Lopez-Nunez, Oscar and Alaggio, Rita and John, Ivy and Ciolfi, Andrea and Pedace, Lucia and Mastronuzzi, Angela and Gianno, Francesca and Giangaspero, Felice and Rossi, Sabrina and Donofrio, Vittoria and Cinalli, Giuseppe and Surrey, Lea F. and Tartaglia, Marco and Locatelli, Franco and Miele, Evelina
(2021)
Melanotic Neuroectodermal Tumor of Infancy (MNTI) and Pineal Anlage Tumor (PAT) Harbor A Medulloblastoma Signature by DNA Methylation Profiling.
Cancers, 13 (4).
p. 706.
ISSN 2072-6694
Abstract
MNTI is a rare tumor of indeterminate histogenesis and molecular signature. We performed methylation and copy number variation (CNV) profiles in patients with MNTI (n = 7) and PAT (n = 1) compared to the methylation brain tumor classifier v11b4 (BT-C) and the medulloblastoma (MB) classifier group 3/4 v1.0 (MB3/4-C). The patients’ mean age was 8 months (range: 4–48). The BT-C classified five MNTIs and one PAT (relapse) as class family MB-G3/G4, subclass group 3 (score: >0.9). The remaining two MNTIs and PAT (primary) were classified as class family plexus tumor, subclass pediatric (scores: >0.45). The MB3/4-C classified all MNTIs as high-risk MB-G3, Subtype II (score: >0.45). The primary PAT was classified as subtype III (score: 0.99) and its relapse as subtype II/III. MNTI and PAT clustered close to MB-G3. CNV analysis showed multiple rearrangements in one PAT and two MNTIs. The median follow-up was 54 months (four MNTIs in remission, one PAT died). In conclusion, we demonstrated that MNTI shares a homogenous methylation profile with MB-G3, and possibly with PAT. The role of a multipotent progenitor cell (i.e., early cranial neural crest cell) in their histogenesis and the influence of the anatomical site, tumor microenvironment, and other cytogenetic events in their divergent biologic behavior deserve further investigation.
Share
| Citation/Export: |
|
| Social Networking: |
|
Details
| Item Type: |
Article
|
| Status: |
Published |
| Creators/Authors: |
| Creators | Email | Pitt Username | ORCID  |
|---|
| Lopez-Nunez, Oscar | | | | | Alaggio, Rita | | | | | John, Ivy | ivj4@pitt.edu | ivj4 | | | Ciolfi, Andrea | | | | | Pedace, Lucia | | | | | Mastronuzzi, Angela | | | | | Gianno, Francesca | | | | | Giangaspero, Felice | | | | | Rossi, Sabrina | | | | | Donofrio, Vittoria | | | | | Cinalli, Giuseppe | | | | | Surrey, Lea F. | | | | | Tartaglia, Marco | | | | | Locatelli, Franco | | | | | Miele, Evelina | | | |
|
| Date: |
9 February 2021 |
| Date Type: |
Publication |
| Journal or Publication Title: |
Cancers |
| Volume: |
13 |
| Number: |
4 |
| Publisher: |
MDPI AG |
| Page Range: |
p. 706 |
| DOI or Unique Handle: |
10.3390/cancers13040706 |
| Schools and Programs: |
School of Medicine > Pathology |
| Refereed: |
Yes |
| Uncontrolled Keywords: |
melanotic neuroectodermal tumor, melanotic progonoma, medulloblastoma, pineal anlage tumor, DNA methylation, copy number variation |
| ISSN: |
2072-6694 |
| Official URL: |
http://dx.doi.org/10.3390/cancers13040706 |
| Funders: |
Italian Ministry of Health Ricerca Corrente, Department of Pathology Research Funds, University of Pittsburgh Medical Center, The Department of Pathology, The Children’s Hospital of Philadelphia |
| Article Type: |
Research Article |
| Date Deposited: |
08 Jun 2021 17:28 |
| Last Modified: |
08 Jun 2021 17:28 |
| URI: |
http://d-scholarship.pitt.edu/id/eprint/41262 |
Metrics
Monthly Views for the past 3 years
Plum Analytics
Altmetric.com
Actions (login required)
 |
View Item |
![[feed]](/images/feed-icon-32x32.png){kind=icon}