Giant-cell fibroblastoma

Giant cell fibroblastoma
SpecialtyPediatrics, Dermatology, Pathology
SymptomsPainless mass in the dermis
Usual onsetPediatric population
CausesThought to be the formation of a COL1A1-PDGFB fusion gene
TreatmentSurgical excision
PrognosisGood
FrequencyRare

Giant cell fibroblastoma (GCF) is a rare type of soft-tissue tumor marked by painless nodules in the dermis (the inner layer of the two main layers of tissue that make up the skin) and subcutaneous (beneath the skin) tissue. These tumors may come back after surgery, but they do not spread to other parts of the body. They occur mostly in boys.[1] GCF tumor tissues consist of bland spindle-shaped or stellate-shaped cells interspersed among multinucleated giant cells.[2]

GCF tumors are closely related to dermatofibrosarcoma protuberans (DFSP) and dermatofibrosarcoma protuberans, fibrosarcomatous (DFSP-FS) (also termed fibrosarcomatous dermatofibrosarcoma protuberans) tumors.[3] The World Health Organization (2020) classified these three tumors as different tumors in the category of fibroblastic and myofibroblastic tumors with GCF and DFSP sub-classified as benign but aggressive tumors and DFSP-FS subclassified as a rarely metastasizing tumor.[4] However, The three tumor types may contain areas that have a microscopic histopathological appearance similar to one of the other types.[5] Furthermore, following their surgical resection GCF tumors may recur as DFSP tumors and vice versa and DFSP tumors may recur as DFSP-FS tumors. CGF, DFSP, and DFSP-FS have been regarded as an increasingly aggressive spectrum of related tumors.[6]

Giant cell fibroblastoma tumors are typically treated by surgical resection but have a very high rate of recurrence at the sites of their resection, particularly in cases where all of the tumor has not been removed. Accordingly, wide, complete tumor resections are the recommended treatment for them.[6]

  1. ^ Cerio, R. (2012). Dermatopathology. Springer Science & Business Media. ISBN 9783642595523. Retrieved 13 November 2017.
  2. ^ Dong SS, Wang N, Yang CP, Zhang GC, Liang WH, Zhao J, Qi Y (2020). "Giant Cell-Rich Solitary Fibrous Tumor in the Nasopharynx: Case Report and Literature Review". OncoTargets and Therapy. 13: 6819–6826. doi:10.2147/OTT.S252696. PMC 7367732. PMID 32764969.
  3. ^ 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.
  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. ^ Braswell DS, Ayoubi N, Motaparthi K, Walker A (April 2020). "Dermatofibrosarcoma protuberans with features of giant cell fibroblastoma in an adult". Journal of Cutaneous Pathology. 47 (4): 317–320. doi:10.1111/cup.13601. PMID 32163628. S2CID 212691248.
  6. ^ a b Jha P, Moosavi C, Fanburg-Smith JC (April 2007). "Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M. Enzinger". Annals of Diagnostic Pathology. 11 (2): 81–8. doi:10.1016/j.anndiagpath.2006.12.010. PMID 17349565.