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Pediatr Dev Pathol. 2018 Sep-Oct;21(5):461-466. doi: 10.1177/1093526617749670. Epub 2018 Feb 9.

Rare MYC-amplified Neuroblastoma With Large Cell Histology.

Author information

1
1 Department of Pathology & Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, California.
2
2 Department of Anatomic Pathology, Prince of Wales Hospital, Randwick, Australia.
3
3 School of Women's and Children's Health, University of New South Wales, Sydney, Australia.
4
4 Department of Pathology, Kaiser Permanente Medical Center, San Francisco, California.
5
5 Cytogenetics Laboratory, SydPath, St Vincent's Hospital, Darlinghurst, Australia.
6
6 Kids Cancer Center, Sydney Children's Hospital, Randwick, Australia.
7
7 Saiseikai Shiga Hospital, Ritto, Shiga, Japan.
8
8 Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, California.
9
9 Division of Oncology and Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
10
10 Department of Anatomy and Cell Biology, University of Illinois at Chicago, Chicago, Illinois.

Abstract

Background Although MYCN (aka N-myc) amplification is reported in ∼20% of neuroblastomas, MYC (aka C-myc) amplification appears to be a rare event in this disease. As of today, only 2 MYC-amplified neuroblastomas have been briefly mentioned in the literature. Methods We studied here the clinicopathological features of 3 MYC-amplified neuroblastomas. Results All 3 patients (2 females and 1 male) had stage 4 disease. One female is currently alive and well 52 months after the diagnosis, while the other female and male patients died of disease 24 and 20 months after the diagnosis, respectively. Further analysis on 2 tumors revealed unfavorable histology with MYC protein overexpression but with neither MYCN amplification nor MYCN protein overexpression. Both of these tumors exhibited "large cell neuroblastoma" histology with enlarged, uniquely open nuclei and nucleolar hypertrophy, along with "aberrant" desmin expression. Conclusions MYC-amplified neuroblastomas are extremely rare and seem to present with distinct clinicopathological features.

KEYWORDS:

MYC amplification; MYC protein overexpression; large cell; neuroblastoma; no MYCN amplification; unfavorable histology

PMID:
29426276
DOI:
10.1177/1093526617749670
[Indexed for MEDLINE]

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