Clinical, Radiological, Histologic and Molecular Features of a Cohort of Melanocytic Tumors of the Central Nervous System

NCT05984108 | Unknown DMC

• 1 other identifier: 2022PI132
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

Primary melanocytic tumors of the central nervous system are rare lesions and occur develop from leptomeningeal melanocytes. The WHO classification of tumors of the central nervous system in its most recent version (2021) distinguishes on the one hand the circumscribed melanocytic tumors including melanocytoma, benign, and its slope malignant, meningeal melanoma, with an intermediate grade lesion in between, called intermediate grade melanocytoma. They are to be distinguished from diffuse tumors or multifocal diseases such as melanocytosis and its malignant corollary, melanomatosis. The main current challenge is to distinguish them from their differential diagnoses, namely metastasis of a cutaneous or extrac-cutaneous melanoma mainly and on the other hand other pigmented entities occuring in the CNS such as malignant melanic tumor of the peripheral nerve sheath (MMNST, formerly "melanotic schwannoma").

Conditions

Meningeal Melanocytoma; Meningeal Melanomatosis; Meningeal Melanocytosis; Meningeal Melanoma

Outcome Measures

Primary Outcomes (7)

• Diagnosis according to WHO classification of CNS tumors (5th edition)

  Histologic diagnosis according to WHO classification of CNS tumors (5th edition) among these 5 options : melanocytoma, intermediate-grade melanocytoma, melanoma, melanocytosis, melanomatosis

  at baseline (initial report)

• Mitotic index

  Number of mitoses per square millimetre

  at baseline (initial report)

• Age

  Age in years

  At baseline

• Sex

  Male or female

  At baseline

• Ki-67

  Proliferation index Ki-67, assessed by immunohistochemistry, counted on 500 cells

  At time of initial diagnosis

• T1-weighted sequence signal

  Radiologic feature : tumor T1-weighted sequence signal on MRI (hyposignal, isosignal, hypersignal)

  At time of initial diagnosis

• Enhancement after gadolinium injection

  Radiologic feature: tumor enhancement after gadolinium injection on MRI

  At time of initial diagnosis

Secondary Outcomes (2)

• Overall survival

  From date of initial diagnosis until the date of death of any cause assessed up to 10 years

• Progression-free survival

  From date of initial diagnosis until the date of first documented progression assessed up to 10 years

Study Arms (4)

Meningeal melanocytoma

Meningeal melanoma

Meningeal melanomatosis

Meningeal melanocytosis

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with primitive meningeal melanocytic tumors

You may qualify if:

• Patient with a primitive meningeal melanocytic tumor

You may not qualify if:

• Patient with metastatic extrameningeal melanoma

Sponsors & Collaborators

• Central Hospital, Nancy, France: lead

Study Sites (1)

Nancy University Hospital Center

Nancy, 54500, France

RECRUITING

Related Publications (3)

• Koelsche C, Hovestadt V, Jones DT, Capper D, Sturm D, Sahm F, Schrimpf D, Adeberg S, Bohmer K, Hagenlocher C, Mechtersheimer G, Kohlhof P, Muhleisen H, Beschorner R, Hartmann C, Braczynski AK, Mittelbronn M, Buslei R, Becker A, Grote A, Urbach H, Staszewski O, Prinz M, Hewer E, Pfister SM, von Deimling A, Reuss DE. Melanotic tumors of the nervous system are characterized by distinct mutational, chromosomal and epigenomic profiles. Brain Pathol. 2015 Mar;25(2):202-8. doi: 10.1111/bpa.12228. Epub 2014 Dec 15.

  PMID: 25399693 BACKGROUND

• Kusters-Vandevelde HV, Kusters B, van Engen-van Grunsven AC, Groenen PJ, Wesseling P, Blokx WA. Primary melanocytic tumors of the central nervous system: a review with focus on molecular aspects. Brain Pathol. 2015 Mar;25(2):209-26. doi: 10.1111/bpa.12241.

  PMID: 25534128 BACKGROUND

• Griewank KG, Koelsche C, van de Nes JAP, Schrimpf D, Gessi M, Moller I, Sucker A, Scolyer RA, Buckland ME, Murali R, Pietsch T, von Deimling A, Schadendorf D. Integrated Genomic Classification of Melanocytic Tumors of the Central Nervous System Using Mutation Analysis, Copy Number Alterations, and DNA Methylation Profiling. Clin Cancer Res. 2018 Sep 15;24(18):4494-4504. doi: 10.1158/1078-0432.CCR-18-0763. Epub 2018 Jun 11.

  PMID: 29891723 BACKGROUND

Biospecimen

Retention: SAMPLES WITH DNA

Formalin-fixed Paraffin-embedded tumor samples

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: PhD

Study Record Dates

• First Submitted: June 25, 2023
• First Posted: August 9, 2023
• Study Start: January 1, 2023
• Primary Completion: June 1, 2024
• Study Completion: June 1, 2024
• Last Updated: August 9, 2023

Record last verified: 2023-07

Locations

FR (1)