Infantile digital fibromatosis

Infantile digital fibromatosis
Other namesInclusion body fibromatosis, Infantile digital myofibroblastoma, Reye's tumor
SpecialtyPediatrics, Dermatology
Usual onsetTypically at birth or infancy
CausesUnknown
PrognosisExcellent
FrequencyRare

Infantile digital fibromatosis (IDF), also termed inclusion body fibromatosis or Reye's tumor, usually occurs as a single, small, asymptomatic, nodule in the dermis on a finger or toe[1] of infants and young children.[2] IMF is a rare disorder with approximately 200 cases reported in the medical literature as of 2021.[3] The World Health Organization in 2020 classified these nodules as a specific benign tumor type in the category of fibroblastic and myofibroblastic tumors.[4] IDF was first described by the Australian pathologist Douglas Reye in 1965.[5]

IDF consists of an overgrowth of spindle-shaped cells in a collagen fiber-rich background located in the dermis (i.e. the layer of skin between the epidermis and subcutaneous tissue) but may extend into the subcutaneous tissue. These spindle-shaped cells contain distinctive inclusion bodies within their cytoplasm that greatly help in distinguishing IDF from other fibrous skin diseases. These inclusions are composed of densely packed vimentin and actin filaments.[6]

IDF lesions are usually painless and have a tendency to regress spontaneously. Consequently, the current recommended treatment for IDF nodules is conservative observation.[3] If the lesions cause local deformities and/or functional impairments or continue to increase in size over long observation periods, surgical removal is recommended. However, surgically removed IDF lesions have had high rates of recurrence and this surgery may cause post-operative digital deformities.[6]

  1. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 989. McGraw-Hill. ISBN 0-07-138076-0.
  2. ^ Laskin WB, Miettinen M, Fetsch JF (January 2009). "Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up". The American Journal of Surgical Pathology. 33 (1): 1–13. doi:10.1097/PAS.0b013e3181788533. PMID 18830128. S2CID 30315278.
  3. ^ a b Agnihotri MA, Sathe PA (2021). "Inclusion body fibromatosis - A report of four cases and review of literature". Journal of Postgraduate Medicine. 67 (1): 24–26. doi:10.4103/jpgm.JPGM_774_20. PMC 8098864. PMID 33565473.
  4. ^ Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC 8167394. PMID 33179614.
  5. ^ REYE RD (September 1965). "Recurring Digital Fibrous Tumors of Childhood". Archives of Pathology. 80: 228–31. PMID 14322942.
  6. ^ a b Adegoke OO, Ajao AE, Ano-Edward GH (December 2020). "Congenital infantile digital fibromatosis: a case report and review of the literature". African Health Sciences. 20 (4): 1865–1869. doi:10.4314/ahs.v20i4.42. PMC 8351811. PMID 34394250.