The Sinewy Assumption-Gardner-Type Fibroma

Anubha Bajaj*

Department of Histopathology, Panjab University/A.B. Diagnostics, India

*Corresponding Author: Anubha Bajaj, Department of Histopathology, Panjab University/A.B. Diagnostics, India.

Received: September 04, 2024; Published: September 20, 2024

Citation: Anubha Bajaj. “The Sinewy Assumption-Gardner-Type Fibroma". Acta Scientific Gastrointestinal Disorders 7.10 (2024):20-22.

Gardner-type fibroma emerges as an exceptionally encountered,  benign soft tissue lesion demonstrating morphological features akin to nuchal-type fibroma. Majority  (~90%) of lesions are concurrent with familial adenomatous polyposis  (FAP) or Gardner’s syndrome delineating specific germline mutations within the APC gene.

Tumefaction is comprised of haphazardly disseminated thickened bands of collagen commingled with a population of bland fibroblasts wherein the cellular component appears to entrap adjacent soft tissue.

Neoplasm commonly occurs within infants, paediatric population or adolescents. An estimated 45% subjects may demonstrate desmoid-type fibromatosis.

Gardner-type fibroma may be confined to superficial or deep seated soft tissue. No site of disease emergence is exempt [1,2].

Gardner-type fibroma may preliminarily indicate the occurrence of Gardner’s syndrome with concurrent genetic mutations within the APC gene. Chromosomal mutation confined to chromosome 5q21 is exemplified [2,3].

Although Gardner’s syndrome is comprised of colonic polyps, osteomas and anomalies within the retinal epithelium, cutaneous manifestations commonly emerge as epidermoid cysts layered by stratified squamous epithelium [2,3].

Grossly, neoplasm appears as an inadequately circumscribed, firm, rubbery, plaque-like, white to tan or pink lesion of magnitude varying from one centimetre to 10 centimetres. Cut surface appears grey/white and yellowish with fibrotic areas demonstrating entrapped mature adipose tissue [3,4].

Upon microscopy, tumefaction expounds haphazard dissemination of thickened collagen bundles commingled with hypo-cellular areas comprised of bland fibroblasts. Miniature vascular articulations appear admixed with the cellular component.

Neoplasm depicts a plaque-like pattern of tumour evolution along with infiltration of adjacent anatomic structures [4,5].

Gardner-type fibroma appears immune reactive to CD34, cyclin D1, vimentin and nuclear β catenin.

Tumour cells appear immune non reactive to muscle specific actin  (MSA), smooth muscle actin (SMA), desmin, oestrogen receptors (ER) and progesterone receptors (PR) [5,6].

Gardner-type fibroma requires segregation from neoplasms as familial adenomatous polyposis, Gardner’s syndrome, Turcot’s syndrome and attenuated variants of familial polyposis associated with chromosomal mutation of APC gene [6,7].

Neoplasm may be appropriately alleviated by surgical extermination, pre-eminently adopted for superior cosmetic outcomes [6,7].   

Bibliography

1. Rabai NA., et al. “A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case”. Cureus9 (2023): e46087.
2. Asaad SK., et al. “Extra abdominal recurrent aggressive fibromatosis: A case series and a literature review”. Molecular and Clinical Oncology 4 (2023): 84.
3. Alba-Pavón P., et al. “Analysis of germline variants in paediatric patients diagnosed with desmoid tumors and nuchal-type fibromas”. Translational Pediatrics9 (2023): 1715-1724.
4. Charifa A., et al. “Gardner Syndrome”. Stat Pearls International. Treasure Island, Florida (2024).
5. Blackwell MC., et al. “Extracolonic manifestations of Gardner syndrome: A case report”. Imaging Science in Dentistry 2 (2023): 169-174.
6. Thomaidis V., et al. “A case of early FAP diagnosis with extraintestinal manifestations on the face”. Acta Medica Academica 48 (2019): 217-224.
7. Huang K., et al. “Efficacy and safety of different thermal ablative therapies for desmoid-type fibromatosis: a systematic review and meta-analysis”. Quantitative Imaging in Medicine and Surgery 10 (2023): 6683-6697.
8. Image 1 Courtesy: Science direct.
9. Image 2 Courtesy: Basic medical key.

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Copyright: © 2024 Anubha Bajaj. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.