Fibromatosis infantile desmoid - Altmeyers Encyclopedia

Synonym(s)

infantile desmoid fibromatosis; Infantile desmoid-like fibromatosis

Definition
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A type of tumor belonging to the infantile fibromatoses, in which the tumor masses are located in the deep dermis and/or in the fascia or adjacent mucosa.

Localization
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Shoulder girdle, thighs, head and neck.

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Clinic
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Thick, not tolerated, little movable, firm knots, which are usually discovered by chance.

Diagnosis
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Histology.

Differential diagnosis
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Fibrosarcoma (clinical and histological).

Therapy
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A sufficiently performed excision in healthy subjects is recommended. Risk of recurrence > 50%.

Literature
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Kaçar A et al (2012) Childhood fibroblastic and myofibroblastic tumors: a multicenter documentation and review of the literature.Turk Patoloji Derg 28:24-30.
Pilz T et al (1999) Chemotherapy in fibromatoses of childhood and adolescence: results from the Cooperative soft tissue sarcoma study (CWS) and the Italian Cooperative study group (ICG-AIEOP)]. Clinic Padiatr 211:291-295.