Congenital infantile digital fibromatosis: a case report and review of the literature

Abstract

Infantile digital fibromatosis (IDF) is a rare benign fibroproliferative tumor of early childhood. IDF preferentially affects the fingers and the toes. Malignant transformation or metastases have never been reported. Surgical treatment has been advocated previously but local recurrences were observed frequently. Recent literature supports clinical surveillance without any medical or surgical intervention as spontaneous regression usually occurs after two to three years. A six-month-old Caucasian girl with IDF on the left fourth digit is presented here. The tumor progressively increased in size after birth. Topical imiquimod cream and diflucortolone valerate cream, both displaying antifibrotic properties, had no effect on tumor growth. Currently the lesion size remains stable without any treatment. Early recognition of IDF is important in order to avoid unnecessary surgical intervention that may prove to be potentially aggravating, unless serious functional or cosmetic concerns intervene. Parents should be reassured concerning the benign nature of IDF and be informed that spontaneous involution of IDF might be expected.

Infantile Digital Fibromatosis/Inclusion Body Fibromatosis: A Comprehensive Literature Review

“…Topical therapies have also been tried. For example, topical imiquimod has been attempted with mixed results [31, 32]. The case with a successful outcome used imiquimod 5% cream 3 days per week in combination with methylprednisolone acetonate 0.1% ointment on the other days, achieving partial improvement by 8 weeks and near complete resolution by 22 weeks [31].…”

“…For example, topical imiquimod has been attempted with mixed results [31, 32]. The case with a successful outcome used imiquimod 5% cream 3 days per week in combination with methylprednisolone acetonate 0.1% ointment on the other days, achieving partial improvement by 8 weeks and near complete resolution by 22 weeks [31]. In another report, one in which impaired mobility spurred treatment, application of topical tacrolimus 1% ointment twice daily for 1 month resulted in improvement of the lesion by 2 months and complete resolution with restoration of mobility by 6 months [33].…”

Fibromatosis digital infantil congénita ulcerada, reporte de caso

“…Para confirmar el diagnóstico de fibromatosis digital infantil, se recomienda practicar una biopsia de piel, la cual muestra un entrelazado de fascículos de células fusiformes, haces de colágeno que forman un nódulo dérmico y característicos cuerpos de inclusióncitoplásmicos eosinofílicos de localización perinuclear (6) . Algunos autores consideran que los cuerpos citoplásmicos de inclusión son patognomónicos de esta enfermedad (7) , pese a que se han encontrado en otras entidades histopatológicas, como en pólipos endocervicales atípicos y en tumores filodes de la glándula mamaria (8) . Sin embargo, la presencia de esta inclusión es muy característica y permite el diagnóstico diferencial histológico con otras fibromatosis (5) .…”

“…La fibromatosis digital infantil, si bien se cataloga como una entidad de naturaleza benigna, se considera recidivante en el 60 % de los casos, aún después de la extirpación quirúrgica (6) , aunque la escisión amplia con un injerto de piel o colgajo loca,l reduce esta probabilidad (7) . Se recomienda la cirugía si hay deterioro funcional (4) , pero, además, se deben considerar las consecuencias estéticas (8) , ya que solo el 12 % de los casos se resuelven espontáneamente (6,9) o después de la toma de la biopsia (10) . Los esteroides tópicos no han mostrado producir ningún beneficio, aunque su aplicación intralesional puede resultar útil (6) .…”