Fibromatosis digital infantile M72.8

Authors: Prof. Dr. med. Peter Altmeyer, Alexandros Zarotis

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Last updated on: 29.10.2020

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Synonym(s)

desmoid fibromatosis; Digital Fibroma recurrent; digital fibrous swellings in children; Fibroma infantile digital; Inclusion body fibromatosis; Infantile digital fibroma; juvenile aponeurotic fibroma; Keloid fibromatosis; recurrent digital fibroma; recurrent digital fibromatosis of childhood; Recurrent digital fibromatosis of childhood; recurring digital fibrous tumor of childhood

History
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Franck, 1908; Reye, 1965

Definition
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Rare, benign, myofibroblastically differentiated tumour on fingers or toes in infants and small children, with a tendency to infiltrative growth. Very rare in adults.

Manifestation
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Congenital (approx. 30% of cases), occurring shortly after birth or in the first months of life, or more rarely acquired later.

Localization
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Dorsal or lateral side of fingers or toes (except thumbs and big toes)

Clinical features
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Mostly single, more rarely multiple, coarse, flat to spherical, 1-2 cm large, reddish, cutaneous to subcutaneously located nodes.

Histology
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Fuzzily defined, moderately cell-rich tumor parenchyma arranged in vertebrae and fascicles, whose spindle cells are partially oriented perpendicular to the epidermis. The tumor cells have a poorly delimited, moderately eosinophilic cytoplasm with eosinophilic inclusion bodies. They react positively with desmin and alpha-smooth muscular actin.

Therapy
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Leading total excision in sano without safety distance. Recurrences occur in up to 60% of cases. Spontaneous healing is possible.

Literature
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  1. Hügel et al (1995) Fibromatoses and other fibroblastically/myofibroblastically differentiated connective tissue tumors from the dermatologist's point of view. Z Hautkr 70: 717-724
  2. Kanwar AJ et al (2002) Congenital infantile digital fibromatosis. Pediatric Dermatol 19: 370-371
  3. Kawaguchi M et al (1998) A case of infantile digital fibromatosis with spontaneous regression. J Dermatol 25: 523-526
  4. Reye RDK (1965) Recurring digital fibrous tumor of childhood. Arch pathogen 80: 228-231
  5. Sarma DP, Hoffmann EO (1980) Infantile digital fibroma-like tumour in an adult. Arch Dermatol 116: 578-579

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Last updated on: 29.10.2020