-
Pediatric Dermatology Mar 2020Neurothekeoma is a rare, benign, cutaneous neoplasm consisting of Schwann cells and perineural cells in myxoid stroma. Cellular neurothekeoma (CNT) was previously...
BACKGROUND/OBJECTIVES
Neurothekeoma is a rare, benign, cutaneous neoplasm consisting of Schwann cells and perineural cells in myxoid stroma. Cellular neurothekeoma (CNT) was previously thought to represent a morphologic variant of neurothekeoma, but recent studies have shown that CNTs are unrelated to neurothekeomas and are more likely of histiocytic lineage.
METHODS
Herein, we describe seven cases of CNT in pediatric patients. A comprehensive search of PubMed was performed, and 71 cases of cellular neurothekeoma in pediatric patients were reviewed.
RESULTS
The clinical differential diagnosis for these lesions included Spitz nevi, keloid, juvenile xanthogranuloma, cutaneous lymphoid hyperplasia, and lymphomatoid papulosis. All cases were treated by excision or excisional biopsy. Histopathologically, all demonstrated multilobular, primarily intradermal neoplasms composed of plump spindled or epithelioid mononuclear cells with abundant eosinophilic pale-staining cytoplasm. Immunophenotypic findings included CD68 and NKI/C3 positivity, and negative staining with cytokeratin, S-100, Melan-A, and SOX-10.
CONCLUSION
Cellular neurothekeoma is distinguished from conventional neurothekeoma by increased cellularity, a lack of myxoid stroma, and a lack of neural expression with immunohistochemical stains. These uncommon neoplasms should be included in the differential diagnosis of dermal nodules in children. Accurate diagnosis of these lesions is essential, as they can be mistaken for malignancy leading to unnecessary treatment.
Topics: Adolescent; Child; Child, Preschool; Cohort Studies; Female; Humans; Infant; Male; Neurothekeoma; Skin Neoplasms
PubMed: 31930561
DOI: 10.1111/pde.14043 -
Reviews in Cardiovascular Medicine Dec 2019Fever of unknown origin refers to a prolonged fever with an unknown cause despite adequate medical evaluations. This condition often leads to unnecessary extensive...
Fever of unknown origin refers to a prolonged fever with an unknown cause despite adequate medical evaluations. This condition often leads to unnecessary extensive laboratory work-ups and antimicrobial therapies. The atypical presentations often cause a delayed diagnosis and an improper treatment with an increased morbidity rate. In cardiac surgical patients, fever of unknown origin remains an intriguing problem during the diagnostic process of cardiac surgical diseases. Cardiac myxoma or aortic dissection are often misdiagnosed when patients present with fever of unknown origin as an onset symptom. Under such circumstances, medical examinations by echocardiography and chest computed tomography, particularly fluorodeoxyglucose-positron emission tomography/computed tomography, have been proved crucial for early diagnosis. A better understanding of the clinical features of cardiac surgical disorders presenting with fever of unknown origin would facilitate early diagnosis of fever of unknown origin. A further decision-making of prompt treatment of choices of a cardiac operation is important for improving patients' outcomes.
Topics: Cardiovascular Diseases; Diagnosis, Differential; Fever of Unknown Origin; Humans; Predictive Value of Tests; Prognosis; Risk Assessment; Risk Factors
PubMed: 31912716
DOI: 10.31083/j.rcm.2019.04.565 -
The British Journal of Oral &... Dec 2019Our aim was to establish the recurrence rate of odontogenic myxoma after different treatments. Our search covered papers from 1972-2017 from different sources. The...
Our aim was to establish the recurrence rate of odontogenic myxoma after different treatments. Our search covered papers from 1972-2017 from different sources. The papers were evaluated and critically appraised by two independent investigators. The recurrence rate and 95% CI were calculated in relation to each specific treatment, and the chi squared test was calculated to find out if there was any significant difference in the recurrence rate between conservative treatment and resection. The overall recurrence rate was 5 of 39 patients (13%) during a mean follow up period of 10 years. With conservative treatment the recurrence rate was 4/22 (19%) (mean follow up 11 years) and after resection it was 1/17 (6%) (mean follow up nine years). Maxillary lesions were more likely to recur than mandibular ones. Quality of life variables such as disfigurement and neural deficit were more common after resection than with conservative treatment. The frequency of recurrence was relatively low over 10 years' follow up, irrespective of whether resection or a more conservative approach was used, despite being slightly lower (as might be expected) after resection. Conservative treatment should be considered first to avoid resection-associated morbidity and the effect on the quality of life. Maxillary lesions have more room to spread before they are clinically evident, making them difficult to treat optimally and contributing to the recurrence rate.
Topics: Humans; Maxilla; Myxoma; Neoplasm Recurrence, Local; Odontogenic Tumors; Quality of Life
PubMed: 31551163
DOI: 10.1016/j.bjoms.2019.09.005 -
Journal of Neurosurgery Nov 2018Primary intracranial myxomas (PICMs) are extremely rare neoplasms, and their management and prognostic factors remain ambiguous. The authors aimed to elaborate the...
OBJECTIVE
Primary intracranial myxomas (PICMs) are extremely rare neoplasms, and their management and prognostic factors remain ambiguous. The authors aimed to elaborate the radiological features, evaluate the risk factors for progression-free survival (PFS), and propose a treatment protocol based on pooled data from cases treated at their institute and those found in the literature.
METHODS
Clinical data from all cases of PICMs treated at the authors' institute and those cases reported in the English-language literature between 1987 and December 2017 were reviewed. The authors searched the Ovid MEDLINE, Embase, PubMed, and Cochrane databases using the keywords "myxoma" and "central nervous system," "intracranial," "cerebral," "skull base," "skull," or "brain." Previously published data were processed and used according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Risk factors in the pooled cohort were evaluated.
RESULTS
Cases from the authors' institute included 21 males and 9 females, with a mean age of 35.7 ± 1.7 years. Gross-total resection (GTR) and non-GTR were achieved in 6 (20.0%) and 24 (80.0%) patients, respectively. After a mean follow-up of 86.7 ± 14.1 months, recurrence occurred in 6 (24%) patients, for a median PFS time of 85.2 months (range 36.0-136.0 months) and no deaths. In the literature between 1987 and 2017, 35 cases of PICM were identified in 14 males and 21 females with a mean age of 31.7 ± 3.2 years. GTR and non-GTR were achieved in 23 (65.7%) and 9 (25.7%) cases, respectively. After a mean follow-up of 25.8 ± 6.9 months (range 1.0-156.0 months), recurrence occurred in 4 (14.3%) patients, for a median PFS time of 11.0 months (range 3.0-36.0 months) and no deaths. Actuarial PFS rates at 1, 5, and 10 years were 93.0%, 80.6%, and 67.9%, respectively. A multivariate model demonstrated that GTR (HR 0.058, 95% CI 0.005-0.680, p = 0.023) was the only factor that favored PFS.
CONCLUSIONS
PICMs are rare neoplasms with a slightly higher occurrence in males. GTR was the only favorable factor for PFS. Based on statistical results, GTR alone, if tolerable, is advocated as the optimal treatment for PICM. Nevertheless, conservative excision may be preferred to avoid damage to vital structures. PICMs have a tendency to recur within a few years of the initial surgery if resection is incomplete; therefore, close postoperative follow-up is mandatory. Future studies with larger cohorts are necessary to verify the study findings.Systematic review registration no.: CRD42018091517 (www.crd.york.ac.uk/prospero/).
PubMed: 30544354
DOI: 10.3171/2018.6.JNS181132 -
Dento Maxillo Facial Radiology Feb 2018Therapeutic assessment of odontogenic myxoma (OM) is poorly standardized. Unidimensional size criteria have shown to be unreliable in therapeutic decision-making. We... (Review)
Review
OBJECTIVES
Therapeutic assessment of odontogenic myxoma (OM) is poorly standardized. Unidimensional size criteria have shown to be unreliable in therapeutic decision-making. We evaluate the size distribution of OM and scan for associated clinicoradiological signs of aggressiveness. Additionally, we evaluate three-dimensional size delineation of OM aiming to improve future therapeutic assessment of this destructive neoplasm.
METHODS
Primarily, we reviewed the database "PubMed" for data concerning the size of OMs as radiologically determined. Afterwards, the impact of age, sex, locularity and location on the size was investigated by χ² test, Student's t-test and regression analysis. Furthermore, we statistically evaluated the impact of size on the occurrence of clinicoradiological signs of aggressiveness. Secondly, we approximated the volume of five unpublished cases of OM by semi-automatic image segmentation of cone-beam CT images.
RESULTS
Multilocular OMs were significantly larger than unilocular ones (p < 0.002). Age (0.042) and multilocularity (<0.002) significantly impacted size. Size was significantly associated with cortical perforation (0.032) and multilocularity (<0.002), further regression analysis revealed tooth resorption (0.019), cortical perforation (0.005) and multilocularity (<0.002) as significant predictors of size. Employing the volume as a mean of comparison, we found that the biggest OM (38.42 ml; multilocular) was 124 times larger than the smallest (0.31 ml; unilocular). However, using the maximum diameter (cm) as a surrogate for size, the biggest lesion (6.3) was only 5.25 times larger than the smallest (1.2).
CONCLUSIONS
Locularity and volumetric size characterization might help in therapeutic decision-making and could help to improve our understanding of OM.
Topics: Cone-Beam Computed Tomography; Humans; Imaging, Three-Dimensional; Myxoma; Odontogenic Tumors; Tumor Burden
PubMed: 29082773
DOI: 10.1259/dmfr.20170262 -
World Journal For Pediatric &... Sep 2017This systematic review sought to investigate the current evidence regarding surgical management of primary cardiac tumors in children and adolescents. Twenty-eight... (Review)
Review
This systematic review sought to investigate the current evidence regarding surgical management of primary cardiac tumors in children and adolescents. Twenty-eight studies were deemed eligible, reporting on 745 pediatric patients. Rhabdomyoma was the most prevalent histologic type and echocardiography was the most common diagnostic tool. Cumulative 30-day mortality rate was 6.7%. Rhabdomyomas and teratomas had the highest 30-day mortality. The higher percentage of tumor relapse was noted for myxoma and teratoma. Although cardiac tumors are rare, their atypical clinical presentation, potential for recurrence, and the poor prognosis associated with recurrence elucidate the need for reliable diagnostic and therapeutic management.
Topics: Cardiac Surgical Procedures; Child; Echocardiography; Global Health; Heart Neoplasms; Humans; Prevalence
PubMed: 28901236
DOI: 10.1177/2150135117723904 -
Ophthalmic Plastic and Reconstructive... 2018To summarize the clinical, radiographic, and intraoperative characteristics of isolated neurofibromas of the orbit (INFO) in the literature and report a case of INFO. (Review)
Review
PURPOSE
To summarize the clinical, radiographic, and intraoperative characteristics of isolated neurofibromas of the orbit (INFO) in the literature and report a case of INFO.
METHODS
Case report and a systematic review of the literature.
RESULTS
A total of 45 patients were identified from 18 previous studies. There was no sex predilection and mean ages were between 32.3 and 40.0 years with a standard deviation of 9.8 to 19.5 years, median age was 32 to 38 years with a range of 1.5 to 82 years. On CT imaging, INFO reveals homogeneous precontrast radiodensity similar to that of extraocular muscles. On MR imaging, INFO appears hypointense to orbital fat and isointense to brain on precontrast T1-weighted images. On T2-weighted images, they have been described as being predominantly hyperintense with possible hypointense intralesional islands. To the best of our knowledge, our case identifies the youngest patient with the myxoid histologic variant of INFO.
CONCLUSIONS
Patients with orbital neurofibromas should be evaluated for the presence of systemic neurofibromatosis, and the plexiform variant is a commonly associated subtype. The myxoid variant can be part of a lesser known condition called NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma, and ephelides) which may warrant echocardiography in patients to evaluate for associated cardiac tumors.
Topics: Child, Preschool; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Neurofibroma; Orbit; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 28582370
DOI: 10.1097/IOP.0000000000000930 -
Oncology Letters Jan 2016Intramuscular myxoma (IM) is a rare mesenchymal tumor of the head and neck region. The current study reports a case of a 45-year-old man who presented with a painless...
Intramuscular myxoma (IM) is a rare mesenchymal tumor of the head and neck region. The current study reports a case of a 45-year-old man who presented with a painless neck mass. Imaging showed involvement of the levator scapulae and scalene muscles. Core needle biopsy was consistent with intramuscular myxoma. Surgical excision was performed and follow-up for 30 months showed no recurrence. The present study includes a systematic review of head and neck IMs, with a summary of the clinical and demographic parameters of all reported cases in the head and neck region. Surgery was curative in 28 of the 29 published cases, as well as in the current case (96.7%), with the lone recurrent tumor cured following re-resection. Females constituted 57% of the cases and the mean age was 49.7±20.4 years. Although uncommon, IM should be considered in the differential diagnosis of deep neck masses, and surgical excision is the treatment of choice with a low risk of recurrence.
PubMed: 26870235
DOI: 10.3892/ol.2015.3864 -
Brazilian Journal of Cardiovascular... 2015This study aims to present an updated clinical picture of the infected cardiac myxoma. Revankar & Clark made a systematic review of infected cardiac myxoma based on the... (Review)
Review
OBJECTIVE
This study aims to present an updated clinical picture of the infected cardiac myxoma. Revankar & Clark made a systematic review of infected cardiac myxoma based on the literature before 1998. Since then, there has not been any updated information describing its recent changing trends.
METHODS
A comprehensive literature search of infected cardiac myxoma was conducted on MEDLINE, Highwire Press and Google between 1998 and 2014.
RESULTS
In comparison with Revankar & Clark's series, the present series disclosed a significantly decreased overall mortality. It is believed that refinement of the prompt diagnosis and timely management (use of sensitive antibiotics and surgical resection of the infected myxoma) have resulted in better outcomes of such patients.
CONCLUSION
The present series of infected cardiac myxoma illustrated some aggravated clinical manifestations (relative more occasions of high-grade fever, multiple embolic events and the presence of refractory microorganisms), which should draw enough attention to careful diagnosis and treatment. In general, the prognosis of infected cardiac myxoma is relatively benign and the long-term survival is always promising.
Topics: Embolism; Fever; Heart Neoplasms; Humans; Myxoma; Streptococcal Infections
PubMed: 26735605
DOI: 10.5935/1678-9741.20140112 -
F1000Research 2015: Studies have reported varied prevalence estimates of coronary artery disease (CAD) in cardiac myxoma patients. We performed a systematic review and meta-analysis of...
: Studies have reported varied prevalence estimates of coronary artery disease (CAD) in cardiac myxoma patients. We performed a systematic review and meta-analysis of observational studies to summarize the point prevalence of CAD in adults with cardiac myxomas. : Two independent investigators searched MEDLINE and LILACS databases using the terms " ", " " and " " from inception through December 2014 for all relevant studies. We included 6 observational studies. Publication bias was evaluated through Egger's test and Trim and Fill method. A pooled estimate of CAD prevalence with corresponding 95% confidence interval (CI) was calculated based on a random-effects model. The pooled CAD prevalence in adult cardiac myxoma patients was 20.7% with low heterogeneity (I = 14.86%). : It is a matter of debate if preoperative coronary angiography must be done as a routine procedure. Although coronary disease and angiographically detectable neovascularity can alter surgical management, more studies are needed to evaluate this question.
PubMed: 28620449
DOI: 10.12688/f1000research.6641.1