Global InformationFibrous hamartoma of infancy information
| Fibrous hamartoma of infancy | |
|---|---|
| Other names | Subdermal fibromatous tumor of infancy |
| Specialty | Dermatology, General surgery, Pathology |
| Symptoms | Benign usually painless tumor in subcutaneous tissues |
| Usual onset | First 2 years of life; congenital in ~20% of cases |
| Causes | Unknown |
| Treatment | Surgical resection |
| Prognosis | Excellent |
| Frequency | rare |
Fibrous hamartoma of infancy (FHI) is a rare, typically painless, benign tumor that develops in the subcutaneous tissues of the axilla (i.e. armpit), arms, external genitalia, or, less commonly, various other areas. It is diagnosed in children who are usually less than 2 years old or, in up to 20% of cases, develops in utero and is diagnosed in an infant at birth.[1]
The cells involved in FHI include bland fibroblasts/myofibroblasts, mature fat cells, and primitive-appearing spindle-shaped and/or star-shaped cells.[2] The tumor was first described by R.D. Reye in 1956 who termed the disorder "subdermal fibromatous tumor of infancy" and regarded it as a reactive lesion.[3] In a 1964 study of 30 patients, F.M. Enzinger renamed the tumor hamartoma of infancy; he regarded it to be a hamartoma (i.e. a local malformation of various normal cell types due to a systemic genetic condition) rather than a reactive lesion.[4] However, subsequent studies have found gene mutations in FHI tumor cells and conclude that it is a true neoplasm, i.e. a growth of cells which is uncoordinated with that of the normal surrounding tissue and persists in growing abnormally even if the original trigger is removed.[5] In 2020, the World Health Organization classified FHI in the category of benign fibroblastic and myofibroblastic tumors.[6]
Fibrous hamartoma of infancy is generally a benign tumor but may be locally aggressive,[7] locally infiltration,[8] and in uncommon cases produce symptoms such as tenderness.[9] Surgical excision is the treatment of choice for FHI tumors.[7]
- ^ Baranov E, Hornick JL (March 2020). "Soft Tissue Special Issue: Fibroblastic and Myofibroblastic Neoplasms of the Head and Neck". Head and Neck Pathology. 14 (1): 43–58. doi:10.1007/s12105-019-01104-3. PMC 7021862. PMID 31950474.
- ^ Al-Ibraheemi A, Martinez A, Weiss SW, Kozakewich HP, Perez-Atayde AR, Tran H, Parham DM, Sukov WR, Fritchie KJ, Folpe AL (April 2017). "Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features". Modern Pathology. 30 (4): 474–485. doi:10.1038/modpathol.2016.215. PMID 28059097. S2CID 3477224.
- ^ REYE RD (July 1956). "A consideration of certain subdermal fibromatous tumours of infancy". The Journal of Pathology and Bacteriology. 72 (1): 149–54. doi:10.1002/path.1700720120. PMID 13367990.
- ^ ENZINGER FM (February 1965). "Fibrous hamartoma of infancy". Cancer. 18 (2): 241–8. doi:10.1002/1097-0142(196502)18:2<241::aid-cncr2820180216>3.0.co;2-c. PMID 14254080. S2CID 42199990.
- ^ John I, Fritchie KJ (January 2020). "What is new in pericytomatous, myoid, and myofibroblastic tumors?". Virchows Archiv. 476 (1): 57–64. doi:10.1007/s00428-019-02700-y. PMID 31705190. S2CID 207941071.
- ^ 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.
- ^ a b Sheybani EF, Eutsler EP, Navarro OM (December 2016). "Fat-containing soft-tissue masses in children". Pediatric Radiology. 46 (13): 1760–1773. doi:10.1007/s00247-016-3690-z. PMID 27866258. S2CID 19576285.
- ^ Martos-Cabrera L, Sampedro-Ruiz R, Pérez-González YC, Mentzel T, Llamas-Velasco M (June 2021). "Fibrous Hamartoma of Infancy: A Series of 21 Cases and Review of the Literature". Actas Dermo-Sifiliográficas (English Edition). 112 (6): 520–527. doi:10.1016/j.adengl.2021.03.010. PMID 34088477.
- ^ Dickey GE, Sotelo-Avila C (1999). "Fibrous hamartoma of infancy: current review". Pediatric and Developmental Pathology. 2 (3): 236–43. doi:10.1007/s100249900119. PMID 10191347. S2CID 3022223.