head and neck region is more frequently affected in children and adolescents than in adults. The neck is one of the most common sites of occurrence, with 80% of cases occurring in the anterio-lateral region. The incidence rate is estimated at 2.4 to 4.5 cases per million people annually, with 7-25% of cases involving the head and neck region.
Complete surgical excision of desmoid tumours is considered the only effective treatment. However, due to the infiltrative nature of the tumour in the head and neck region and the difficulty of achieving complete surgical resection, a multi-modality management strategy is often employed to control the disease. Surgery is typically followed by radiation therapy to manage residual or recurrent disease.
In children, desmoid-type fibromatosis of the head and neck can manifest as soft tissue lesions in the sinonasal and paramandibular regions. CTNNB1 gene sequencing has revealed mutations in some cases, indicating the involvement of specific genetic factors.
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Plantar fibromatosis
The diagnosis of plantar fibromatosis is primarily clinical, involving a physical examination of the foot. An MRI or ultrasound can also be used as a confirmatory adjunct. The most common symptom of plantar fibromatosis is pain on the bottom of the foot, usually in the arch. This pain is often first noticed when wearing shoes that put pressure on the plantar fibroma. Depending on its size, a plantar fibroma can cause pressure and discomfort on the foot.
The treatment for plantar fibromatosis has traditionally been symptomatic management, with conservative treatment strategies including steroid injections, verapamil, radiation therapy, extracorporeal shock wave therapy, tamoxifen, and collagenase. When conservative measures fail, surgical removal of the fibromas and adjacent plantar fascia may be performed, although recurrence is common.
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Frequently asked questions
Fibromatosis is a condition where fibrous overgrowths of dermal and subcutaneous connective tissue develop tumours called fibromas. These are noncancerous tumours made up of fibrous tissue.
There are various types of fibromatosis, including:
- Palmar fibromatosis (Dupuytren's contracture)
- Plantar fibromatosis
- Infantile myofibromatosis
- Head and neck desmoid fibromatosis
- Intra-abdominal fibromatosis
The symptoms of fibromatosis vary depending on the type and location of the tumour. For example, plantar fibromatosis appears as a small, benign growth on the arch of the foot, while infantile myofibromatosis can occur as solitary tumours in the head and neck regions or multiple tumours in the skin, muscles, or bones.
The treatment for fibromatosis depends on the specific type and severity of the condition. In some cases, surgical excision may be required, while in others, physiotherapy or medication may be sufficient.
