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Journal of Investigative and Clinical... Feb 2018The aim of the present review was to systematically present the clinicopathological data of desmoplastic ameloblastoma (DA) from articles published in the literature. A... (Review)
Review
The aim of the present review was to systematically present the clinicopathological data of desmoplastic ameloblastoma (DA) from articles published in the literature. A comprehensive search of the databases (PubMed, Medline, SCOPUS, Web of Science, and Google Scholar) for published articles on DA was conducted. A total of 238 cases were identified and analyzed from 76 published papers. DA showed a slight male predilection (male: female=1.07:1) with a predominance in the fourth and fifth decades of life. Mandibular involvement (52.55%) was most commonly seen with a marked tendency for the anterior region (mandible: 40.9%, maxilla: 48.07%). The size of the lesion ranged from .5 cm to 20.4 cm, with the majority of cases measuring more than 3 cm in size (53.84%). Radiologically, most of the lesions presented mixed radiolucency and radiopacity (62%), and root resorption was observed in only seven cases. The majority of the lesions showed ill-defined margins upon radiographic examination (65.78%). Most of the cases were treated with resection (78.57%), and five of the 10 recurrent cases were treated by enucleation/curettage. DA is characterized by the unique presentation of clinicopathological parameters. It is not possible to comment on its aggressive/recurrent nature and best treatment modality due to inadequate follow-up data.
Topics: Ameloblastoma; Databases, Factual; Female; Humans; Jaw Neoplasms; Male; Mandible; Maxilla; Odontogenic Tumors; Radiology; Root Resorption
PubMed: 28707772
DOI: 10.1111/jicd.12282 -
Journal of Neuro-oncology Nov 2021Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are classified together as grade I neuronal and mixed neuronal-glial tumor of the... (Meta-Analysis)
Meta-Analysis
PURPOSE
Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are classified together as grade I neuronal and mixed neuronal-glial tumor of the central nervous system by the World Health Organization (WHO). These tumors are rare and have not been well characterized in terms of clinical outcomes. We aimed to identify clinical predictors of mortality and tumor recurrence/progression by performing an individual patient data meta-analysis (IPDMA) of the literature.
METHODS
A systematic literature review from 1970 to 2020 was performed, and individualized clinical data for patients diagnosed with DIA/DIG were extracted. Aggregated data were excluded from collection. Outcome measures of interest were mortality and tumor recurrence/progression, as well as time-to-event (TTE) for each of these. Participants without information on these outcome measures were excluded. Cox regression survival analyses were performed to determine predictors of mortality and tumor recurrence / progression.
RESULTS
We identified 98 articles and extracted individual patient data from 188 patients. The cohort consisted of 58.9% males with a median age of 7 months. The majority (68.1%) were DIGs, while 24.5% were DIAs and 7.5% were non-specific desmoplastic infantile tumors; DIAs presented more commonly in deep locations (p = 0.001), with leptomeningeal metastasis (p = 0.001), and was associated with decreased probability of gross total resection (GTR; p = 0.001). Gender, age, and tumor pathology were not statistically significant predictors of either mortality or tumor recurrence/progression. On multivariate survival analysis, GTR was a predictor of survival (HR = 0.058; p = 0.007) while leptomeningeal metastasis at presentation was a predictor of mortality (HR = 3.27; p = 0.025). Deep tumor location (HR = 2.93; p = 0.001) and chemotherapy administration (HR = 2.02; p = 0.017) were associated with tumor recurrence/progression.
CONCLUSION
Our IPDMA of DIA/DIG cases reported in the literature revealed that GTR was a predictor of survival while leptomeningeal metastasis at presentation was associated with mortality. Deep tumor location and chemotherapy were associated with tumor recurrence / progression.
Topics: Astrocytoma; Brain Neoplasms; Female; Ganglioglioma; Humans; Infant; Male; Meningeal Carcinomatosis; Neoplasm Recurrence, Local
PubMed: 34613581
DOI: 10.1007/s11060-021-03860-1 -
Dermatologic Surgery : Official... Apr 2023Few prospective studies have evaluated local recurrence rates (LRR) after excision of desmoplastic melanoma (DM); however, several retrospective studies have reported... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Few prospective studies have evaluated local recurrence rates (LRR) after excision of desmoplastic melanoma (DM); however, several retrospective studies have reported high LRR.
OBJECTIVE
To determine LRR after excision of DM and evaluate factors affecting LRR.
MATERIALS AND METHODS
Systematic review of the PubMed, Embase, and Web of Science databases was performed to identify studies reporting local recurrence after excision of DM with conventional wide local excision (WLE), Mohs micrographic surgery (MMS), or staged excision (SE). Meta-analysis was performed to calculate summary LRR and pooled risk ratios (RR).
RESULTS
Literature search identified 4 studies evaluating MMS or SE (total n = 61 DM). 53 studies assessed WLE ( n = 3,080) and were analyzed quantitatively. The overall LRR after WLE of DM was 21% (95% CI, 0.16-0.28; n = 2,308). Local recurrence rate was higher with positive/unknown histologic excision margins (49%, 95% CI, 0.25-0.74; n = 91) versus negative histologic margins (11%, 95% CI, 0.07-0.17; n = 1,075; [ p < .01]). Neurotropism was also associated with increased LRR (RR, 1.79; 95% CI, 1.34-2.38, p < .01; n = 644).
CONCLUSION
DM has high LRR after WLE. Local recurrence risk was greatest with positive excision margins, indicating the importance of achieving negative microscopic margins. Greater study of MMS and SE for DM is required.
Topics: Humans; Skin Neoplasms; Retrospective Studies; Margins of Excision; Prospective Studies; Neoplasm Recurrence, Local; Mohs Surgery; Melanoma
PubMed: 36857167
DOI: 10.1097/DSS.0000000000003699 -
American Journal of Clinical Oncology Dec 2014A subset of patients with melanoma present in rare and unique clinical circumstances requiring specific considerations with respect to diagnostic and therapeutic... (Review)
Review
A subset of patients with melanoma present in rare and unique clinical circumstances requiring specific considerations with respect to diagnostic and therapeutic interventions. Herein, we present our review of patients with: (1) primary mucosal melanoma of the head and neck, gastrointestinal, and genitourinary tracts; (2) primary melanoma of the eye; (3) desmoplastic melanoma; (4) subungual melanoma; (5) melanoma in special populations: children, nonwhites, as well as a discussion of familial melanoma.
Topics: Adult; Child; Eye Neoplasms; Gastrointestinal Neoplasms; Head and Neck Neoplasms; Humans; Melanoma; Mucous Membrane; Nail Diseases; Skin Neoplasms; Urogenital Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 23563206
DOI: 10.1097/COC.0b013e3182868e82 -
Frontiers in Oncology 2022Conventional parameters show limited and unreliable correlations with medulloblastoma prognosis.
BACKGROUND
Conventional parameters show limited and unreliable correlations with medulloblastoma prognosis.
AIM
To evaluate the factors influencing overall survival (OS), event-free survival (EFS), and progression-free survival (PFS) in patients with medulloblastoma.
METHODS
PubMed, EMBASE, the Cochrane Library, and Web of Science were searched for studies published up to May 2021. The associations between various clinical and treatment factors and survival parameters were assessed.
RESULTS
Twenty-nine studies (8455 patients) were included. Desmoplastic medulloblastoma (HR=0.41, 95%CI: 0.31-0.56), M0 disease (HR=2.07, 95%CI: 1.48-2.89), WNT, SSH, group 4 (all P<0.05 vs. group 3), GTR vs. STR (HR=1.37, 95%CI: 1.04-1.08), radiotherapy (HR=0.45, 95%CI: 0.20-0.80), craniospinal irradiation (HR=0.49, 95%CI: 0.38-0.64), and high 5hmC levels (HR=2.90, 95%CI: 1.85-4.55) were associated with a better OS. WNT, SSH, group 4 (all P<0.05 vs. group 3), residual tumor ≤1.5 cm (HR=2.08, 95%CI: 1.18-3.68), GTR vs. STR (HR=1.31, 95%CI: 1.03-1.68), craniospinal irradiation (HR=0.46, 95%CI: 0.37-0.57), high 5hmC levels (HR=3.10, 95%CI: 2.01-4.76), and <49 days between resection and radiotherapy (HR=2.54, 95%CI: 1.48-4.37) were associated with better PFS. Classic vs. desmoplastic medulloblastoma (HR=1.81, 95%CI: 1.04-3.16), SSH, WNT (both P<0.05 vs, non-SSH/non-WNT), GTR vs. STR (HR=2.01, 95%CI: 1.42-2.85), and radiotherapy (HR=0.31, 95%CI: 0.15-0.64) were associated with a better EFS.
CONCLUSION
Histology, molecular subgroup, GTR, and radiotherapy are significantly associated with survival parameters in patients with medulloblastoma. Nevertheless, high-quality prospective cohort studies are necessary to improve the conclusions.
PubMed: 35311074
DOI: 10.3389/fonc.2022.827054 -
Journal of Plastic, Reconstructive &... Feb 2017Desmoplastic melanoma (DM) is an uncommon malignancy associated with a high local recurrence rate. The aim of this systematic review was to determine the positivity rate... (Review)
Review
BACKGROUND
Desmoplastic melanoma (DM) is an uncommon malignancy associated with a high local recurrence rate. The aim of this systematic review was to determine the positivity rate of sentinel lymph node biopsy (SLNB) in patients with DM. The secondary outcome was to establish if SLNB is warranted for both pure DM (PDM) and mixed DM (MDM).
METHODS
A full systematic literature review of SLNB in DM was performed by two authors in January 2016. Ovid MEDLINE, Ovid EMBASE and the Cochrane Central Register of Controlled Trials were searched.
RESULTS
Sixteen studies involving 1519 patients having SLNB in DM were included, of which 99 patients had positive SLNB (6.5%). Two articles reported a significantly reduced disease-free survival (DFS) with positive SLNB and three published a reduced melanoma-specific survival (MSS). Six studies compared SLNB in MDM and PDM. Of the 275 patients, 38 (13.8%) had a positive SLNB in MDM compared to 17 of 313 patients (5.4%) with positive SLNB in PDM.
CONCLUSIONS
Rates of positive SLNB in DM are reduced compared to other variants of melanoma; however, nodal status may still predict DFS and MSS. MDM is associated with a higher rate of micro-metastases to regional lymph nodes than PDM, and DFS and MSS may be lesser in MDM than in PDM. We would recommend the consideration of SLNB in MDM. However, with such low rates of positive SLNB in PDM, and in the absence of high-risk features to stratify patients, we would not recommend SLNB in PDM.
Topics: Humans; Lymphatic Metastasis; Melanoma; Sentinel Lymph Node; Sentinel Lymph Node Biopsy; Skin Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 28017261
DOI: 10.1016/j.bjps.2016.11.003 -
International Journal of Oral and... Jun 2020The aim of this study was to integrate the available data published on desmoplastic ameloblastoma (DA) into a comprehensive analysis of its clinical/radiographic...
The aim of this study was to integrate the available data published on desmoplastic ameloblastoma (DA) into a comprehensive analysis of its clinical/radiographic features. As it is not clear whether the hybrid ameloblastoma has a unique biological behaviour, the clinical/radiographic features of conventional DA were compared with those of the hybrid variant. An electronic search was undertaken in May 2019. Eligibility criteria included publications with sufficient clinical/radiographic/histological information to confirm the diagnosis. A total of 128 publications reporting 285 DAs were included (246 central non-hybrid, 33 central hybrid, 6 peripheral). There was a statistically significant difference between non-hybrid and hybrid cases concerning lesion location, radiographic limits, and size. In contrast to non-hybrid tumours, which showed a similar distribution in the mandible and maxilla, hybrid tumours showed a high predominance of mandible location. Non-hybrid lesions more often showed ill-defined radiographic margins, and were smaller tumours than hybrid DAs. Enucleation with an additional therapy (either curettage or peripheral osteotomy) or resection led to a lower recurrence risk in comparison to curettage or enucleation without additional therapy. The location of the lesion and type of treatment are related to tumour recurrence. There are important differences in location and radiographic limits between hybrid and non-hybrid DAs, which support their classification as distinct lesions.
Topics: Ameloblastoma; Humans; Mandible; Margins of Excision; Maxilla; Neoplasm Recurrence, Local
PubMed: 31810564
DOI: 10.1016/j.ijom.2019.11.004 -
Frontiers in Oncology 2021Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor...
Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.
PubMed: 34604040
DOI: 10.3389/fonc.2021.705760 -
Journal of Plastic, Reconstructive &... Dec 2022Desmoplastic melanoma (DM) is a less common form of cutaneous melanoma that has been described for decades; however, controversy remains regarding the management and use...
BACKGROUND
Desmoplastic melanoma (DM) is a less common form of cutaneous melanoma that has been described for decades; however, controversy remains regarding the management and use of sentinel lymph node biopsy (SLNB). The purpose of this study is to identify whether SLNB is indicated in all cases of DM, including the pure subtype.
METHODS
A systematic review was conducted using PubMed (with access to MEDLINE) along with the Cochrane Central Register of Controlled Trials from 2001 to 2019. Case series and case-control studies were included.
RESULTS
Eighteen studies were included for a total population of 3,914 patients. SLNB was performed in 2229 patients. The percentage of positive SLNB results was 8.5%. However, patients with pure DM (>90% desmoplastic component) were found to have a significantly lower rate of occult metastatic node positivity when compared with that of mixed DM (4.9% and 14.8%, respectively).
CONCLUSIONS
Our findings underscore the importance of the pathologist reporting percentage of desmoplastic component in melanoma. SLNB should be strongly considered for patients with mixed DM. However, the low rate of occult metastatic node positivity in pure DM is beneath the threshold for using SLNB as a staging procedure.
SUMMARY
Previous studies have suggested that desmoplastic melanoma is less likely to metastasize to regional lymph nodes when compared with conventional melanoma. This review suggests that it is imperative to distinguish the histologic subtype of desmoplastic melanoma to determine if staging procedure is indicated.
Topics: Humans; Sentinel Lymph Node Biopsy; Melanoma; Skin Neoplasms; Lymph Nodes; Case-Control Studies; Prognosis; Sentinel Lymph Node
PubMed: 36283925
DOI: 10.1016/j.bjps.2022.08.044 -
JPMA. the Journal of the Pakistan... Dec 2020To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome.
METHODS
The systematic review comprised medical literature in English language published from January 1997 to January 2017 on Scopus, PubMed and Google Scholar databases to establish consensus about the possible mechanism of spontaneous regression, the role of therapeutic intervention and failure of management strategies in low-grade gliomas. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed during the review.
RESULTS
Of the 176 articles identified, 73(41.5%) were shortlisted for detailed assessment. Of them, 10(13.7%) were included; 5(50%) case reports and 5(50%) case series. There were 23 cases of spontaneous regression; 15(65.2%) males and 8(34.7%) females. The interval of regression varied from 3 months to 15.5 years, and the most commonly presenting low-grade glioma type was optic pathway glioma 11(47.4%).
CONCLUSIONS
The phenomenon of regression was most evident in optic pathway glioma. Literature suggested that low-grade gliomas should undergo serial imaging before implying any therapeutic intervention. However, the evidencebased proof, large-scale experimental studies and ethical considerations are still required to standardise this strategy.
Topics: Astrocytoma; Brain Neoplasms; Consensus; Female; Glioma; Humans; Language; Male
PubMed: 33475560
DOI: 10.47391/JPMA.581