Facial paediatric desmoid fibromatosis: A case series, literature review and management algorithm

Wilks, Daniel J.; Mowatt, David; Merchant, W.; Liddington, Mark

doi:10.1016/j.bjps.2011.09.027

Cited by 6 publications

(1 citation statement)

References 33 publications

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“…The pathogenesis of desmoid fibromatosis is multifactorial and includes genetic predisposition, endocrine factors, and physical factors such as trauma [ 6 ]. These tumors are characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize [ 6 , 18 ]. Clinically, the extra-abdominal fibromatosis appears as a deep-seated and circumscribed mass growing insidiously, often with no pain [ 6 ] and a variable clinical course [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review

Moro

Angelis

Gasparini

et al. 2018

Case Reports in Oncological Medicine

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.Purpose. Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, and musculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoid fibromatosis. In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit is presented. e aim of the article is to describe this unusual and rare location for the desmoid fibromatosis and outline the principle phases in the decision-making process and the therapeutic alternatives for a patient affected by desmoid fibromatosis. Methods. e protocol of this review included study objectives, search strategy, and selection criteria. e primary end point of this study was to analyze the head and neck desmoid fibromatosis. e secondary end point was to identify the available therapies and assess their specific indications. Results. e mean age of patients was 18.9 years ranging from 0 to 66, and 52% were female. A bimodal age distribution was observed, and two age peaks were identified: 0-14 years (57%) and 28-42 years (18%). e most common involved areas were the mandible (25%) followed by the neck (21%). In 86% of the cases, the treatment was the surgical resection of the disease, and only in 5% of the cases, the surgical resection was followed by adjuvant radiotherapy. Conclusion. e orbital location is extremely rare, especially in the pediatric population. e management of desmoid fibromatosis is based on the function preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of the disease or risk of functional damages, the surgical approach may be considered. erapeutic alternatives to surgical resection are radiotherapy and systemic therapy.

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Section: Introductionmentioning

confidence: 99%

Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review

Moro

Angelis

Gasparini

et al. 2018

Case Reports in Oncological Medicine

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Massive congenital cervicofacial desmoid-type fibromatosis in a 5-month-old infant

Albokashy

Halawani

Eshky

et al. 2021

Journal of Surgical Case Reports

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Desmoid-type fibromatosis (DF) is a borderline tumor of soft tissues that has low malignant potential but described as infiltrative, locally aggressive and rapidly growing. In the pediatric population, it occurs in the head and neck. Presentation varies based on tumor size and location. Despite the high recurrence rate, surgical excision remains the modality of choice with. Here, we report a case of a 5-month-old boy, with extensive head and neck DF that was managed twice with conservative debulking surgery through a combined transoral-transcervical approach. On 2-year follow-up, he was gaining weight with no developmental delay and had no clinical evidence tumor regrowth.

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