Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors

First, the investigators plan to use a retrospective analysis to determine the clinical landscape of neurofibromatosis (NF)1-associated malignant peripheral nerve sheath tumor (MPNST) and precursor lesions (e.g., atypical or nodular plexiform neurofibromas). A worldwide database will be established, collecting, in a standardized manner, histologic, immunohistochemical, molecular, radiographic, treatment, and related clinical data from centers worldwide with expertise in these NF1-related cancers. Although retrospective in nature, the resulting data from this registry may reveal previously unanticipated patterns, similar to the INFACT effort outcome. This registry would then allow the acquisition of data associated with MPNST biospecimens collected under associated banks (frozen or paraffin-embedded, germline (or normal tissue DNA) samples, and any previously somatic whole-exome or whole-genome sequencing data for aggregate analyses). Second, the investigators plan to co-register patients to institutional banks in order to prospectively collect MPNST samples for analysis. These patients will be consented in order to collect the above information and for banking of tumor tissue and future studies that include genomic characterization of the tumors.

Study Overview

• Status: Recruiting
• Conditions: Malignant Peripheral Nerve Sheath Tumors

• Study Type: Observational
• Enrollment (Estimated): 1050

Contacts and Locations

• Study Contact: Name: Angela Hirbe, M.D., Ph.D.; Phone Number: (314) 362-8965; Email: hirbea@wustl.edu

Study Locations

• Canada
  • Ontario
    • Toronto, Ontario, Canada, M5T 3L9
      • Recruiting
      • Mount Sinai
      • Contact: Jay Wunder, M.D.; Phone Number: 416-586-5995; Email: jay.wunder@sinaihealthsystem.ca
      • Principal Investigator: Jay Wunder, M.D.
• India
  • Mumbai, India
    • Completed
    • Global Gene Corporation
• Japan
  • Showa Ward
    • Nagoya, Showa Ward, Japan
      • Recruiting
      • Nagoya University Hospital
      • Contact: Yoshihiro Nishida, M.D., Ph.D.; Phone Number: +81 52-741-2111; Email: ynishida@med.nagoya-u.ac.jp
      • Principal Investigator: Yoshihiro Nishida, M.D., Ph.D.
• United Kingdom
  • London, United Kingdom
    • Terminated
    • Royal National Orthopedic Hospital
• United States
  • Florida
    • Tampa, Florida, United States, 33612
      • Completed
      • Moffitt Cancer Center
  • Maryland
    • Baltimore, Maryland, United States, 21205
      • Recruiting
      • Johns Hopkins
      • Contact: Christine Pratilas, M.D.; Phone Number: 410-955-8751
      • Principal Investigator: Christine Pratilas, M.D.
    • Bethesda, Maryland, United States, 20892
      • Not yet recruiting
      • National Institutes of Health (NIH)
      • Contact: Jack F Shern, M.D.; Phone Number: 301-402-1444; Email: john.shern@nih.gov
      • Principal Investigator: Jack F Shern, M.D.
  • Massachusetts
    • Boston, Massachusetts, United States, 02215
      • Withdrawn
      • Dana Farber Cancer Institute
  • Minnesota
    • Minneapolis, Minnesota, United States, 55455
      • Withdrawn
      • University of Minnesota
  • Missouri
    • St Louis, Missouri, United States, 63110
      • Recruiting
      • Washington University School of Medicine
      • Contact: Angela Hirbe, M.D., Ph.D.; Phone Number: (314) 362-8965; Email: hirbea@wustl.edu
      • Principal Investigator: Angela Hirbe, M.D., Ph.D.
      • Sub-Investigator: David Gutmann, M.D., Ph.D.
      • Sub-Investigator: Sonika Dahiya, M.D., MBBS
      • Sub-Investigator: Peter Oppelt, M.D.
      • Sub-Investigator: Brian Van Tine, M.D., Ph.D.
  • New York
    • New York, New York, United States, 10065
      • Not yet recruiting
      • Memorial Sloan Kettering Cancer Center
      • Contact: Ping Chi, M.D., Ph.D.; Phone Number: 646-888-4166
      • Principal Investigator: Ping Chi, M.D., Ph.D.
  • Rhode Island
    • Providence, Rhode Island, United States, 02903
      • Completed
      • Lifespan
  • Utah
    • Salt Lake City, Utah, United States, 84112
      • Completed
      • University of Utah

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

The oncology clinic of each participating institution

Description

Inclusion Criteria and Exclusion Criteria:

-Any patient diagnosed with an MPNST is eligible for enrollment to this registry provided s/he consents to participate (or consent form his/her parent or legal guardian or legally authorized representative is obtained).

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Other

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Histologic characteristics of malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years
Immunohistochemical characteristics of malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years
Molecular characteristics of malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years
Radiographic characteristics of malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years
Treatment given to patients with malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years
Future genomic characterization of malignant peripheral nerve sheath tumor (MPNST)	-The data collected through this registry will be used to analyze previously unanticipated patterns and prognostic clues about MPNST	10 years

Collaborators and Investigators

• Sponsor: Washington University School of Medicine
• Collaborators: Boston Children's Hospital
• Investigators: Principal Investigator: Angela Hirbe, M.D., Ph.D., Washington University School of Medicine

Publications and helpful links

Helpful Links

• Alvin J. Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine

Study record dates

Study Major Dates

• Study Start (Actual): April 28, 2017
• Primary Completion (Estimated): April 30, 2053
• Study Completion (Estimated): April 30, 2053

Study Registration Dates

• First Submitted: April 13, 2017
• First Submitted That Met QC Criteria: May 3, 2017
• First Posted (Actual): May 4, 2017

Study Record Updates

• Last Update Posted (Actual): March 9, 2026
• Last Update Submitted That Met QC Criteria: March 5, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Neoplasms; Neuromuscular Diseases; Peripheral Nervous System Diseases; Neoplasms by Histologic Type; Neoplasms, Nerve Tissue; Nervous System Neoplasms; Nerve Sheath Neoplasms; Peripheral Nervous System Neoplasms; Sarcoma; Neoplasms, Connective and Soft Tissue; Neoplasms, Connective Tissue; Neurofibroma; Fibrosarcoma; Neoplasms, Fibrous Tissue; Neurofibrosarcoma
• Other Study ID Numbers: 201702006

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No