Nodular fasciitis: imaging staging and management
The radiological semiotics of nodular fasciitis directly depends on the histological stage of development of the pathological process.
- Early (myxoid) stage: the lesion contains a large amount of myxoid tissue. On magnetic resonance imaging, the signal intensity of such a lesion is very close to fluid. A characteristic feature is mild hyperintensity on T1-weighted images (compared to the signal from surrounding muscle tissue).
- Late (fibrous) stage: over time, the myxoid tissue is replaced by coarse fibers. The lesion undergoes fibrosis, causing the signal to become hypointense on all scanner pulse sequences, including T1.
A confirmatory biopsy is required for a definitive diagnosis. Therapeutic management includes intralesional steroid injections and surgical intervention. Notably, recurrence after excision is extremely rare.
Elastofibroma: a fibroelastic pseudotumor
Elastofibroma (Elastofibroma) is a tumor-like lesion that occurs almost exclusively in elderly patients. It is a fairly common finding, detected in 2% of individuals over 60 years of age undergoing chest CT according to statistics. More than half of the cases are completely asymptomatic and are incidental findings at autopsy.
Etiology and localization: The process is thought to be triggered by mechanical friction between the scapula and the chest wall (often associated with heavy physical labor), leading to local collagen degeneration. Familial forms of the disease have also been described. In the overwhelming majority of cases (>99%), elastofibroma is localized on the chest wall, adjacent to the inferior angle of the scapula, situated deep to the serratus anterior muscle (serratus anterior). The lesions are frequently bilateral.
Imaging features:
- Ultrasound: the appearance is nonspecific, but the mass can be visualized quite clearly if the patient is asked to perform arm adduction.
- CT and MRI: an elliptical mass with typical fibrofatty content is visualized. Characteristically, there is an alternation of fat stripes (hyperintense signal on T1) and fibrous tissue (hypointense signal) running parallel to the chest wall.
Important nuance: bilateral symmetrical elastofibromas can confuse the diagnostician, as on superficial examination they resemble normal muscle tissue. With a typical location and characteristic MRI findings, a biopsy is not required, and marginal resection is sufficient for treatment.
Superficial fibromatoses
Fibromatoses in general are locally aggressive lesions of intermediate malignancy. Superficial forms are classified depending on the location of the process.
Palmar fibromatosis (Dupuytren's disease): Manifests as the development of fibrous nodules and dense cords in the subcutaneous tissue along the ulnar aspect of the palm. This leads to thickening of the skin, palmar aponeurosis, and flexor tendons (the ring finger and little finger are most frequently affected). Clinically, an incapacitating flexion contracture develops. On ultrasound, the nodules have low echogenicity. MRI allows assessment of cellularity: lesions with a high cellular content (relatively hyperintense to the tendon on T1 and T2) are more likely to recur after surgical treatment.
Plantar fibromatosis (Ledderhose disease): Histologically identical to the palmar variant, but affects the plantar fascia (more commonly the medial border). Often asymptomatic, but large nodules can cause pain during walking and standing. On MRI (especially coronal PD-weighted images), it appears as a lobulated hypointense mass with low to intermediate signal intensity. Treatment is conservative; surgery is indicated only for severe pain.
Other superficial forms include knuckle pads (fibrous masses on the dorsum of the metacarpophalangeal and proximal interphalangeal joints) and penile fibromatosis (Peyronie's disease). Different superficial forms may coexist in the same patient.
Desmoid-type fibromatosis
Also known as deep, aggressive, or extra-abdominal fibromatosis. This is a clonal proliferation of fibroblasts in deep soft tissues. The tumor is locally invasive, destroying adjacent structures, but never metastasizes. Most commonly seen in young adults in the shoulder, chest wall, back, thigh, or neck.
Clinically, the mass is usually painless, but when superficial, it is palpated as an extremely firm lesion. Due to this high firmness (caused by collagenization), the lesion can be very difficult to biopsy with a needle. Symptoms appear only when the mass begins to compress nerves or invade joints.
MRI findings can vary significantly, however, a specific sign exists: the presence of characteristic very low-signal bands on T2-weighted images. These zones correspond to dense collagen bundles, allowing for a confident diagnosis.
Briefly: answers to topical questions
What are the ultrasound findings in plantar fibromatosis (Ledderhose disease)?
The ultrasound appearance of plantar fibromatosis (Ledderhose disease) is not described in detail in the literature. The disease is often detected as an asymptomatic incidental finding on ultrasound.
MRI is the primary imaging modality: the mass, originating from the plantar fascia, shows low to intermediate signal intensity and often appears as a lobulated hypointense mass.
What genetic syndromes are associated with the development of desmoid fibromatosis?
The development of desmoid fibromatosis is associated with familial adenomatous polyposis and its variant, Gardner syndrome.
- Familial adenomatous polyposis (FAP) is a genetic disorder; when combined with it, fibromatosis has a more invasive course and a high rate of local recurrence.
- Gardner syndrome (Gardner syndrome / Gardner’s syndrome) is an autosomal dominant disorder whose clinical presentation includes intestinal adenomatous polyps, multiple osteomas, and desmoid fibromatosis.
Intra-abdominal desmoid tumors frequently arise specifically in association with this syndrome.
What treatment methods are used for various forms of superficial fibromatosis?
Both conservative and surgical treatments are used for various forms of superficial fibromatosis, depending on the location and severity of the process.
- Palmar fibromatosis (Dupuytren’s disease) — surgical treatment is used; the need for surgery is determined by the severity of the deformity and functional deficit. Recurrences are frequent.
- Plantar fibromatosis (Ledderhose disease) — treatment is usually conservative. Excision is used for large, disabling lesions.
In palmar fibromatosis, the process may stabilize after a few years, and regression is sometimes possible.
Superficial fibromatoses often present as a group. Remember: Dupuytren — hands (ulnar side), Ledderhose — feet (medial side), Peyronie — specific location, and knuckle pads — on the knuckles.
Class and exam questions
Why is biopsy often omitted in elastofibroma?
If the lesion is located in a typical site (inferior angle of the scapula) and has a characteristic layered MRI structure (alternating fat and fibrosis), the diagnosis is considered reliable and does not require histological confirmation.
Which MRI features indicate a high risk of recurrence in Dupuytren's disease?
Relative hyperintensity of the nodules on T1- and T2-weighted images relative to the adjacent tendon indicates high cellularity of the lesion, which is statistically associated with frequent recurrences after excision.
Is desmoid fibromatosis capable of metastasizing?
No. Although it is a lesion of intermediate malignancy and aggressively infiltrates local tissues (nerves, joints), it does not metastasize.
What else to study on this topic
- Histological pattern of the myxoid and fibrous stages of nodular fasciitis.
- Differential diagnosis of elastofibroma and soft tissue sarcomas.
- Surgical approaches and resection techniques in palmar fibromatosis.
- The role of ultrasound in the differential diagnosis of deep desmoid-type fibromatoses.
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