Plantar Fibromatosis – an overview | ScienceDirect Topics

Superficial Fibromatosis

The superficial fibromatoses109,121 include palmar fibromatosis, knuckle pads, and plantar fibromatosis. Palmar fibromatosis (Dupuytren’s contracture) almost always occurs in adults, increasing in incidence with age, with a marked preference for males. It is rare in blacks and Asians. Bilateral lesions occur in about 50% of cases, and a subset of cases is associated with knuckle pads and plantar fibromatosis. Patients present with a nodule in the palmar surface of the hand that progresses to flexion contracture. Treatment consists of fasciectomy or aponeurectomy, but the lesion may recur. Knuckle pads are thickenings on the dorsal aspect of the joints of the fingers; they do not lead to contractures. The epidemiologic features are similar to those of palmar fibromatosis, with which they are often associated. Treatment is generally not necessary. Plantar fibromatosis (Ledderhose’s disease) has epidemiologic features similar to those of palmar fibromatosis but occurs in a wider age range, including children and preadolescents.122 Lesions are bilateral in about 20% of cases and occur in approximately 10% of patients with palmar fibromatosis. Patients present with a nodule on the plantar aspect of the foot that causes discomfort on standing; contractures generally do not occur. Most cases do not require treatment. Fasciectomy is performed when discomfort is caused; however, recurrences are not uncommon.

The histologic appearances of all three lesions are similar. In the early proliferative nodular stage, a uniform population of plump spindle cells with vesicular nuclei is present in loose fascicles. The mitotic rate is often high, but atypical forms are generally not seen. In the later involutional stage, when specimens are most likely to be obtained, a population of thin spindle cells with slender nuclei is present in dense, collagenized fibrous tissue. Mitoses are usually not evident at this time. Biopsy specimens may also show features intermediate between these two appearances. Plantar fibromatosis more often shows features of early lesions, probably because biopsy is performed at an earlier stage of the disease. Plantar fibromatosis may also exhibit occasional multinucleated giant cells.123 Immunohistochemical studies show positivity for muscle-specific actin and the α-isoform of smooth muscle actin in early lesions; negativity for these markers is found in late lesions.

Early lesions may simulate fibrosarcoma, but attention to the clinical features should avoid misdiagnosis. Fibrosarcomas occurring in adults are rarely found in the hands or feet and generally involve deeper soft tissues.

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