Epithelioid Enigma: Proximal‐Type Epithelioid Sarcoma in Cyst Fluid Cytology

Epithelioid sarcoma (ES) is a rare, aggressive soft tissue malignancy with substantial morphologic overlap with other epithelioid neoplasms, creating diagnostic difficulty in cytology specimens. We report proximal-type ES identified in cyst fluid from a lesion clinically interpreted as an epidermal inclusion cyst or abscess. A 43-year-old man presented with right thigh pain and a small cystic lesion on ultrasound. The fluid specimen was hypocellular and contained malignant epithelioid to rhabdoid cells with marked atypia, prominent nucleoli, and focal perinuclear clearing. Tumor cells expressed keratins and showed complete loss of INI1/SMARCB1, while claudin-4, MOC31, desmin, and SOX10 were negative. A concurrent core biopsy confirmed proximal-type ES. This case expands the recognized cytologic spectrum of proximal-type ES and highlights that a hypocellular cyst fluid specimen may nevertheless provide diagnostically useful material when morphology is integrated with a focused immunohistochemical panel.

Authors

Institutions

Publication Details

Journal
Diagnostic Cytopathology
Published
2026-09-09
DOI
https://doi.org/10.1002/dc.70210
Primary Topic
Chromatin Remodeling and Cancer
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Epithelioid Enigma: Proximal‐Type Epithelioid Sarcoma in Cyst Fluid Cytology

Samer Khader, N G McGrath, Azfar Neyaz
Diagnostic Cytopathology
Chromatin Remodeling and Cancer
article

Epithelioid Enigma: Proximal‐Type Epithelioid Sarcoma in Cyst Fluid Cytology

Samer Khader, N G McGrath, Azfar Neyaz
article en

Abstract

Epithelioid sarcoma (ES) is a rare, aggressive soft tissue malignancy with substantial morphologic overlap with other epithelioid neoplasms, creating diagnostic difficulty in cytology specimens. We report proximal-type ES identified in cyst fluid from a lesion clinically interpreted as an epidermal inclusion cyst or abscess. A 43-year-old man presented with right thigh pain and a small cystic lesion on ultrasound. The fluid specimen was hypocellular and contained malignant epithelioid to rhabdoid cells with marked atypia, prominent nucleoli, and focal perinuclear clearing. Tumor cells expressed keratins and showed complete loss of INI1/SMARCB1, while claudin-4, MOC31, desmin, and SOX10 were negative. A concurrent core biopsy confirmed proximal-type ES. This case expands the recognized cytologic spectrum of proximal-type ES and highlights that a hypocellular cyst fluid specimen may nevertheless provide diagnostically useful material when morphology is integrated with a focused immunohistochemical panel.

Diagnostic Cytopathology
University of Pittsburgh Medical Center (US)
Good health and well-being
Openalex Percentile: Top 18%
Chromatin Remodeling and Cancer
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.