Evidence map›Paper›PMID 31078563›Full record

ReviewHuman pathology2019

EWSR1/FUS-NFATc2 rearranged round cell sarcoma: clinicopathological series of 4 cases and literature review.

Julio A Diaz-Perez, G Petur Nielsen, Cristina Antonescu, Martin S Taylor, Santiago A Lozano-Calderon, Andrew E Rosenberg

Open access · greenAbstract readCase ReportsReview
In one paragraph

Review in Human pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.

0numbers the graph read from it
0cells of the map it votes in
35citing papers in PubMed
7.3field-weighted citation impact, top 2% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

35 citing papers in PubMed, 83 citations in OpenAlex.

  1. Article
  2. Review
  3. Article
  4. [Challenges in the research and diagnosis of rare diseases].Pathologie (Heidelberg, Germany) · 2025
    Article
  5. EWSR1::BEND2 fusion sarcoma in bone: a report of two rare cases.Virchows Archiv : an international journal of pathology · 2025
    Article
  6. Article
  7. Article
  8. Article
  9. Article
  10. Clinicopathological analysis ofWorld journal of clinical cases · 2024
    Article
  11. Article
  12. Review
  13. Review
  14. Article
  15. Article
  16. Review
  17. The Recent Advances in Molecular Diagnosis of Soft Tissue Tumors.International journal of molecular sciences · 2023
    Review
  18. Aging and obesity prime the methylome and transcriptome of adipose stem cells for disease and dysfunction.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2023
    Article
  19. Article
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 4 institutions in 1 country.

Julio A Diaz-PerezDepartment of Pathology and Laboratory Medicine, Miller School of Medicine, University of Miami, Miami, FL.
G Petur NielsenDepartment of Pathology and Laboratory Medicine, Massachusetts General Hospital, Harvard University, Boston, MA.
Cristina AntonescuDepartment of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY.
Martin S TaylorDepartment of Pathology and Laboratory Medicine, Massachusetts General Hospital, Harvard University, Boston, MA.
Santiago A Lozano-CalderonDepartment of Orthopedics, Massachusetts General Hospital, Harvard University, Boston, MA.
Andrew E RosenbergDepartment of Pathology and Laboratory Medicine, Miller School of Medicine, University of Miami, Miami, FL. Electronic address: ARosenberg@med.miami.edu.
Harvard University · USUniversity of Miami · USMassachusetts General Hospital · USMemorial Sloan Kettering Cancer Center · US

Funding

X-RAY CRYSTALLOGRAPHYP30CA008748 · NCI · SLOAN-KETTERING INSTITUTE FOR CANCER RES · PI Hongbiao Carl Lekaye · 1985 to 2026
$347.4M
Targeting Oncogenic Pathways in Genetically Complex SarcomasP50CA217694 · NCI · SLOAN-KETTERING INST CAN RESEARCH · PI William D. Tap · 2018 to 2026
$21.5M
NCI NIH HHS P30 CA008748NCI NIH HHS P50 CA217694
6 · The paper itself

Abstract

The classification of bone neoplasms composed of small round cells is experiencing a transformation after the discovery of various gene fusion rearrangements that determine diagnosis, behavior, and response to therapy. We present herein 4 new cases of small round cell tumor of the bone that harbor NFATc2 rearrangements involving either EWSR1 or FUS genes. We studied the clinical presentation, pathologic features, genetics (FISH, targeted RNA sequencing) and outcome in these 4 patients. We also reviewed the literature describing similar cases. All our patients were male. The median age at diagnosis was 33.5 years. All tumors presented in long bones of the extremities as a large destructive mass with a mean size of 12.5 cm. All cases were hypercellular with prominent collagenous stroma and consisted of small to medium size round cells arranged in cords, thin trabeculae, and pseudoacinar structures. Most cases showed focal or diffuse membrane staining for CD99; whereas S100, synaptophysin and chromogranin were negative. EMA showed cytoplasmic staining in one case. Genetic studies identified EWSR1-NFATc2 fusion in 3 cases, and FUS-NFATc2 fusion in one case. Two patients were treated with neoadjuvant chemotherapy using Ewing sarcoma regimens, and surgical excision was performed on 3 patients; necrosis was minimal. Follow-up is limited; after a median follow-up of 8.7 months, one patient developed local recurrence and metastases to the lungs. Poorly differentiated round cell sarcoma with EWSR1/FUS-NFATc2 fusions are uncommon. The tumors have consistent clinical findings, morphology, and immunoprofile that in combination are distinctive and differ from that of Ewing sarcoma. Importantly, these tumors do not respond to Ewing sarcoma chemotherapy regimens.

Indexed as

Gene FusionAdultBone NeoplasmsHumansMaleMiddle AgedNFATC Transcription FactorsRNA-Binding Protein EWSRNA-Binding Protein FUSSarcomaNFATC Transcription FactorsRNA-Binding Protein EWSRNA-Binding Protein FUSBone neoplasmsEwing sarcomaFUSNFATc2-EWSR1Round cell sarcomaUndifferentiated sarcoma

Identifiers

PMID31078563
PMCPMC6714048
OpenAlexW2944609925

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.