Musculoskeletal fibromatoses are grouped in those involving the superficial or the deep tissue,
and they share similar clinical and pathological findings. The superficial fibromatoses include palmar and plantar fibromatosis.
Patients usually present with painless,
subcutaneous nodules that show slow progression into bands that cause traction on the underlying tendons,
resulting in dupuytren’s contracture.
Plantar fibromatoses (Ledderhose disease) are less common and can occur concomitantly,
affecting the middle aspect of the sole of the foot in a similar fashion than its palmar counterpart.
Palmar fibromatosis
Clinical features
Palmar fibromatosis is the most common superficial musculoskeletal fibromatosis and affects between 1 and 2% of the population,
almost eclusively caucasians.
The male-to-female ratio is around 3-4 to 1 and lesions are bilateral in 40-60% of patients.
Although there are genetic factors involved in this disorder,
it is thought that there are several etiologic factors taking place,
such as immunological,
micro-traumatic and vascular factors,
diabetes mellitus,
epilepsy,
alcoholism and keloids.
Patients present with painless subcutaneous nodules,
composed of fibrous bands or cords attach to one another,
adjacent to the flexor tendons.
With progression,
the nodules exert traction on the flexor tendons and the consequence is the development of digital contractures - Dupuytren's contracture.
The forth and fifth rays are prone to development of fibromatosis,
and aside from being often bilateral,
patients frequently present with other types of fibromatosis,
namely plantar fibromatosis and knuckle pads.
the only effective treatment for palmarfibromatosis is surgery and it is indicated if the disease causes glexion contracture greater than 20º at the metacarpo-phalangeal joints or more than 30º at the proximal inter-phalangeal joints.
Either radical or segmental fasciectomy are performed to relieve the contracture and there is an important rate of recurrence (circa 30-50%).
Imaging features
- Radiography - normal; flexion contractures
- Ultrasound - hypervascular hypoechoic nodules in the palmar subcutaneous tissues,
superficial to the flexor tendons
- Computed tomography - unspecific hypodense areas of nodular tickening (appearance similar to muscle)
- Magnetic resonance - superficial soft-tissue masses with a cord-like appearance that extend from the palmar aponevrosis and extend parallel to the flexor tendons; behaviour is typically similar to tendons (low-signal on T1 and T2-weighted images) or,
in some cases, of intermediate signal intensity on T1WI and T2WI (depends on the collagen and cellular content proportion).
Gadolinium injection shows diffuse enhancement of variable degree.
Magnetic resonance plays an important role in distinguishing lesions with higher cellular content (and,
also,
less collagen density),
because they have a higher signal intensity compared to muscle.
This observation should be reported,
as lesions with high cellular content are more prone to post-surgical recurrence.
Plantar fibromatosis
Clinical features
Plantar fibromatosis is frequently described as Ledderhose disease and is less frequent than its palmar counterpart.
Its occurence increases with age (> 30-50 years).
Men are affected twice as commonly as women and bilateral involvement is relatively frequent (20-50%).
As previously mentioned,
concomitant presence of palmar fibromatosis is seen in around 10-65% of patients and knuckle pads can also be found in such patients.
There is also a multifactorial etiology for the disease,
which includes genetic and micro-traumatic causes,
diabetes mellitus,
keloids,
alcoholism and epilepsy.
A soft-tissue mass composed of one or several fibromas can be palpated.
These consist of subcutaneous nodules on the medial side of the plantar aspect of the foot.
One third of patients have multiple nodules.
Despite being symptom-free in most cases,
there may be complaints of pain after prolonged standing or walking.
There is no evidence of contracture.
Treatment is often conservative - foot patds or orthotics to relieve symptoms.
Steroid injections can be employed and surgical resection should only be considered in rare cases of fibrous infiltration of adjacent tissues.
There is a high risk of recurrence and development of post-operative neuromas.
Imaging features
- Radiography - normal
- Ultrasound - similar appearance to palmar fibromatosis,
with hypoechoic or mixed echogenic nodules located in the subcutaneous tissue,
superficial to the plantar aponevrosis.
The aponevrosis may be thickened.
Lesions may have poor definition.
- Computed tomography - subcutaneous soft-tissue nodules with attenuation comparable to muscle
- Magnetic resonance - fusiform soft-tissue mass extending along the deep plantar aponevrosis; it may be difficult to determine the boundaries between the lesion and the adjacent musculature.
Low-signal intensity on T1WI and T2WI is characteristic; heterogenous enhancement after gadolinium administration is typical.
Intermediate signal on T1WI and T2WI is not as common as in palmar fibromatosis.