Gardner fibroma | |
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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]