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Palmar and Plantar Fibromatosis

Palmar and plantar fibromatoses are benign fibroblastic/myofibroblastic nodules that typically develop in the volar aspect of the hands and fingers or the plantar aponeuroses.

Symptoms & Causes

Introduction

Palmar and plantar fibromatoses are benign (non-cancerous) growths that develop in the fibrous tissues of the hands and feet. Palmar fibromatosis affects the palms and fingers, while plantar fibromatosis affects the soles of the feet. They are characterized by the formation of nodules and thickened tissue, which can sometimes lead to contractures (stiffness) and discomfort.

Reference
WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [cited 2024 09 11]. (WHO classification of tumours series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/chapters/33.

Related Terminology
Palmar fibromatosis: Acceptable: Dupuytren disease/contracture.
Plantar fibromatosis: Acceptable: Ledderhose disease.

Subtype(s)
None

Symptoms

Palmar fibromatosis typically has an insidious onset, with development of small, painless nodules that slowly evolve to form cords or band-like indurations between nodules in the subcutis and underlying fascia, leading to digit contractures and puckering of overlying skin. In contrast, plantar fibromatosis does not typically cause contractures and is often asymptomatic, although mild pain may develop after prolonged standing or walking.

Localization

Palmar fibromatosis primarily involves the volar or flexor aspect (palm side) of the hands and is bilateral (affects both hands) in as many as 50% of cases. Plantar fibromatosis is typically located in non-weight-bearing areas, such as the aponeurosis (thickened fibrous tissue) of the medial plantar arch, from the region of the navicular bone to the base of the first metatarsal. It is bilateral in as many as 35% of cases. Pediatric cases of plantar fibromatosis typically involve the anteromedial portion of the heel pad.

Epidemiology

Palmar fibromatosis predominantly affects adults, with a male predominance (male-to-female ratio: 3:1) and highest prevalence in white people. Its incidence increases with age, and it is rare among individuals aged < 30 years. Plantar fibromatosis is more common in younger patients (including those as young as 9 months), with almost half of all patients aged < 30 years. In pediatric cases, plantar fibromatosis shows a female predominance. Palmar or plantar fibromatosis can be associated with synchronous (occurring at the same time) or metachronous (occurring at different times) development of the other form (with 5–20% of patients developing both), but not desmoid fibromatosis. Associations with epilepsy and phenobarbital therapy, diabetes mellitus, cigarette smoking, and alcoholism with cirrhosis are reported but have not been widely validated.

Etiology

Unknown

Diagnosis & Treatment

Staging

Not clinically relevant

Pathogenesis

Although still driven at least in part by the WNT/β-catenin signaling pathway, this tumor lacks the CTNNB1 and APC mutations characteristic of desmoid fibromatosis.

Macroscopic appearance

Tumors are small, poorly defined 0.5–3.0 cm nodules, intimately associated with a tendon or aponeurosis and extending into subcutaneous tissue. The cut surface is firm, grayish-yellow or white (depending on the collagen content), and slightly whorled.

Histopathology

Typically, tumors involve a thickened palmar/plantar aponeurosis and form single to multiple discontinuous, moderately cellular spindle cell nodules in collagenous stroma. Morphologically, there are three phases of growth (proliferative, involutional, and late-stage). The proliferative phase comprises cellular, parallel fascicles of bland, plump, relatively uniform spindled fibroblasts with tapering nuclei, vesicular chromatin, and small or inconspicuous nucleoli, with minimal stromal collagen. Some plantar lesions are hypercellular and can mimic spindle cell sarcomas, but they lack atypia. There may be scattered, often perivascular chronic inflammation and occasional typical mitotic figures, especially in early lesions. Mitotic activity may be particularly prominent in pediatric patients. Occasional, particularly plantar lesions show interspersed multinucleated cells. Late-stage histology is associated with increased collagenous matrix and decreased cellularity. Rarely, there is osseous or cartilaginous metaplasia. By immunohistochemistry, tumor cells are variably positive for SMA and occasionally for desmin. A subset show nuclear β-catenin expression, despite the absence of CTNNB1 or APC gene mutations.

Cytology

Not clinically relevant

Prognosis and prediction

The risk of local recurrence is mostly dependent on the extent of surgical resection, although this is reserved for patients who have failed conservative management.

Clinical Features

Diagnostic molecular pathology

Not clinically relevant

Essential and desirable diagnostic criteria

Essential: bland, variably cellular proliferation of spindled fibroblasts/myofibroblasts; collagenous stroma; involvement of aponeurosis and variably subcutis and dermis.

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