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The Cochrane Database of Systematic... Feb 2023Carpal tunnel syndrome (CTS) is a compression neuropathy of the median nerve causing pain and numbness and tingling typically in the thumb, index and middle finger. It... (Review)
Review
BACKGROUND
Carpal tunnel syndrome (CTS) is a compression neuropathy of the median nerve causing pain and numbness and tingling typically in the thumb, index and middle finger. It sometimes results in muscle wasting, diminished sensitivity and loss of dexterity. Splinting the wrist (with or without the hand) using an orthosis is usually offered to people with mild-to-moderate findings, but its effectiveness remains unclear.
OBJECTIVES
To assess the effects (benefits and harms) of splinting for people with CTS.
SEARCH METHODS
On 12 December 2021, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, AMED, CINAHL, ClinicalTrials.gov, and WHO ICTRP with no limitations. We checked the reference lists of included studies and relevant systematic reviews for studies.
SELECTION CRITERIA
Randomised trials were included if the effect of splinting could be isolated from other treatment modalities. The comparisons included splinting versus no active treatment (or placebo), splinting versus another disease-modifying non-surgical treatment, and comparisons of different splint-wearing regimens. We excluded studies comparing splinting with surgery or one splint design with another. We excluded participants if they had previously undergone surgical release.
DATA COLLECTION AND ANALYSIS
Review authors independently selected trials for inclusion, extracted data, assessed study risk of bias and the certainty in the body of evidence for primary outcomes using the GRADE approach, according to standard Cochrane methodology.
MAIN RESULTS
We included 29 trials randomising 1937 adults with CTS. The trials ranged from 21 to 234 participants, with mean ages between 42 and 60 years. The mean duration of CTS symptoms was seven weeks to five years. Eight studies with 523 hands compared splinting with no active intervention (no treatment, sham-kinesiology tape or sham-laser); 20 studies compared splinting (or splinting delivered along with another non-surgical intervention) with another non-surgical intervention; and three studies compared different splinting regimens (e.g. night-time only versus full time). Trials were generally at high risk of bias for one or more domains, including lack of blinding (all included studies) and lack of information about randomisation or allocation concealment in 23 studies. For the primary comparison, splinting compared to no active treatment, splinting may provide little or no benefits in symptoms in the short term (< 3 months). The mean Boston Carpal Tunnel Questionnaire (BCTQ) Symptom Severity Scale (SSS) (scale 1 to 5, higher is worse; minimal clinically important difference (MCID) 1 point) was 0.37 points better with splint (95% confidence interval (CI) 0.82 better to 0.08 worse; 6 studies, 306 participants; low-certainty evidence) compared with no active treatment. Removing studies with high or unclear risk of bias due to lack of randomisation or allocation concealment supported our conclusion of no important effect (mean difference (MD) 0.01 points worse with splint; 95% CI 0.20 better to 0.22 worse; 3 studies, 124 participants). In the long term (> 3 months), we are uncertain about the effect of splinting on symptoms (mean BCTQ SSS 0.64 better with splinting; 95% CI 1.2 better to 0.08 better; 2 studies, 144 participants; very low-certainty evidence). Splinting probably does not improve hand function in the short term and may not improve hand function in the long term. In the short term, the mean BCTQ Functional Status Scale (FSS) (1 to 5, higher is worse; MCID 0.7 points) was 0.24 points better (95% CI 0.44 better to 0.03 better; 6 studies, 306 participants; moderate-certainty evidence) with splinting compared with no active treatment. In the long term, the mean BCTQ FSS was 0.25 points better (95% CI 0.68 better to 0.18 worse; 1 study, 34 participants; low-certainty evidence) with splinting compared with no active treatment. Night-time splinting may result in a higher rate of overall improvement in the short term (risk ratio (RR) 3.86, 95% CI 2.29 to 6.51; 1 study, 80 participants; number needed to treat for an additional beneficial outcome (NNTB) 2, 95% CI 2 to 2; low-certainty evidence). We are uncertain if splinting decreases referral to surgery, RR 0.47 (95% CI 0.14 to 1.58; 3 studies, 243 participants; very low-certainty evidence). None of the trials reported health-related quality of life. Low-certainty evidence from one study suggests that splinting may have a higher rate of adverse events, which were transient, but the 95% CIs included no effect. Seven of 40 participants (18%) reported adverse effects in the splinting group and 0 of 40 participants (0%) in the no active treatment group (RR 15.0, 95% CI 0.89 to 254.13; 1 study, 80 participants). There was low- to moderate-certainty evidence for the other comparisons: splinting may not provide additional benefits in symptoms or hand function when given together with corticosteroid injection (moderate-certainty evidence) or with rehabilitation (low-certainty evidence); nor when compared with corticosteroid (injection or oral; low certainty), exercises (low certainty), kinesiology taping (low certainty), rigid taping (low certainty), platelet-rich plasma (moderate certainty), or extracorporeal shock wave treatment (moderate certainty). Splinting for 12 weeks may not be better than six weeks, but six months of splinting may be better than six weeks of splinting in improving symptoms and function (low-certainty evidence).
AUTHORS' CONCLUSIONS
There is insufficient evidence to conclude whether splinting benefits people with CTS. Limited evidence does not exclude small improvements in CTS symptoms and hand function, but they may not be clinically important, and the clinical relevance of small differences with splinting is unclear. Low-certainty evidence suggests that people may have a greater chance of experiencing overall improvement with night-time splints than no treatment. As splinting is a relatively inexpensive intervention with no plausible long-term harms, small effects could justify its use, particularly when patients are not interested in having surgery or injections. It is unclear if a splint is optimally worn full time or at night-time only and whether long-term use is better than short-term use, but low-certainty evidence suggests that the benefits may manifest in the long term.
Topics: Adult; Humans; Middle Aged; Carpal Tunnel Syndrome; Hand; Occupational Therapy; Quality of Life; Upper Extremity
PubMed: 36848651
DOI: 10.1002/14651858.CD010003.pub2 -
Neurosurgery Jun 2023Stereotactic radiosurgery (SRS) is one of the main treatment options in the management of small to medium size vestibular schwannomas (VSs), because of high tumor... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Stereotactic radiosurgery (SRS) is one of the main treatment options in the management of small to medium size vestibular schwannomas (VSs), because of high tumor control rate and low cranial nerves morbidity. Series reporting long-term hearing outcome (>3 years) are scarce.
OBJECTIVE
To perform a systematic review of the literature and meta-analysis, with the aim of focusing on long-term hearing preservation after SRS.
METHODS
Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we reviewed articles published between January 1990 and October 2020 and referenced in PubMed or Embase. Inclusion criteria were peer-reviewed clinical study or case series of VSs treated with SRS (single dose), reporting hearing outcome after SRS with a median or mean audiometric follow-up of at least 5 years. Hearing preservation, cranial nerves outcomes, and tumor control were evaluated.
RESULTS
Twenty-three studies were included. Hearing preservation was found in 59.4% of cases (median follow-up 6.7 years, 1409 patients). Main favorable prognostic factors were young age, good hearing status, early treatment after diagnosis, small tumor volume, low marginal irradiation dose, and maximal dose to the cochlea. Tumor control was achieved in 96.1%. Facial nerve deficit and trigeminal neuropathy were found in 1.3% and 3.2% of patients, respectively, both significantly higher in Linear Accelerator series than Gamma Knife series ( P < .05).
CONCLUSION
Long-term hearing preservation remains one of the main issues after SRS, with a major impact on health-related quality of life. Our meta-analysis suggests that hearing preservation can be achieved in almost 60% of patients after a median follow-up of 6.7 years, irrespective of the technique.
Topics: Humans; Neuroma, Acoustic; Radiosurgery; Treatment Outcome; Quality of Life; Hearing; Follow-Up Studies; Retrospective Studies
PubMed: 36735500
DOI: 10.1227/neu.0000000000002354 -
The Cochrane Database of Systematic... Feb 2023Carpal tunnel syndrome (CTS) is a very common clinical syndrome manifested by signs and symptoms of irritation of the median nerve at the carpal tunnel in the wrist.... (Review)
Review
BACKGROUND
Carpal tunnel syndrome (CTS) is a very common clinical syndrome manifested by signs and symptoms of irritation of the median nerve at the carpal tunnel in the wrist. Direct and indirect costs of CTS are substantial, with estimated costs of two billion US dollars for CTS surgery in the USA in 1995 alone. Local corticosteroid injection has been used as a non-surgical treatment for CTS many years, but its effectiveness is still debated.
OBJECTIVES
To evaluate the benefits and harms of corticosteroids injected in or around the carpal tunnel for the treatment of carpal tunnel syndrome compared to no treatment or a placebo injection.
SEARCH METHODS
We used standard, extensive Cochrane search Methods. The searches were 7 June 2020 and 26 May 2022.
SELECTION CRITERIA
We included randomised controlled trials (RCTs) or quasi-randomised trials of adults with CTS that included at least one comparison group of local injection of corticosteroid (LCI) into the wrist and one group that received a placebo or no treatment.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methods. Our primary outcome was 1. improvement in symptoms at up to three months of follow-up. Our secondary outcomes were 2. functional improvement, 3. improvement in symptoms at greater than three months of follow-up, 4. improvement in neurophysiological parameters, 5. improvement in imaging parameters, 6. requirement for carpal tunnel surgery, 7. improvement in quality of life and 8.
ADVERSE EVENTS
We used GRADE to assess the certainty of evidence for each outcome.
MAIN RESULTS
We included 14 trials with 994 participants/hands with CTS. Only nine studies (639 participants/hands) had useable data quantitatively and in general, these studies were at low risk of bias except for one quite high-risk study. The trials were conducted in hospital-based clinics across North America, Europe, Asia and the Middle East. All trials used participant-reported outcome measures for symptoms, function and quality of life. There is probably an improvement in symptoms measured at up to three months of follow-up favouring LCI (standardised mean difference (SMD) -0.77, 95% confidence interval (CI) -0.94 to -0.59; 8 RCTs, 579 participants; moderate-certainty evidence). Up to six months this was still evident favouring LCI (SMD -0.58, 95% CI -0.89 to -0.28; 4 RCTs, 234 participants/hands; moderate-certainty evidence). There is probably an improvement in function measured at up to three months favouring LCI (SMD -0.62, 95% CI -0.87 to -0.38; 7 RCTs, 499 participants; moderate-certainty evidence). We are uncertain if there is a difference in median nerve DML at up to three months of follow-up (mean difference (MD) -0.37 ms, 95% CI -0.75 to 0.02; 6 RCTs, 359 participants/hands; very low-certainty evidence). The requirement for surgery probably reduces slightly in the LCI group at one year (risk ratio 0.84, 95% CI 0.72 to 0.98; 1 RCT, 111 participants, moderate-certainty evidence). Quality of life, measured at up to three months of follow-up using the Short-Form 6 Dimensions questionnaire (scale from 0.29 to 1.0; higher is better) probably improved slightly in the LCI group (MD 0.07, 95% CI 0.02 to 0.12; 1 RCT, 111 participants; moderate-certainty evidence). Adverse events were uncommon (low-certainty evidence). One study reported 2/364 injections resulted in severe pain which resolved over "several weeks" and 1/364 injections caused a "sympathetic reaction" with a cool, pale hand that completely resolved in 20 minutes. One study (111 participants) reported no serious adverse events, but 65% of LCI-injected and 16% of the placebo-injected participants experienced mild-to-moderate pain lasting less than two weeks. About 9% of participants experienced localised swelling lasting less than two weeks. Four studies (229 participants) reported that they experienced no adverse events in their studies. Three studies (220 participants) did not specifically report adverse events.
AUTHORS' CONCLUSIONS
Local corticosteroid injection is effective for the treatment of mild and moderate CTS with benefits lasting up to six months and a reduced need for surgery up to 12 months. Where serious adverse events were reported, they were rare.
Topics: Adult; Humans; Adrenal Cortex Hormones; Carpal Tunnel Syndrome; Hand; Randomized Controlled Trials as Topic
PubMed: 36722795
DOI: 10.1002/14651858.CD015148 -
Clinical Neurology and Neurosurgery Feb 2023This study aims to describe the clinical characteristics of patients with isolated oculomotor nerve palsy from COVID-19 infection, and provide guidance on their...
AIM
This study aims to describe the clinical characteristics of patients with isolated oculomotor nerve palsy from COVID-19 infection, and provide guidance on their treatment and management.
METHODS
We performed a systematic review and retrospective analysis on the clinical features and outcomes of patients with isolated oculomotor nerve palsy from COVID-19 reported in literature over the past three years.
RESULTS
We analyzed a total of 11 cases; 9 identified in literature from January 2020 to September 2022, together with our two patients. Their median age was 46 years (range 2-65), and three were children. More than half (6/11, 55 %) were without medical history. Oculomotor nerve palsies tended to occur early (longest interval of 16 days), but they can also occur concurrently (2/11, 18 %) or before the appearance of COVID-19 symptoms (1/11, 9 %). COVID-19 symptoms tended to be mild (8/11, 73 %). Oculomotor nerve palsies, however, displayed neither a clear gender predilection, nor consistent clinical features in terms of the severity of extraocular weakness and the involvement of pupillary light responses. Nearly two-thirds (7/11, 64 %) received no pharmacological treatment. Regardless, recovery was complete in nearly all (9/10, 90 %), with most occurring within a month (8/9, 89 %) CONCLUSION: Isolated oculomotor nerve palsies are early but uncommon complications of COVID-19. They affect patients with mild infections, and can be the first symptom. Prognosis is excellent, with recovery being often complete and early. Early discharge and outpatient clinical review, with or without short courses of oral steroids, are reasonable treatment measures.
Topics: Child; Humans; Child, Preschool; Adolescent; Young Adult; Adult; Middle Aged; Aged; Retrospective Studies; COVID-19; Oculomotor Nerve Diseases; Prognosis; Oculomotor Nerve
PubMed: 36696848
DOI: 10.1016/j.clineuro.2023.107601 -
BMC Anesthesiology Dec 2022The objective of this systematic review and network meta-analysis was to compare the effects of single-shot ultrasound-guided regional anesthesia techniques on... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The objective of this systematic review and network meta-analysis was to compare the effects of single-shot ultrasound-guided regional anesthesia techniques on postoperative opioid consumption in patients undergoing open cardiac surgery.
METHODS
This systematic review and network meta-analysis involved cardiac surgical patients (age > 18 y) requiring median sternotomy. We searched PubMed, EMBASE, The Cochrane Central Register of Controlled Trials (CENTRAL), Scopus, and Web of Science. The effects of the single-shot ultrasound-guided regional anesthesia technique were compared with those of placebo and no intervention. We conducted a risk assessment of bias for eligible studies and assessed the overall quality of evidence for each outcome.
RESULTS
The primary outcome was opioid consumption during the first 24 h after surgery. The secondary outcomes were pain after extubation at 12 and 24 h, postoperative nausea and vomiting, extubation time, intensive care unit discharge time, and length of hospital stay. Fifteen studies with 849 patients were included. The regional anesthesia techniques included pecto-intercostal fascial block, transversus thoracis muscle plane block, erector spinae plane (ESP) block, and pectoralis nerve block I. All the regional anesthesia techniques included significantly reduced postoperative opioid consumption at 24 h, expressed as morphine milligram equivalents (MME). The ESP block was the most effective treatment (-22.93 MME [-34.29;-11.56]).
CONCLUSIONS
In this meta-analysis, we concluded that fascial plane blocks were better than placebo when evaluating 24 h MMEs. However, it is still challenging to determine which is better, given the paucity of studies available in the literature. More randomized controlled trials are required to determine which regional anesthesia technique is better.
TRIAL REGISTRATION
PROSPERO; CRD42022315497.
Topics: Humans; Adult; Middle Aged; Analgesics, Opioid; Network Meta-Analysis; Pain, Postoperative; Anesthesia, Conduction; Cardiac Surgical Procedures; Ultrasonography, Interventional
PubMed: 36581838
DOI: 10.1186/s12871-022-01952-7 -
Health Security 2023Plague meningitis is a serious and often fatal manifestation of infection. In the aftermath of a bioweapon attack with , this typically rare manifestation may develop...
Plague meningitis is a serious and often fatal manifestation of infection. In the aftermath of a bioweapon attack with , this typically rare manifestation may develop in a substantial number of patients, particularly if treatment delays occur. Risk factors, clinical evolution, and optimal treatment strategies for plague meningitis are not well understood. We searched PubMed Central and other databases for reports of plague meningitis in any language. Articles containing descriptions of patients with plague meningitis and their treatment and outcomes were included. Among 1,496 articles identified in our search, 56 articles describing 84 cases from 1898 to 2015 met inclusion criteria. The median age of patients was 16 years (range 6 weeks to 64 years); 68% were male. Most patients (n = 50, 60%) developed meningitis following primary bubonic plague. Common signs and symptoms included fever (n = 56, 66%), nuchal rigidity (n = 38, 45%), and headache (n = 33, 36%); 29% (n = 24) of patients had focal neurologic deficits such as cranial nerve abnormalities. Almost all (n = 23, 96%) of the 24 patients who did not receive antimicrobials died, and 42% (n = 25) of the 59 patients treated with antimicrobials died. The case fatality rate of patients grouped by antimicrobial received was 50% (1 out of 2) for fluoroquinolones, 19% (4 out of 21) for aminoglycosides, 14% (2 out of 14) for sulfonamides, 11% (2 out of 18) for chloramphenicol, and 0% (0 out of 13) for tetracyclines. Plague meningitis most often occurs as a complication of bubonic plague and can cause focal neurologic deficits. Survival is more likely in patients who receive antimicrobials; tetracyclines, aminoglycosides, and chloramphenicol had the lowest associated case fatality rates.
Topics: Humans; Male; Infant; Female; Plague; Anti-Bacterial Agents; Chloramphenicol; Aminoglycosides; Meningitis; Disease Progression
PubMed: 36576503
DOI: 10.1089/hs.2022.0081 -
Journal of Neurophysiology Jan 2023Neurological manifestations associated with Coronavirus Disease-2019 (COVID-19) are commonly reported, but patients were not referred to perform the electrophysiological... (Review)
Review
Neurological manifestations associated with Coronavirus Disease-2019 (COVID-19) are commonly reported, but patients were not referred to perform the electrophysiological assessment. We aimed to review the existing literature on clinical studies on COVID-19 peripheral neuropathy to correlate patients' symptoms and characteristics with nerve conduction studies/electromyography (NCS/EMG) outcomes. This protocol is registered in the Open Science Framework (https://www.doi.org/10.17605/OSF.IO/ZF4PK). The systematic search included PubMed, ScienceDirect, and Google Scholar, for articles published from December 2019 to March 2022. A total of 727 articles were collected, and according to our inclusion and exclusion criteria, only 6 articles were included. Of 195 participants, only 175 underwent NCS/EMG assessment. Of these, 44 participants (25.1%) had abnormal EMG, 54 participants (30.8%) had abnormal motor NCS, and only 7 participants (4%) had abnormal sensory NCS. All cases presented with myopathy, while a limited number of cases presented with polyneuropathy. According to motor NCS and EMG, the most affected nerves were the tibial and peroneal in the lower extremities and the ulnar nerve in the upper extremities. Interestingly, the median nerve was reported to be associated with the severity and the rate of motor recovery of patients with COVID-19. COVID-19 generates a demyelinating motor neuropathy and myopathy. Clinicians are encouraged to refer patients with COVID-19 presenting with neurological symptoms to be assessed by electrophysiological methods to objectively determine the nature of their symptoms, follow their prognosis, and plan their rehabilitation.
Topics: Humans; Peripheral Nervous System Diseases; Neural Conduction; COVID-19; Polyneuropathies; Electromyography; Muscular Diseases
PubMed: 36475865
DOI: 10.1152/jn.00386.2022 -
The Journal of International Medical... Nov 2022To identify whether median nerve stimulation (MNS) may be a potential candidate for the treatment of consciousness disorders via a systematic review and meta-analysis. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To identify whether median nerve stimulation (MNS) may be a potential candidate for the treatment of consciousness disorders via a systematic review and meta-analysis.
METHODS
PubMed, Cochrane Library, China National Knowledge Infrastructure, Chinese VIP Information, Wanfang, and SinoMed databases were searched. Risk of bias was assessed using the Cochrane Collaboration's tool. The Glasgow Coma Scale (GCS), Disability Rating Scale (DRS), electroencephalogram (EEG), days in the Intensive Care Unit (ICU), and cerebral blood flow measures were compared between the median nerve stimulation and control groups. The meta-analysis was conducted using Review Manager software.
RESULTS
We identified 2244 studies, of which 23 (with data from 1856 patients) qualified for the analysis. MNS improved GCS scores (mean difference [MD] = 2.15), EEG scores (MD = 1.61), cerebral mean blood flow velocity (MD = 4.23), and cerebral systolic blood flow velocity (MD = 10.51). Furthermore, it decreased DRS scores (MD = -1.77) and days in the ICU (MD = -2.02). The effects of MNS on GCS scores increased with longer treatments (1 week, MD = 1.03; 1 month, MD = 2.35) and were better with right MNS (right, MD = 2.36; bilateral, MD = 1.72).
CONCLUSIONS
MNS may promote recovery from consciousness disorders.
Topics: Humans; Consciousness Disorders; Median Nerve; Electric Stimulation; Electroencephalography; Asian People
PubMed: 36448965
DOI: 10.1177/03000605221134467 -
Frontiers in Pharmacology 2022In recent years, people pay more and more attention to diabetic peripheral neuropathy (DPN). As a neurotrophic agent, mecobalamin is able to repaire nerves, which has...
The efficacy and safety of mecobalamin combined with Chinese medicine injections in the treatment of diabetic peripheral neuropathy: A systematic review and Bayesian network meta-analysis of randomized controlled trials.
In recent years, people pay more and more attention to diabetic peripheral neuropathy (DPN). As a neurotrophic agent, mecobalamin is able to repaire nerves, which has already become a consensus among experts. However, it has been found that mecobalamin has poor effect to increase nerve conduction velocity, which is an important indicator. Clinical data have shown that Chinese medicine injection, combined with mecobalamin injection, can significantly improve nerve conduction velocity of the limbs. Nevertheless, several kinds of Chinese medicine injections have been used to treat DPN. The effect of these Chinese medicine injections for DPN are various. Therefore, it is necessary to evaluate the effectiveness of Chinese medicine injections combined with mecobalamin in the treatment of DPN. All relevant articles published before 12 March 2022 were searched in eight electronic databases. Randomized controlled trials (RCTs) on Chinese medicine injections plus Mecobalamin for DPN were identified according to inclusion criteria, and were assessed using the revised Cochrane risk of bias tool (ROB2.0). R software and stata15 was used to create the ranking probabilities and network meta-analysis. A total of 80 RCTs involving 6,980 patients were included. The results showed that mecobalamin plus Dengzhanxixin injection (ME + DZXX) ranked first in overall response rate [RR = 1.64, 95% CI (1.26, 2.21)] and median motor nerve conduction velocity [MD = 9.46, 95% CI (5.67, 13.28)]. Then, mecobalamin plus Kudiezi Injection (ME + KDZ) had the best effect in median sensory nerve conduction velocity [MD = 10.41, 95% CI (-13.31, -7.52)], and mecobalamin plus Honghua injection (ME + HH) ranked highest in common peroneal motor nerve conduction velocity [MD = 6.8, 95% CI (4.13, 9.49)] and common peroneal sensory nerve conduction velocity [MD = -6.25, 95% CI (-8.85, -3.65)]. This study determined the efficacy of different Chinese medicine injections combined with mecobalamin. DZXX may be the best adjunctive Chinese medicine injection for DPN patients. However, due to potential risk of bias and limited RCTs, our results need to be treated with reservations.
PubMed: 36408262
DOI: 10.3389/fphar.2022.957483 -
Acta Neurochirurgica Apr 2023Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of...
PURPOSE
Hybrid peripheral nerve sheath tumors (HPNST) are a newly recognized class of peripheral nerve sheath tumor, composed of at least two areas characteristic of perineurioma, schwannoma, or neurofibroma. The literature consists only of case reports and small series; therefore, we present an illustrative case and an analysis of all reported cases of HPNST with a perineurioma component in the literature.
METHODS
A systematic search of the literature was performed to identify all reported cases of hybrid perineurioma-schwannoma or perineurioma-neurofibroma in the world's literature. Individual cases were analyzed for demographics, clinical features, imaging, and outcomes.
RESULTS
A total of 159 cases were identified across 41 studies. Hybrid tumors tended to present in mid-adulthood (median 38.5 years), predominantly affected females (57%, 89/156), as a painless (63%, 63/100) mass, or swelling. Ten patients (10/74, 14%) had a history of neurofibromatosis 1, and 2 patients a history of neurofibromatosis 2 (2/74, 3%). The majority (78%, 122/157) of cases occurred superficially, most commonly in the lower extremity (25%, 39/157). Perineurioma-schwannoma was the most reported (86%, 137/159) pathologic diagnosis, with 3 cases presenting with malignant features. Two cases reocurred after resection.
CONCLUSION
HPNST tend to occur in mid-adulthood and present as slowly progressive, painless, superficial masses, with a heterogeneous appearance on imaging. These entities pose a unique diagnostic challenge and likely remain under-recognized in the literature and current clinical practice. They pose low risk of recurrence or malignant transformation, and future work regarding the association with neurofibromatosis and genetic profiles is needed.
Topics: Female; Humans; Adult; Nerve Sheath Neoplasms; Neurilemmoma; Neurofibroma; Neurofibromatosis 2; Neurofibromatosis 1
PubMed: 36396843
DOI: 10.1007/s00701-022-05413-5