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Article ; Online: Therapeutic Strategies for Gliomas Associated With Cancer Predisposition Syndromes.

Lam, Keng / Kamiya-Matsuoka, Carlos / Slopis, John M / McCutcheon, Ian E / Majd, Nazanin K

JCO precision oncology

2024 Volume 8, Page(s) e2300442

Abstract: Purpose: The purpose of this article was to provide an overview of syndromic gliomas.: Design: The authors conducted a nonsystematic literature review.: Results: Cancer predisposition syndromes (CPSs) are genetic conditions that increase one's ... ...

Abstract	Purpose: The purpose of this article was to provide an overview of syndromic gliomas. Design: The authors conducted a nonsystematic literature review. Results: Cancer predisposition syndromes (CPSs) are genetic conditions that increase one's risk for certain types of cancer compared with the general population. Syndromes that can predispose one to developing gliomas include neurofibromatosis, Li-Fraumeni syndrome, Lynch syndrome, and tuberous sclerosis complex. The standard treatment for sporadic glioma may involve resection, radiation therapy, and/or alkylating chemotherapy. However, DNA-damaging approaches, such as radiation and alkylating agents, may increase the risk of secondary malignancies and other complications in patients with CPSs. In some cases, depending on genetic aberrations, targeted therapies or immunotherapeutic approaches may be considered. Data on clinical characteristics, therapeutic strategies, and prognosis of syndromic gliomas remain limited. Conclusion: In this review, we provide an overview of syndromic gliomas with a focus on management for patients with CPSs and the role of novel treatments that can be considered.
MeSH term(s)	Humans ; Glioma/genetics ; Glioma/therapy ; Li-Fraumeni Syndrome/complications ; Li-Fraumeni Syndrome/diagnosis ; Li-Fraumeni Syndrome/genetics ; Prognosis ; Genotype
Language	English
Publishing date	2024-02-23
Publishing country	United States
Document type	Journal Article ; Review
ISSN	2473-4284
ISSN (online)	2473-4284
DOI	10.1200/PO.23.00442
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Article ; Online: Surgical treatment of common peroneal neuropathy in schwannomatosis: illustrative cases.

North, Robert Y / Snyder, Rita / Slopis, John M / McCutcheon, Ian E

Journal of neurosurgery. Case lessons

2021 Volume 1, Issue 26, Page(s) CASE21176

Abstract: Background: Neurofibromatosis syndromes such as neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis often result in painful symptoms related to tumor burden.: Observations: Painful symptoms classically associated with common ... ...

Abstract	Background: Neurofibromatosis syndromes such as neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis often result in painful symptoms related to tumor burden. Observations: Painful symptoms classically associated with common points of peripheral nerve entrapment, such as common peroneal neuropathy at the fibular tunnel, may present in patients both with and without focal tumor involvement. Lessons: Surgical decompression at the point of entrapment, with or without resection of tumor, may provide symptomatic relief. Examples of surgical decompression at the point of entrapment, both with and without resection of tumor, are presented.
Language	English
Publishing date	2021-06-28
Publishing country	United States
Document type	Case Reports
ISSN	2694-1902
ISSN (online)	2694-1902
DOI	10.3171/CASE21176
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Article ; Online: Coincidental Expression of Classic Hodgkin Lymphoma and Neurofibromatosis Type I and Literature Review.

Cabrera, Trevor B / Wang, Wei / Yedururi, Sireesha / Slopis, John M / Steiner, Raphael E / Rytting, Michael E / Cuglievan, Branko

Journal of pediatric hematology/oncology

2020 Volume 43, Issue 4, Page(s) e535–e538

Abstract: Neurofibromatosis Type 1 (NF1) is a genetic disorder with an incidence of 1 in 2600 to 3000 individuals. It is a clinical diagnosis characterized by café-au-lait macules, neurofibromas, and axillary and/or groin freckling. Because of genetic mutations in ...

Abstract	Neurofibromatosis Type 1 (NF1) is a genetic disorder with an incidence of 1 in 2600 to 3000 individuals. It is a clinical diagnosis characterized by café-au-lait macules, neurofibromas, and axillary and/or groin freckling. Because of genetic mutations in the NF1 gene affecting the Ras/mitogen-activated protein kinase pathway, there is also risk of associated soft tissue sarcomas and hematologic malignancies. However, reports of classic Hodgkin lymphoma in patients with NF1 are sparse. We report an adolescent with NF1 who developed classic Hodgkin lymphoma. Although there is an unclear association between mutations in the NF1 gene and classic Hodgkin lymphoma, further studies are warranted.
MeSH term(s)	Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Cafe-au-Lait Spots/complications ; Cafe-au-Lait Spots/drug therapy ; Cafe-au-Lait Spots/genetics ; Cafe-au-Lait Spots/pathology ; Female ; Hodgkin Disease/complications ; Hodgkin Disease/drug therapy ; Hodgkin Disease/genetics ; Hodgkin Disease/pathology ; Humans ; Mutation ; Neurofibromatosis 1/complications ; Neurofibromatosis 1/drug therapy ; Neurofibromatosis 1/genetics ; Neurofibromatosis 1/pathology ; Neurofibromin 1/genetics
Chemical Substances	Neurofibromin 1
Language	English
Publishing date	2020-05-04
Publishing country	United States
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000001811
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Article: Immune Effector Cell Associated Neurotoxicity (ICANS) in Pediatric and Young Adult Patients Following Chimeric Antigen Receptor (CAR) T-Cell Therapy: Can We Optimize Early Diagnosis?

Brown, Brandon Douglas / Tambaro, Francesco Paolo / Kohorst, Mira / Chi, Linda / Mahadeo, Kris Michael / Tewari, Priti / Petropoulos, Demetrios / Slopis, John M / Sadighi, Zsila / Khazal, Sajad

Frontiers in oncology

2021 Volume 11, Page(s) 634445

Abstract: The Cornell Assessment for Pediatric Delirium (CAPD) was first proposed by the Pediatric Acute Lung Injury and Sepsis Investigators Network-Stem Cell Transplantation and Cancer Immunotherapy Subgroup and MD Anderson CARTOX joint working committees, for ... ...

Abstract	The Cornell Assessment for Pediatric Delirium (CAPD) was first proposed by the Pediatric Acute Lung Injury and Sepsis Investigators Network-Stem Cell Transplantation and Cancer Immunotherapy Subgroup and MD Anderson CARTOX joint working committees, for detection of immune effector cell associated neurotoxicity (ICANS) in pediatric patients receiving chimeric antigen receptor (CAR) T-cell therapy. It was subsequently adopted by the American Society for Transplantation and Cellular Therapy. The utility of CAPD as a screening tool for early diagnosis of ICANS has not been fully characterized. We conducted a retrospective study of pediatric and young adult patients (n=15) receiving standard-of-care CAR T-cell products. Cytokine release syndrome (CRS) and ICANS occurred in 87% and 40% of patients, respectively. ICANS was associated with significantly higher peaks of serum ferritin. A change in CAPD from a prior baseline was noted in 60% of patients with ICANS, 24-72 h prior to diagnosis of ICANS. The median change from baseline to maximum CAPD score of patients who developed ICANS versus those who did not was 13 versus 3, respectively (p=0.0004). Changes in CAPD score from baseline may be the earliest indicator of ICANS among pediatric and young adult patients which may warrant closer monitoring, with more frequent CAPD assessments.
Language	English
Publishing date	2021-03-08
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2649216-7
ISSN	2234-943X
ISSN	2234-943X
DOI	10.3389/fonc.2021.634445
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Article ; Online: Prognostic Role of Conventional and Dynamic Contrast-Enhanced MRI in Optic Pathway Gliomas.

Jittapiromsak, Nutchawan / Hou, Ping / Liu, Ho-Ling / Sun, Jia / Slopis, John M / Chi, T Linda

Journal of neuroimaging : official journal of the American Society of Neuroimaging

2017 Volume 27, Issue 6, Page(s) 594–601

Abstract: Background and purpose: The natural history of optic pathway glioma (OPG) is highly variable and unpredictable. We present a pilot study of the prognostic role of conventional and dynamic contrast-enhanced magnetic resonance imaging (DCE MRI) in the ... ...

Abstract	Background and purpose: The natural history of optic pathway glioma (OPG) is highly variable and unpredictable. We present a pilot study of the prognostic role of conventional and dynamic contrast-enhanced magnetic resonance imaging (DCE MRI) in the evaluation of OPG. Methods: We retrospectively reviewed 17 patients with 20 pretreatment OPG lesions who underwent conventional and DCE MRI between January 2010 and December 2016. Conventional MRI was evaluated for enhancement pattern, cystic component, optic nerve tortuosity, optic nerve dural ectasia, and optic nerve perineural thickening. The DCE MRI data were analyzed for quantitative parameters using the two-compartment pharmacokinetic model (K Results: Five progressive OPGs and 15 nonprogressive OPGs were included. Conventional MRI findings of diffuse enhancement and cystic component were significantly correlated with progressive OPGs (both P = .01). Conventional MRI yielded sensitivity of 60%, specificity of 100%, and accuracy of 90%. K Conclusion: DCE MRI accurately distinguished progressive and nonprogressive OPGs, with high sensitivities and specificities. DCE MRI has a significant prognostic role in predicting progressive OPGs, thus making it useful for the identification of patients who need close clinical and imaging follow-up.
MeSH term(s)	Adolescent ; Adult ; Child ; Child, Preschool ; Contrast Media ; Female ; Glioma/diagnostic imaging ; Glioma/pathology ; Humans ; Image Enhancement/methods ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Optic Nerve Neoplasms/diagnostic imaging ; Optic Nerve Neoplasms/pathology ; Pilot Projects ; Prognosis ; Retrospective Studies ; Sensitivity and Specificity ; Young Adult
Chemical Substances	Contrast Media
Language	English
Publishing date	2017-05-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	1071724-9
ISSN	1552-6569 ; 1051-2284
ISSN (online)	1552-6569
ISSN	1051-2284
DOI	10.1111/jon.12450
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Zs.A 3211: Show issues

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Article ; Online: Efficacy and Safety of Topical Rapamycin in Patients With Facial Angiofibromas Secondary to Tuberous Sclerosis Complex: The TREATMENT Randomized Clinical Trial.

Koenig, Mary Kay / Bell, Cynthia S / Hebert, Adelaide A / Roberson, Joan / Samuels, Joshua A / Slopis, John M / Tate, Patti / Northrup, Hope

JAMA dermatology

2018 Volume 154, Issue 7, Page(s) 773–780

Abstract: Importance: Facial angiofibromas occur in approximately 75% of individuals with tuberous sclerosis complex (TSC), causing substantial morbidity and disfigurement. Current therapies are partially effective, uncomfortable, produce scarring, and need ... ...

Abstract	Importance: Facial angiofibromas occur in approximately 75% of individuals with tuberous sclerosis complex (TSC), causing substantial morbidity and disfigurement. Current therapies are partially effective, uncomfortable, produce scarring, and need repeating to treat recurrence. Objective: To evaluate the efficacy and safety of topical rapamycin for TSC-related facial angiofibromas. Design, setting, and participants: This prospective, multicenter, randomized, double-blind, vehicle-controlled trial with 6 monthly clinic visits enrolled 179 patients with TSC-related facial angiofibromas not treated within 6 months from May 2012 to March 2014 in 9 clinical sites in the United States and 1 in Australia. Interventions: Patients were randomized (1:1:1) to topical formulation containing 0.3 g per 30 g (1%) rapamycin, 0.03 g per 30 g (0.1%) rapamycin, or vehicle alone. Participants applied 1.0 mL to designated areas daily at bedtime. Main outcomes and measures: Angiofibroma Grading Scale (AGS) change from baseline scored from photographs by independent masked dermatologists. Safety analyses included adverse events (AEs) and serum rapamycin levels. Results: All 179 patients randomized (99 [55.3%] female) comprised the primary analysis population (59 in the 1% rapamycin group, 63 in the 0.1% rapamycin group, and 57 in the vehicle-only group). The mean age was 20.5 years (range 3-61 years). Clinically meaningful and statistically significant improvement in facial angiofibromas was observed for both 1% and 0.1% rapamycin relative to the vehicle-only control group, and for 1% vs 0.1% rapamycin, with most of the improvement realized within the first month. At 6 months, AGS mean improvement for 1% rapamycin was 16.7 points compared with 11.0 for 0.1% rapamycin and 2.1 points for vehicle only (P < .001 for 1% and 0.1% vs vehicle only). Compared with baseline, end-of-treatment photos were rated "better" for 81.8% of patients in the 1% rapamycin group, compared with 65.5% for those in the 0.1% rapamycin group and 25.5% for those in the vehicle-only group (P < .001, all 3 pairwise comparisons). Topical rapamycin was generally well-tolerated, with no measurable systemic absorption. Apparent drug-related adverse effects were limited to 10% or less incidence of application site discomfort and/or pain, pruritus, erythema, and irritation. Nearly all AEs were mild, with no drug-related moderate, severe, or serious events. Conclusions and relevance: Topical rapamycin appears effective and safe for treatment of TSC-related facial angiofibromas. In this trial, the preferred dose was 1% once daily. Future studies are needed to evaluate prophylactic, early, and long-term use of topical rapamycin, durability of response, and combination therapy with oral mammalian target of rapamycin (mTOR) inhibitors. Trial registration: ClinicalTrials.gov Identifier: NCT01526356.
MeSH term(s)	Administration, Cutaneous ; Adolescent ; Adult ; Angiofibroma/drug therapy ; Angiofibroma/etiology ; Child ; Child, Preschool ; Double-Blind Method ; Facial Neoplasms/drug therapy ; Facial Neoplasms/etiology ; Female ; Humans ; Immunosuppressive Agents/adverse effects ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; Prospective Studies ; Quality of Life ; Severity of Illness Index ; Sirolimus/adverse effects ; Sirolimus/therapeutic use ; Skin Neoplasms/drug therapy ; Skin Neoplasms/etiology ; Time Factors ; Tuberous Sclerosis/complications ; Young Adult
Chemical Substances	Immunosuppressive Agents ; Sirolimus (W36ZG6FT64)
Language	English
Publishing date	2018-05-24
Publishing country	United States
Document type	Journal Article ; Multicenter Study ; Randomized Controlled Trial ; Research Support, U.S. Gov't, Non-P.H.S.
ZDB-ID	2701761-8
ISSN	2168-6084 ; 2168-6068
ISSN (online)	2168-6084
ISSN	2168-6068
DOI	10.1001/jamadermatol.2018.0464
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Article ; Online: Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.

Landry, Jace P / Schertz, Kelsey L / Chiang, Yi-Ju / Bhalla, Angela D / Yi, Min / Keung, Emily Z / Scally, Christopher P / Feig, Barry W / Hunt, Kelly K / Roland, Christina L / Guadagnolo, Ashleigh / Bishop, Andrew J / Lazar, Alexander J / Slopis, John M / McCutcheon, Ian E / Torres, Keila E

JAMA network open

2021 Volume 4, Issue 3, Page(s) e210945

Abstract: Importance: Neurofibromatosis type 1 (NF1) is a complex genetic disorder that is associated with not only neurofibromas, but also an increased susceptibility to other neoplasms.: Objective: To evaluate the prevalence of neoplasia and outcomes among ... ...

Abstract	Importance: Neurofibromatosis type 1 (NF1) is a complex genetic disorder that is associated with not only neurofibromas, but also an increased susceptibility to other neoplasms. Objective: To evaluate the prevalence of neoplasia and outcomes among patients with NF1. Design, setting, and participants: This cohort study was conducted among patients with NF1 at a single academic cancer center from 1985 to 2020 with median (range) follow-up of 2.9 years (36 days to 30.5 years). Of 2427 patients evaluated for NF1, 1607 patients who met the National Institutes of Health consensus criteria for NF1 were included. This group was compared with estimates from Surveillance, Epidemiology, and End Results (SEER) Cancer Statistics Review 1975 to 2015 and SEER participants database unless otherwise specified. Data were analyzed from August 2018 to March 2020. Main outcomes and measures: Disease-specific survival (DSS) was measured from diagnosis date to date of neoplasm-specific death or censorship and calculated using the Kaplan-Meier method. Survival curves were compared using the log-rank test. Deaths from disease were considered a DSS end point; other deaths were considered censored observations. Secondary outcome measures were comparisons of (1) overall survival of patients with NF1 with neurofibroma neoplasms vs those without nonneurofibroma neoplasms, (2) neoplasm prevalence in the NF1 group vs general population estimates, and (3) age at diagnosis in the NF1 group vs general population estimates for the most common neoplasms in the NF1 group. Results: Among 1607 patients with NF1, the median (range) age at initial visit was 19 years (1 month to 83 years) and 840 (52.3%) were female patients. Among 666 patients who developed other neoplasms in addition to neurofibromas (41.4%), 295 patients (18.4%) developed glioma and 243 patients (15.1%) developed malignant peripheral nerve sheath tumor (MPNST), the most common neoplasms. Patients with NF1, compared with the general population, developed several neoplasms at a younger mean (SD) age (low-grade glioma: 12.98 [11.09] years vs 37.76 [24.53] years; P < .0001; high-grade glioma [HGG]: 27.31 [15.59] years vs 58.42 [19.09] years; P < .0001; MPNST: 33.88 [14.80] years vs 47.06 [20.76] years; P < .0001; breast cancer: 46.61 [9.94] years vs 61.71 [13.85] years; P < .0001). Patients with NF1 developed neoplasms more frequently compared with the general population (odds ratio, 9.5; 95% CI, 8.5-10.5; P < .0001). Among patients with NF1, significantly lower 5-year DSS rates were found among those with undifferentiated pleomorphic sarcoma (1 of 5 patients [20.0%]), HGG (8 of 34 patients [23.1%]), MPNST (72 of 228 patients [31.6%]), ovarian carcinoma (4 of 7 patients [57.1%]), and melanoma (8 of 12 patients [66.7%]) compared with those who had neoplasms classified as other (110 of 119 patients [92.4%]) (all P < .001) . Conclusions and relevance: This cohort study found that among patients with NF1, those who developed undifferentiated pleomorphic sarcoma, HGG, MPNST, ovarian carcinoma, or melanoma had significantly lower DSS rates compared with those who developed other neoplasms. This study also found that patients with NF1 developed some neoplasms more frequently and at a younger age compared with individuals without NF1. HGGs and MPNST were noteworthy causes of death among patients NF1. This information may be useful for NF1 patient counseling and follow-up.
MeSH term(s)	Academic Medical Centers ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Cohort Studies ; Female ; Humans ; Infant ; Male ; Middle Aged ; Neoplasms/epidemiology ; Neoplasms, Multiple Primary/epidemiology ; Neurofibromatosis 1/epidemiology ; Prevalence ; Time Factors ; Young Adult
Language	English
Publishing date	2021-03-01
Publishing country	United States
Document type	Comparative Study ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ISSN	2574-3805
ISSN (online)	2574-3805
DOI	10.1001/jamanetworkopen.2021.0945
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Article ; Online: Neurofibromas of the Phrenic Nerve: A Case Report and Review of the Literature.

Ghali, Michael G Z / Srinivasan, Visish M / Jea, Andrew / Slopis, John M / McCutcheon, Ian E

World neurosurgery

2015 Volume 88, Page(s) 237–242

Abstract: Background: Phrenic neurofibromas are a rare pathologic entity, with 9 cases described in the English literature. They may occur in conjunction with or independently of neurofibromatosis type 1. Phrenic neurofibromas pose distinct therapeutic challenges ...

Abstract	Background: Phrenic neurofibromas are a rare pathologic entity, with 9 cases described in the English literature. They may occur in conjunction with or independently of neurofibromatosis type 1. Phrenic neurofibromas pose distinct therapeutic challenges compared with the more common phrenic schwannoma. We describe here a 12-year-old boy with neurofibroma of the left phrenic nerve presenting as dextroposition of the heart after paralysis of the left hemidiaphragm allowed herniation of abdominal contents into the left hemithorax and displaced the heart. Method: Surgical resection of the tumor followed by diaphragmatic plication was performed to assess its degree of malignancy, reduce abdominal herniation, and improve lung capacity. Results: The operation markedly improved his hemidiaphragmatic elevation. Conclusions: The spectrum of management options ranges from conservative surveillance to open thoracic surgery. Functional preservation of the phrenic nerve is technically challenging, and although phrenic neurofibromas often present with absent function that cannot be recovered, surgical intervention can be fruitful in restoring lung capacity through diaphragmatic reconstruction.
MeSH term(s)	Child ; Humans ; Male ; Neurofibroma/complications ; Neurofibroma/diagnosis ; Neurofibroma/surgery ; Neurosurgical Procedures/methods ; Peripheral Nervous System Neoplasms/complications ; Peripheral Nervous System Neoplasms/diagnosis ; Peripheral Nervous System Neoplasms/surgery ; Phrenic Nerve/pathology ; Phrenic Nerve/surgery ; Rare Diseases ; Respiratory Insufficiency/diagnosis ; Respiratory Insufficiency/etiology ; Respiratory Insufficiency/prevention & control ; Treatment Outcome
Language	English
Publishing date	2015-12-31
Publishing country	United States
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	2534351-8
ISSN	1878-8769 ; 1878-8750
ISSN (online)	1878-8769
ISSN	1878-8750
DOI	10.1016/j.wneu.2015.12.076
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Zs.A 1159: Show issues

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Article ; Online: Experimental models of undifferentiated pleomorphic sarcoma and malignant peripheral nerve sheath tumor.

Laboratory investigation; a journal of technical methods and pathology

2022 Volume 102, Issue 6, Page(s) 658–666

Abstract: Undifferentiated pleomorphic sarcoma (UPS) and malignant peripheral nerve sheath tumor (MPNST) are aggressive soft tissue sarcomas that do not respond well to current treatment modalities. The limited availability of UPS and MPNST cell lines makes it ... ...

Abstract	Undifferentiated pleomorphic sarcoma (UPS) and malignant peripheral nerve sheath tumor (MPNST) are aggressive soft tissue sarcomas that do not respond well to current treatment modalities. The limited availability of UPS and MPNST cell lines makes it challenging to identify potential therapeutic targets in a laboratory setting. Understanding the urgent need for improved treatments for these tumors and the limited cellular models available, we generated additional cell lines to study these rare cancers. Patient-derived tumors were used to establish 4 new UPS models, including one radiation-associated UPS-UPS271.1, UPS511, UPS0103, and RIS620, one unclassified spindle cell sarcoma-USC060.1, and 3 new models of MPNST-MPNST007, MPNST3813E, and MPNST4970. This study examined the utility of the new cell lines as sarcoma models by assessing their tumorigenic potential and mutation status for known sarcoma-related genes. All the cell lines formed colonies and migrated in vitro. The in vivo tumorigenic potential of the cell lines and corresponding xenografts was determined by subcutaneous injection or xenograft re-passaging into immunocompromised mice. USC060.1 and UPS511 cells formed tumors in mice upon subcutaneous injection. UPS0103 and RIS620 tumor implants formed tumors in vivo, as did MPNST007 and MPNST3813E tumor implants. Targeted sequencing analysis of a panel of genes frequently mutated in sarcomas identified TP53, RB1, and ATRX mutations in a subset of the cell lines. These new cellular models provide the scientific community with powerful tools for detailed studies of tumorigenesis and for investigating novel therapies for UPS and MPNST.
MeSH term(s)	Animals ; Humans ; Mice ; Models, Theoretical ; Mutation ; Neurofibrosarcoma/genetics ; Sarcoma/genetics ; Sarcoma/pathology ; Soft Tissue Neoplasms/genetics
Language	English
Publishing date	2022-02-28
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	80178-1
ISSN	1530-0307 ; 0023-6837
ISSN (online)	1530-0307
ISSN	0023-6837
DOI	10.1038/s41374-022-00734-6
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Article ; Online: Treatment of Disfiguring Cutaneous Lesions in Neurofibromatosis-1 with Everolimus: A Phase II, Open-Label, Single-Arm Trial.

Slopis, John M / Arevalo, Octavio / Bell, Cynthia S / Hebert, Adelaide A / Northrup, Hope / Riascos, Roy F / Samuels, Joshua A / Smith, Keri C / Tate, Patti / Koenig, Mary Kay

Drugs in R&D

2018 Volume 18, Issue 4, Page(s) 295–302

Abstract: Background: Cutaneous neurofibromas cause disfigurement and discomfort in individuals with neurofibromatosis type 1 (NF-1).: Methods: The primary objective of this phase II, open-label, single-arm trial was to assess whether orally administered ... ...

Abstract	Background: Cutaneous neurofibromas cause disfigurement and discomfort in individuals with neurofibromatosis type 1 (NF-1). Methods: The primary objective of this phase II, open-label, single-arm trial was to assess whether orally administered everolimus reduced the surface volume of cutaneous neurofibromas in patients with NF-1. Results: Of 22 patients who took the study drug, 17 completed the trial; 5 patients withdrew due to adverse events. Sixteen patients had photographs of sufficient quality for assessment of the primary outcome. A significant reduction in lesion surface volume, defined as an end of trial volume > 2 standard errors (SE) less than baseline volume, was observed for 4/31 lesions (13%) from 3/16 patients (19%). Additionally, a statistically significant absolute change in average height for paired lesions was observed (p = 0.048). Although not a prespecified outcome measure, a dramatic reduction in the size of 3 large plexiform neurofibromas with a cutaneous component was also noted and documented by measurement of maximum circumference or magnetic resonance imaging-based volumetric analysis. Adverse events were common in this trial, but no serious adverse events occurred. Conclusions: Although this was a small, exploratory trial that was not powered for significance, the reduction in surface volume observed in this study is noteworthy assuming that the natural course for untreated lesions is to maintain or increase in volume. Future studies are needed with larger study populations that incorporate longer durations of treatment and better standardization of volumetric measurements. Trial Registration ClinicalTrials.gov Identifier: NCT02332902.
MeSH term(s)	Administration, Oral ; Adult ; Everolimus/administration & dosage ; Everolimus/therapeutic use ; Female ; Humans ; Male ; Neurofibromatosis 1/drug therapy ; Skin Diseases/drug therapy ; Young Adult
Chemical Substances	Everolimus (9HW64Q8G6G)
Language	English
Publishing date	2018-10-03
Publishing country	New Zealand
Document type	Clinical Trial, Phase II ; Journal Article
ZDB-ID	2020476-0
ISSN	1179-6901 ; 1174-5886
ISSN (online)	1179-6901
ISSN	1174-5886
DOI	10.1007/s40268-018-0248-6
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