Global InformationGardner fibroma information
| Gardner fibroma | |
|---|---|
| Other names | Gardner-associated fibroma |
| Specialty | Pediatrics, Dermatology, Pathology, Surgical oncology |
| Symptoms | Typically a painless tumor in the skin |
| Usual onset | All ages but mostly in the first decade of life |
| Types | May be an isolated disorder or a manifestation of the genetic disease, adenomatous polyposis coli |
| Treatment | Surgical resection and follow-up testing for the presence or development of adenomatous polyposis coli |
| Frequency | Rare |
Gardner fibroma (GF) (also termed Gardner-associated fibroma[1]) is a benign fibroblastic tumor (i.e. a tumor containing fibroblasts, the most common cell type in connective tissue). GF tumors typically develop in the dermis (i.e. layer of skin underneath the epidermis) and adjacent subcutaneous tissue lying just below the dermis.[2] These tumors typically occur on the back, abdomen, and other superficial sites[2] but in rare cases have been diagnoses in internal sites such as the retroperitoneum[3] and around the large blood vessels in the upper thoracic cavity.[4] The World Health Organization, 2020, classified Gardner fibroma as a benign tumor in the category of fibroblastic and myofibroblastic tumors.[5]
In the majority of cases,[6] GF tumors are manifestations of the genetic disease, familial adenomatous polyposis (FAP), or its variant, the Gardner syndrome (GS).[7] (Hereafter, references to FAP will include its GS variant.) Furthermore, some cases of GF tumors, including those which are not associated with FAP, progress to or, after their surgical removal, recur as desmoid tumors (DT). A minority[1] of desmoid tumors, mainly those developing in the abdominal cavity,[3] are also associated with FAP.[3] Cases of Gardner fibroma and desmoid tumors that lack any other evidence of being associated with FAB at the time of diagnosis are often termed sporadic Gardner fibromas[1][8] and sporadic desmoid tumors.[9] However, some cases of sporadic GF and DT will, over the ensuing months or years, present with other signs of, and be diagnosed as, FAP. In these cases, the initial GF and DT tumors are considered the first sign of FAP and termed sentinel GF[2] and sentinel DT[9] tumors.
There are no large studies that clearly define the best treatment(s) for Gardner fibroma tumors. Common treatment strategies for these tumors include: surgical removal;[1] evaluations of the individuals bearing these tumors as well as their family members for evidence of FAP; genetic counseling; and long-term follow-up studies to detect evidence of FAP and recurrences of resected tumors.[1][2][10]
- ^ a b c d e Coffin CM, Hornick JL, Zhou H, Fletcher CD (March 2007). "Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas". The American Journal of Surgical Pathology. 31 (3): 410–6. doi:10.1097/01.pas.0000213348.65014.0a. PMID 17325483. S2CID 25831659.
- ^ a b c d 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.
- ^ a b c Goldstein JA, Cates JM (July 2015). "Differential diagnostic considerations of desmoid-type fibromatosis". Advances in Anatomic Pathology. 22 (4): 260–6. doi:10.1097/PAP.0000000000000077. PMID 26050263. S2CID 3388912.
- ^ Manappallil RG, Nambiar H, Mampilly N, Harigovind D (December 2019). "Superior vena cava syndrome due to mediastinal Gardner fibroma presenting as syncope". BMJ Case Reports. 12 (12): e232433. doi:10.1136/bcr-2019-232433. PMC 6904175. PMID 31806632.
- ^ 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.
- ^ Bakker A, Slack JC, Caragea M, Kurek KC, Bründler MA (2021). "Adipocyte-rich CTNNB1-mutated Intramuscular Gardner Fibroma Progressing to Desmoid Fibromatosis". Pediatric and Developmental Pathology. 24 (1): 62–67. doi:10.1177/1093526620968807. PMID 33104413.
- ^ Cates JM, Stricker TP, Sturgeon D, Coffin CM (October 2014). "Desmoid-type fibromatosis-associated Gardner fibromas: prevalence and impact on local recurrence". Cancer Letters. 353 (2): 176–81. doi:10.1016/j.canlet.2014.07.020. PMID 25064609.
- ^ Dahl NA, Sheil A, Knapke S, Geller JI (July 2016). "Gardner Fibroma: Clinical and Histopathologic Implications of Germline APC Mutation Association". Journal of Pediatric Hematology/Oncology. 38 (5): e154–7. doi:10.1097/MPH.0000000000000493. PMID 26840078. S2CID 40473308.
- ^ a b Coffin CM, Davis JL, Borinstein SC (January 2014). "Syndrome-associated soft tissue tumours". Histopathology. 64 (1): 68–87. doi:10.1111/his.12280. PMID 24236688. S2CID 5166124.
- ^ Chokoeva AA, Patterson JW, Tchernev G (December 2017). "Giant Subcutaneous Solitary Gardner Fibroma of the Head of a Bulgarian Child". The American Journal of Dermatopathology. 39 (12): 950–952. doi:10.1097/DAD.0000000000000787. PMID 27805922.