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Oral Surgery, Oral Medicine, Oral... Feb 2022The aim of the present study was to carry out a systematic review of available data regarding case reports and case series of oral and maxillofacial benign fibrous... (Review)
Review
OBJECTIVE
The aim of the present study was to carry out a systematic review of available data regarding case reports and case series of oral and maxillofacial benign fibrous histiocytoma (BFH).
STUDY DESIGN
A search strategy was performed using the PubMed, Web of Science, Scopus, and EMBASE electronic databases.
RESULTS
Male individuals (56.89%) were more affected, with a mean age of 34.55 ± 20 years. The buccal mucosa (20.33%) represented the most common site, with the clinical presentation of a painless (86.95%) nodule (98.03%). The clinical hypothesis of a fibroma was reported in most cases (31.57%). Radiographic presentation of intraosseous lesions showed multilocular radiolucent images (55.55%). Regarding the histopathologic features, the biphasic population of fibroblastic and histiocytic cells was seen in 21 cases (39.62%), and a spindle-shaped fibroblastic cell population organized into a storiform pattern was observed in 25 cases (47.16%). CD68 (n = 26) and vimentin (n = 25) showed immunoreactivity in all BFH cases in which they were used. All cases were treated with a surgical resection, and 8.10% recurred.
CONCLUSIONS
The current systematic review demonstrated that BFH represents a rare lesion that mainly affects the buccal mucosa of male individuals, and the treatment is mainly surgical with a good prognosis.
Topics: Adolescent; Adult; Head; Histiocytoma, Benign Fibrous; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Young Adult
PubMed: 34511357
DOI: 10.1016/j.oooo.2021.07.003 -
World Neurosurgery Jun 2022Craniofacial chondromyxoid fibromas (CMFs) are a rare benign tumor of cartilaginous origin. They are commonly misdiagnosed due to the paucity of information on tumor... (Review)
Review
BACKGROUND
Craniofacial chondromyxoid fibromas (CMFs) are a rare benign tumor of cartilaginous origin. They are commonly misdiagnosed due to the paucity of information on tumor characteristics. We performed a systematic review to characterize CMFs located in different regions of the craniofacial skeleton.
METHODS
A search of the literature was executed using the search phrase "chondromyxoid fibroma" and included articles from 1990-2020. Sixty-eight articles met the inclusion criteria, with a total of 91 patients with analyzable data (22 with calvarial and 69 with sinonasal tumor locations). Descriptive analyses were performed to compare pre-selected characteristics between the 2 groups.
RESULTS
Sinonasal CMF frequently presented with cranial nerve palsy and expectedly had a high rate of nasal symptoms. Calvarial tumors frequently presented with an external mass and headache. Gross total resection (GTR) was achieved in a higher proportion of cases in the calvarial group versus the sinonasal group (83.3% vs. 53.1%). Overall recurrence rate at 17.7% was higher in sinonasal CMF compared with the calvarial tumors at 8.3%. Recurrences after GTR were similar in the sinonasal and calvarial groups (9.7% vs. 9.1%). In patients who did not achieve GTR, recurrence was higher in the sinonasal compared with the calvarial group (27.6% vs. 0%).
CONCLUSIONS
Craniofacial CMF in calvarial and sinonasal locations have distinct clinical characteristics and response to treatment. Sinonasal lesions tend to have higher recurrence compared to calvarial CMF. Performance of GTR is associated with decreased recurrence in all CMFs.
Topics: Fibroma; Humans
PubMed: 34710582
DOI: 10.1016/j.wneu.2021.10.139 -
Pediatric Blood & Cancer Sep 2022Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm, which occurs in children and adults, with a peak incidence in young adults. For the... (Review)
Review
Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm, which occurs in children and adults, with a peak incidence in young adults. For the majority of the patients, DTF is a chronic and symptomatic disease, which affects health-related quality of life. Systemic treatment regimens tend to differ for patients treated by pediatric oncologists compared to medical oncologists. This systematic review identified 14 clinical trials in children and adults with DTF. Tumor response and progression-free survival rates varied widely between studies and study populations. Treatment choices for patients with DTF are based on a paucity of (randomized) trials. Treatment principles of DTF are similar in pediatric and adult oncology, but the treatment itself is different. This seems mostly driven by a lack of tyrosine kinase inhibitor (TKI) accessibility in pediatric oncology. An insufficient number of studies examined patient-reported outcomes, which are extremely important for patients with a chronic disease like DTF.
Topics: Child; Fibromatosis, Aggressive; Humans; Progression-Free Survival; Quality of Life; Soft Tissue Neoplasms; Young Adult
PubMed: 35714333
DOI: 10.1002/pbc.29831 -
BMC Musculoskeletal Disorders Aug 2016Dupuytren's disease is a common fibrotic disorder of the palm characterized by the development of progressive flexion deformities in the digits, leading to significant... (Review)
Review
BACKGROUND
Dupuytren's disease is a common fibrotic disorder of the palm characterized by the development of progressive flexion deformities in the digits, leading to significant functional impairment. Surgical excision remains the most common treatment. However, this is only indicated in patients with established contractures rather than those with early disease. Early disease is generally characterized by the presence of palmar nodules with limited or no contracture of the fingers. The ideal treatment would be directed at patients with early progressive disease to prevent future deterioration. Various non-surgical treatment modalities have been described but there is currently no systematic assessment of the role and efficacy of these treatments in patients with early disease.
METHODS
Using a PICOS analysis we reviewed publications of studies of patients with early disease who had received physical therapies, pharmacological treatment, or radiotherapy. Following PRISMA guidelines titles and abstract were screened using predefined criteria to identify those reporting outcomes specifically relating to the treatment of early disease. In the absence of a definition of early disease studies were included if early DD was described clinically, with digital contractures not exceeding 30°, Tubiana grades N to 1, and which reported identifiable data. Studies were excluded if data for early DD patients could not be extracted for analysis.
RESULTS
In this systematic review, 26 studies were identified and analyzed to evaluate the effect of pharmacological therapy (n = 11), physical therapy (n = 5) and radiotherapy (n = 10) on early Dupuytren's disease. The studies comprised 20 case series, 1 cohort study with the remainder reporting case studies. All publications were graded level of evidence 4 or 5 assessed using the Oxford Centre for Evidence Based Medicine grading. Narrative descriptions of the data are presented.
CONCLUSIONS
Physical therapies were the most robustly assessed, using objective measures but the studies were under powered, providing insufficient evidence of efficacy. Intralesional steroid injection and radiotherapy appeared to lead to softening of nodules and to retard disease progression but lacked rigorous evaluation and studies were poorly designed. There is an urgent need for adequately powered double blinded randomized trials for this common disorder which affects 4 % of the population.
TRIAL REGISTRATION
The protocol was registered ( CRD42015008986 16 November 2015) with the PROSPERO international prospective register of systematic reviews.
Topics: Disease Progression; Drug Therapy; Dupuytren Contracture; Hand; Humans; Physical Therapy Modalities; Radiotherapy; Treatment Outcome
PubMed: 27526686
DOI: 10.1186/s12891-016-1200-y -
Plastic and Reconstructive Surgery Mar 2018The role of diabetes mellitus, liver disease, and epilepsy as risk factors for Dupuytren disease remains unclear. In this systematic review and meta-analysis, the... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The role of diabetes mellitus, liver disease, and epilepsy as risk factors for Dupuytren disease remains unclear. In this systematic review and meta-analysis, the strength and consistency of these associations were examined.
METHODS
The MEDLINE, EMBASE, and Web of Science databases were searched for articles reporting an association between Dupuytren disease and diabetes mellitus, liver disease, and epilepsy published before September 26, 2016. The frequencies of Dupuytren disease and diabetes mellitus, liver disease, and epilepsy were extracted, as was information on potential confounders. Generalized linear mixed models were applied to estimate pooled odds ratios, adjusted for confounders. Heterogeneity between studies was quantified using an intraclass correlation coefficient and was accounted for by a random effect for study.
RESULTS
One thousand two hundred sixty unique studies were identified, of which 32 were used in the meta-analyses. An association between Dupuytren disease and diabetes mellitus was observed (OR, 3.06; 95 percent CI, 2.69 to 3.48, adjusted for age), which was stronger for type 1 diabetes mellitus than for type 2 diabetes mellitus but was not statistically significant (p = 0.24). An association between Dupuytren disease and liver disease was observed (OR, 2.92; 95 percent CI, 2.08 to 4.12, adjusted for sex). Dupuytren disease and epilepsy were associated, yielding an OR of 2.80 (95 percent CI, 2.49 to 3.15). Heterogeneity between studies was moderate to low.
CONCLUSIONS
These findings demonstrate an association between Dupuytren disease and diabetes mellitus, liver disease, and epilepsy. Prospective, longitudinal studies are needed to elucidate the pathways causing these associations.
Topics: Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Dupuytren Contracture; Epilepsy; Humans; Liver Diseases; Odds Ratio; Risk Factors
PubMed: 29481401
DOI: 10.1097/PRS.0000000000004120 -
Irish Journal of Medical Science Apr 2023Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically but more commonly are associated with genetic syndromes such as familial adenomatous polyposis (Sakorafas et al. in Surg Oncol 16(2):131-142, 2007) (FAP). Mutations in either the Wnt, β-catenin or APC genes are 'key' triggers for the development of these tumours (Howard and Pollock in Oncol Ther 4(1):57-72, 2016). Classically, these tumours do not metastasise; however, they are associated with significant morbidity and mortality due to their infiltrative pattern and/or local invasion. Historically, surgical resection was the cornerstone of treatment. There remains paucity of data regarding outcomes following the surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
OBJECTIVES
The aim of this review was to assess the current evidence for surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
METHODS
A systematic search of articles in PubMed, EMBASE and The Cochrane Library databases was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for the period from January 2000 to November 2020.
RESULTS
Twenty-three studies were included, of which, 749 patients had surgical resection (696 for primary and 53 for recurrent desmoids), 243 patients (18.8%) were medically managed and 353 patients (27.3%) underwent surveillance. Median follow-up was 51.4 months (range 1-372). Six-hundred and ninety-six of the 749 resections (92.9%) underwent primary desmoid resection, with the remaining 53 (7.1%) undergoing resection for recurrence. One-hundred and two surgically managed patients (19%) developed a (re)recurrence, with mesenteric involvement the commonest site for recurrence (55%). When comparing recurrence post-surgery to progression following medical therapy, there was a trend towards better outcomes with surgery, with 25% of surgical patients having a recurrence versus 50.5% having progression with medical therapy [OR 0.40 (95% CI 0.06-2.70), p = 0.35]. Major morbidity following surgery was 4.4% (n = 33) with 2% (n = 14) mortality within 30 days of resection.
CONCLUSION
The management of desmoids has considerable heterogeneity. Surgical resection for abdominal desmoids remains a valid treatment option in highly selective cases where negative margins can be obtained, with low major morbidity and/or mortality.
Topics: Humans; Fibromatosis, Aggressive; Fibromatosis, Abdominal; Adenomatous Polyposis Coli; Mutation; Colectomy
PubMed: 35445926
DOI: 10.1007/s11845-022-03008-8 -
Cardiovascular and Interventional... May 2022Desmoid tumours (DT) are rare locally infiltrative soft-tissue tumours which do not metastasise. DT arise sporadically or are associated with familial syndromes, with... (Review)
Review
Desmoid tumours (DT) are rare locally infiltrative soft-tissue tumours which do not metastasise. DT arise sporadically or are associated with familial syndromes, with different clinical and genetic patterns. In recent years there has been an increasing therapeutic role of cryoablation for the treatment of sporadic DT. Therefore, in this present review, we: (a) summarize all the main epidemiological, clinical, and therapeutic aspects of sporadic DT that are relevant to an interventional radiologists' practice; (b) present the results of a systematic review that has been conducted with the intent of highlighting the main clinical outcomes available thus far with cryoablation; and (c) discuss the current and future potential applications of cryoablation in this field. Five studies were included in the systematic review accounting for 146 patients. Only 18.5% patients received cryoablation as a first-line treatment. Overall, the volume of the DT undergoing cryoablation was very large (mean total DT volume of 237 cm). Major complications were noted for 13.3-30% patients and following 2.4-6.7% interventional sessions. The rates of complete tumour response ranged between 0 and 43.3%. 1- and 3-year local progression-free survival rates were 85.1-85.8%, and 77.3-82.9%, respectively. Complete pain relief was reported in 40-66.7% symptomatic patients. In conclusion, cryoablation is currently proposed as a therapeutic strategy to very large DT, which is recognized to be associated with an increased procedure-related morbidity and reduced rates of complete tumour response. Proposing cryoablation as the first-line treatment may improve these clinical outcomes.
Topics: Catheter Ablation; Cryosurgery; Fibromatosis, Aggressive; Humans; Progression-Free Survival; Retrospective Studies; Soft Tissue Neoplasms; Treatment Outcome
PubMed: 35237861
DOI: 10.1007/s00270-022-03091-5 -
Annals of Plastic Surgery Oct 2015Desmoid tumors (DT) represent a group of rare, distinct lesions. There are few published studies examining outcomes and safety of complex reconstruction after DT... (Review)
Review
BACKGROUND
Desmoid tumors (DT) represent a group of rare, distinct lesions. There are few published studies examining outcomes and safety of complex reconstruction after DT resection.
METHODS
A retrospective review identified 39 patients who underwent surgical treatment of DT at St. Jude Children's Research Hospital over a 12-year period. A systematic review of the literature identified 17 further studies for inclusion. Treatment characteristics were analyzed.
RESULT
Thirty-nine patients were treated during the study period, with a total number of 67 resections. Median age was 12.2 years; 49% of patients were male, and 51% were female. Median tumor size was 9.8 cm. DT most commonly arose in the extremities (40%), thorax (23%), head and neck (21%), and trunk (16%). One- and 5-year recurrence-free survival were 97.1% and 73.1%, respectively. The majority of defects were closed primarily, with the exception of head and neck defects. Long-term outcomes were good for chest, abdomen, and upper extremity defects, but were problematic for head and neck, breast, and lower extremity defects. There were no recurrences at the site of flap harvest in either the study population or in reviewed studies.
CONCLUSION
For patients with DT, surgical extirpation should not come at the expense of functional preservation, as overall survival is excellent. However, specific defects, including those of the lower extremity, breast, and head and neck, will benefit from improved techniques for resection and reconstruction.
Topics: Adolescent; Child; Child, Preschool; Female; Fibromatosis, Aggressive; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Plastic Surgery Procedures; Retrospective Studies; Survival Analysis; Treatment Outcome; Young Adult
PubMed: 26360656
DOI: 10.1097/SAP.0000000000000129 -
The Journal of Hand Surgery, European... Sep 2017Radiotherapy has been advocated as an alternative treatment in early Dupuytren's disease. We have systematically reviewed the evidence on the use of radiotherapy in... (Review)
Review
UNLABELLED
Radiotherapy has been advocated as an alternative treatment in early Dupuytren's disease. We have systematically reviewed the evidence on the use of radiotherapy in Dupuytren's disease. Only six articles met a minimum set standard, five of which were retrospective cohort studies and one a randomized controlled study. A total of 770 Dupuytren's hands, nearly all with Tubiana stage 0-1 disease, were irradiated with an average 30 Gy. Disease regression ranged from 0%-56%, stability from 14%-98% and progression from 2%-86%. Salvage surgery was successful in all cases of disease progression post-radiotherapy. There were no reports of adverse wound healing problems associated with such surgery or radiotherapy-associated malignancy. On balance, radiotherapy should be considered an unproven treatment for early Dupuytren's disease due to a scarce evidence base and unknown long-term adverse effects. Well-designed randomized controlled studies are required to confirm the benefits of radiotherapy treatment.
LEVEL OF EVIDENCE
II.
Topics: Disease Progression; Dupuytren Contracture; Humans; Radiotherapy
PubMed: 28490266
DOI: 10.1177/1753193417695996 -
PloS One 2021Desmoid tumor is a locally-invasive neoplasm that causes significant morbidity. There is recent interest in cryotherapy for treatment of extra-abdominal desmoid tumors.... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Desmoid tumor is a locally-invasive neoplasm that causes significant morbidity. There is recent interest in cryotherapy for treatment of extra-abdominal desmoid tumors. This systematic review assesses evidence on safety and efficacy of cryotherapy in the treatment of extra-abdominal desmoid tumors.
MATERIALS AND METHODS
The systematic review was conducted with reference to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Literature search was performed using MEDLINE and the Cochrane Central Register of Controlled Trials. 9 full text papers were reviewed and meta-analysis was performed for measures of safety, efficacy and symptom relief.
RESULTS
The estimated pooled proportion of major and minor complications was 4.2% (95% CI, 1.8-9.6; I 2 = 0%) and 10.2% (95% CI, 5.7-17.8; I 2 = 0%) respectively. The estimated pooled proportion of non-progressive disease rate of all studies was 85.8% (95% CI, 73.4-93.0; I 2 = 32.9%). The estimated progression free survival rate at 1 year was 84.5% (95% CI:74.6-95.8) and 78.0% at 3 years (95% CI: 63.8-95.3). As for pain control, the estimated pooled proportion of patients with decrease in visual analogue scale (VAS) > = 3 for those with VAS > = 3 before treatment for 2 studies was 87.5% (95% CI, 0.06-100; I 2 = 71.5%) while 37.5% to 96.9% of patients were reported to have experienced partial or complete symptom relief in the other studies.
CONCLUSION
Cryotherapy is a safe and effective treatment modality for extra-abdominal desmoid tumors with efficacy similar to those treated with traditional strategies in the short to medium term.
Topics: Cryotherapy; Fibromatosis, Aggressive; Humans; Progression-Free Survival; Treatment Outcome
PubMed: 34941915
DOI: 10.1371/journal.pone.0261657