Superficial
                    CIC::DUX4
                    Sarcoma With Focal
                    WT1
                    Expression: Histomorphologic Clues and Immunohistochemical Pitfalls

Janice Jiang; Brandon Say; Ryanne Brown; Kerri Rieger; Gregory W. Charville; Saisindhu Narala; Atif Saleem; Roberto Novoa

doi:10.1111/cup.70085

journal article Mar 31, 2026

Superficial CIC::DUX4 Sarcoma With Focal WT1 Expression: Histomorphologic Clues and Immunohistochemical Pitfalls

Janice Jiang

Brandon Say Ryanne Brown

Kerri Rieger Gregory W. Charville

Saisindhu Narala

Atif Saleem

Roberto Novoa

Journal of Cutaneous Pathology · Wiley

View at Publisher Save 10.1111/cup.70085

Abstract

ABSTRACT

CIC::DUX4
sarcoma (CDS) is a rare cutaneous and deep soft tissue tumor in the differential diagnosis of small round blue cell sarcomas. CDS, by definition, is negative for rearrangements in
EWSR1
, and has been reported to have strong nuclear WT1 expression and ETV4 expression in most published cases so far. We herein report a case of challenging CDS diagnosed by genomic profiling, with weak, focal cytoplasmic WT‐1 positivity, positive staining for CD56, CD138 (subset), and CD4 (subset). This case highlights the challenges involved in evaluating cutaneous small round blue cell tumors based on morphology and immunohistochemistry alone, especially in the presence of an atypical immunophenotype.

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Citations

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References

Details

Published: Mar 31, 2026
License: View

Authors

J

Janice Jiang

Department of Dermatology MetroHealth Medical Center, Case Western Reserve University Cleveland Ohio USA

B

Brandon Say

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA

R

Ryanne Brown

Department of Dermatology Stanford University Palo Alto California USA

K

Kerri Rieger

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA; Department of Dermatology Stanford University Palo Alto California USA

G

Gregory W. Charville

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA

S

Saisindhu Narala

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA; Department of Dermatology Stanford University Palo Alto California USA

A

Atif Saleem

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA

R

Roberto Novoa

Department of Anatomic and Clinical Pathology Stanford University Palo Alto California USA; Department of Dermatology Stanford University Palo Alto California USA

Cite This Article

Janice Jiang, Brandon Say, Ryanne Brown, et al. (2026). Superficial CIC::DUX4 Sarcoma With Focal WT1 Expression: Histomorphologic Clues and Immunohistochemical Pitfalls. Journal of Cutaneous Pathology. https://doi.org/10.1111/cup.70085

Superficial CIC::DUX4 Sarcoma With Focal WT1 Expression: Histomorphologic Clues and Immunohistochemical Pitfalls

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