Genomic Patterns of Malignant Peripheral Nerve Sheath Tumor (MPNST) Evolution Correlate with Clinical Outcome and Are Detectable in Cell-Free DNA
Cortes-Ciriano I, Steele CD, Piculell K, Al-Ibraheemi A, Eulo V, Bui MM, Chatzipli A, Dickson BC, Borcherding DC, Feber A, Galor A, Hart J, Jones KB, Jordan JT, Kim RH, Lindsay D, Miller C, Nishida Y, Proszek PZ, Serrano J, Sundby RT, Szymanski JJ, Ullrich NJ, Viskochil D, Wang X, Snuderl M, Park PJ, Flanagan AM, Hirbe AC, Pillay N, Miller DT

Mutations involving TGFB and MAPK may be associated with malignancy in granular cell tumors
Dehner CA, Moon T, Lyu Y, Zhang X, Zhou Z, Yang K, Chrisinger JSA, Griffin A, Wunder J, Dickson BC, Hirbe AC

Ex vivo to in vivo model of malignant peripheral nerve sheath tumors for precision oncology
Larsson AT, Bhatia H, Calizo A, Pollard K, Zhang X, Conniff E, Tibbitts JF, Rono E, Cummins K, Osum SH, Williams KB, Crampton AL, Jubenville T, Schefer D, Yang K, Lyu Y, Pino JC, Bade J, Gross JM, Lisok A, Dehner CA, Chrisinger JSA, He K, Gosline SJC, Pratilas CA, Largaespada DA, Wood DK, Hirbe AC
Neuro Oncol. 2023 May 29:noad097. doi: 10.1093/neuonc/noad097. Epub ahead of print. PMID: 37246765.

Loss of Chromosome 3q Is a Prognostic Marker in Fusion-Negative Rhabdomyosarcoma
Cortes-Ciriano I, Steele CD, Piculell K, Al-Ibraheemi A, Eulo V, Bui MM, Chatzipli A, Dehner CA, Bell RC, Cao Y, He K, Chrisinger JSA, Armstrong AE, Yohe M, Shern J, Hirbe AC


The Impact of TSC-1 and -2 Mutations on Response to Therapy in Malignant PEComa: A Multicenter Retrospective Analysis
Liu L, Dehner CA, Grandhi N, Lyu Y, Borcherding DC, Chrisinger JSA, Zhang X, Luo J, Tao Y, Parkes A, Bui NQ, Davis EJ, Milhem MM, Monga V, Weiss M, Van Tine BA, Hirbe AC

Molecular Characterization of Multifocal Granular Cell Tumors
Dehner CA, Schroeder MC, Lyu Y, Bell R, Borcherding DC, Moon T, Hirbe AC, Chrisinger JSA

A systematic review of recent and ongoing clinical trials in patients with the neurofibromatoses
Acar S, Nieblas-Bedolla E, Armstrong AE, Hirbe AC


Plexiform neurofibroma: shedding light on the investigational agents in clinical trials
Acar S, Armstrong AE, Hirbe AC

Cell-free DNA ultra-low-pass whole genome sequencing to distinguish malignant peripheral nerve sheath tumor (MPNST) from its benign precursor lesion: A cross-sectional study
Szymanski JJ, Sundby RT, Jones PA, Srihari D, Earland N, Harris PK, Feng W, Qaium F, Lei H, Roberts D, Landeau M, Bell J, Huang Y, Hoffman L, Spencer M, Spraker MB, Ding L, Widemann BC, Shern JF, Hirbe AC, Chaudhuri AA

TYK2 in Cancer Metastases: Genomic and Proteomic Discovery
Borcherding DC, He K, Amin NV, Hirbe AC

Chromosome 8 gain is associated with high-grade transformation in MPNST
Dehner CA, Moon CI, Zhang X, Zhou Z, Miller CA, Xu H, Wan X, Yang K, Mashl RJ, Gosline SJ, Wang Y, Zhang X, Godec A, Jones PA, Dahiya S, Bhatia H, Primeau T, Li S, Pollard K, Rodriguez FJ, Ding L, Pratilas CA, Shern JF, Hirbe AC


A Retrospective Analysis of the Efficacy of Immunotherapy in Metastatic Soft-Tissue Sarcomas
Monga V, Skubitz KM, Maliske S, Mott SL, Dietz H, Hirbe AC, Van Tine BA, Oppelt P, Okuno S, Robinson S, O’Connor M, Seetharam M, Attia S, Charlson J, Agulnik M, Milhem M

A phase II study of pazopanib as front-line therapy in patients with non-resectable or metastatic soft-tissue sarcomas who are not candidates for chemotherapy
Hirbe AC, Eulo V, Moon CI, Luo J, Myles S, Seetharam M, Toeniskoetter J, Kershner T, Haarberg S, Agulnik M, Monga V, Milhem M, Parkes A, Robinson S, Okuno S, Attia S, Van Tine BA

Unmasking Intra-tumoral Heterogeneity and Clonal Evolution in NF1-MPNST
Moon CI, Tompkins W, Wang Y, Godec A, Zhang X, Pipkorn P, Miller CA, Dehner C, Dahiya S, Hirbe AC
Genes May 2020. https://doi.org/10.3390/genes11050499

Genomics of MPNST (GeM) Consortium: Rationale and Study Design for Multi-Omic Characterization of NF1-Associated and Sporadic MPNSTs
Miller DT, Cortés-Ciriano I, Pillay N, Hirbe AC, Snuderl M, Bui MM, Piculell K, Al-Ibraheemi A, Dickson BC, Hart J, Jones K, Jordan JT, Kim RH, Lindsay D, Nishida Y, Ullrich NJ, Wang X, Park PJ, Flanagan AM.
Genes Apr 2020. https://doi.org/10.3390/genes11040387

Secondary Sarcomas: Biology, Presentation, and Clinical Care
Eulo V, Lesmana H, Doyle LA, Nichols KE, Hirbe AC.
Am Soc Clin Oncol Educ Book Mar 2020. https://doi.org/10.1200/EDBK_280985

Integrative Analysis Identifies Candidate Tumor Microenvironment and Intracellular Signaling Pathways that Define Tumor Heterogeneity in NF1
Banerjee J, Allaway RJ, Taroni JN, Baker A, Zhang X, Moon CI, Pratilas CA, Blakeley JO, Guinney J, Hirbe AC, Greene CS, Gosline SJ.
Genes Feb 2020. https://doi.org/10.3390/genes11020226

Vascularized Ulnar Transposition and Radioulnoscahpolunate Fusion With Volar Locking Plate in a Dorsal Position Following Resection of Giant Cell Tumor of the Distal Radius. 
Van Handel AC, Galvez MG, Brogan DM, Boyer MI, Cipriano CA, Hirbe AC.
Pet MA. Tech Hand Up Extrem Surg Feb 2020. https://doi.org/10.1097/BTH.0000000000000282


Diagnosis and management of malignant peripheral nerve sheath tumors: Current practice and future perspectives
Prudner BC, Ball T, Rathore R, Hirbe AC
Neuro-Oncology Advances Nov 2019. https://doi.org/10.1093/noajnl/vdz047

Whole exome sequencing reveals the maintained polyclonal nature from primary to metastatic malignant peripheral nerve sheath tumor in two patients with NF1
Godec A, Jayasinghe R, Chrisinger JSA, Prudner BC, Ball T, Wang Y, Srihari D, Kaushal M, Dietz H, Zhang X, Pekmezci M, Dahiya S, Tao Y, Luo J, Van Tine BA, Ding L, Gutmann DH, Hirbe AC
Neuro-Oncology Advances Sep 2019. https://doi.org/10.1093/noajnl/vdz047

A Phase II Study of Pazopanib as Front-Line Therapy in Patients with Nonresectable or Metastatic Soft Tissue Sarcomas Who are Not Candidates for Chemotherapy
Hirbe AC, Eulo V, Moon CI, Luo J, Seetharam M, Toeniskoetter J, Kershner T, Agulnik M, Monga V, Milhem M, Parkes AM, Robinson S, Okuno S, Attia S, VanTine BA
EMJ Oncology. 2019;7[1]:41-43. Abstract Review No: AR02.

Targeting Sporadic and Neurofibromatosis Type 1 (NF1) Related Refractory Malignant Peripheral Nerve Sheath Tumors (MPNST) in a Phase II Study of Everolimus in Combination with Bevacizumab (SARC016)
Widemann BC, Lu Y, Reinke D, Okuno SH, Meyer CF, Hirbe AC, Kim A, Turpin B, Pressey JG, Dombi E, Jayaprakash N, Helman LJ, Onwudiwe N, Cichowski K, Perentesis JP.
Sarcoma Jul 2019. doi: 10.1155/2019/7656747

TYK2 promotes malignant peripheral nerve sheath tumor progression through inhibition of cell death.
Qin W, Godec A, Zhang X, Zhu C, Shao J, Tao Y, Bu X, Hirbe AC.
Cancer Med Jul 2019. doi: 10.1002/cam4.2386.

Arginine Starvation and Docetaxel Induce c-Myc-Driven hENT1 Surface Expression to Overcome Gemcitabine Resistance in ASS1-Negative Tumors.
Prudner BC, Rathore R, Robinson AM, Godec AJ, Chang SF, Hawkins WG, Hirbe AC, Van Tine BA.
Clinical Cancer Research May 2019; 10.1158/1078-0432.CCR-19-0206


A Phase II Study of Tumor Ablation in Patients With Metastatic Sarcoma Stable on Chemotherapy
Hirbe AC, Jennings J, Saad N, Giardina JD, Tao Y, Luo J, Berry S, Toeniskoetter J, Van Tine BA
Oncologist 2018 Jul;23(7):760-e76

Aberrant ATRX Protein Expression Is Associated With Poor Overall Survival in NF1-MPNST
Lu HC, Eulo V, Apicelli AJ, Pekmezci M, Tao Y, Luo J, Hirbe AC, Dahiya S
Oncotarget 2018 May 1;9(33):23018-23028

Beta-III-Spectrin Immunohistochemistry as a Potential Diagnostic Tool With High Sensitivity for Malignant Peripheral Nerve Sheath Tumors
Hirbe AC, Zhang X, Dahiya S, Godec A, Chrisinger J, Tao Y, Luo J, Gutmann DH
Neuro Oncol 2018 May 18;20(6):858-860


Gastrointestinal Stromal Tumors (GISTs): Point Mutations Matter in Management, a Review
Oppelt PJ, Hirbe AC, Van Tine BA
J Gastrointest Oncol 2017 Jun;8(3):466-473

Neoadjuvant Ifosfamide and Epirubicin in the Treatment of Malignant Peripheral Nerve Sheath Tumors
Hirbe AC, Cosper PF, Dahiya S, Van Tine BA
Sarcoma 2017;2017:3761292

Clinical Genomic Profiling Identifies TYK2 Mutation and Overexpression in Patients With Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors
Hirbe AC, Kaushal M, Sharma MK, Dahiya S, Pekmezci M, Perry A, Gutmann DH
Cancer 2017 Apr 1;123(7):1194-1201


Spatially- And Temporally-Controlled Postnatal p53 Knockdown Cooperates With Embryonic Schwann Cell Precursor Nf1 Gene Loss to Promote Malignant Peripheral Nerve Sheath Tumor Formation
Hirbe AC, Dahiya S, Friedmann-Morvinski D, Verma IM, Clapp DW, Gutmann DH
Oncotarget 2016 Feb 16;7(7):7403-14


Whole Exome Sequencing Reveals the Order of Genetic Changes During Malignant Transformation and Metastasis in a Single Patient With NF1-Plexiform Neurofibroma
Hirbe AC, Dahiya S, Miller CA, Li T, Fulton RS, Zhang X, McDonald S, DeSchryver K, Duncavage EJ, Walrath J, Reilly KM, Abel HJ, Pekmezci M, Perry A, Ley TJ, Gutmann DH
Clin Cancer Res 2015 Sep 15;21(18):4201-11

Morphologic and Immunohistochemical Features of Malignant Peripheral Nerve Sheath Tumors and Cellular Schwannomas
Pekmezci M, Reuss DE, Hirbe AC, Dahiya S, Gutmann DH, von Deimling A, Horvai AE, Perry A
Mod Pathol 2015 Feb;28(2):187-200

Special Editorial Issue: Genes

Dear Colleagues,

In this Special Issue of the open access journal Genes, we welcome both review articles and original research related to the genomic understanding and modeling of schwannomas, plexiform and diffuse neurofibromas, atypical neurofibromas, and malignant peripheral nerve sheath tumors. This Special Issue, entitled “Genomics and Models of Nerve Sheath Tumors”, will also focus on genomic methods being developed and applied to advance our understanding of these tumors. We aim for the edition to emphasize the ability of genomic knowledge to translate to advances in management of patients affected by these tumors. We look forward to your contribution.

Prof. Angela C. Hirbe
Prof. Christine A. Pratilas
Prof. Rebecca D. Dodd

To learn more about the edition or to submit a manuscript for consideration, please click the link below.