Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts

Eric I. Nayman; Carole Brathwaite; Felipe Pedroso; Maggie E. Fader; Farres Obeidin; Louis P. Dehner

doi:10.1177/10935266241312435

Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts

Eric I. Nayman, Carole Brathwaite, Felipe Pedroso, Maggie E. Fader, Farres Obeidin, Louis P. Dehner

Division of Anatomic and Molecular Pathology (AMP)

Research output: Contribution to journal › Article › peer-review

Abstract

A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+. The tumor had the EWSR1::WT1 fusion. Numerous peritoneal nodules less than 1 cm were noted on repeat laparoscopy 3 months later. These had similar features including the ciliated epithelial cysts. Our case illustrates yet another potential variant pattern of DSRCT with Mullerian-like ciliated epithelial cysts.

Original language	English
Journal	Pediatric and Developmental Pathology
DOIs	https://doi.org/10.1177/10935266241312435
State	Accepted/In press - 2025

Keywords

EWSR1::WT1 fusion
Mullerian cysts
WT1
desmoplastic small round cell tumor
sex-cord stromal tumor
small round blue cell tumor

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10.1177/10935266241312435

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title = "Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts",

abstract = "A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+. The tumor had the EWSR1::WT1 fusion. Numerous peritoneal nodules less than 1 cm were noted on repeat laparoscopy 3 months later. These had similar features including the ciliated epithelial cysts. Our case illustrates yet another potential variant pattern of DSRCT with Mullerian-like ciliated epithelial cysts.",

keywords = "EWSR1::WT1 fusion, Mullerian cysts, WT1, desmoplastic small round cell tumor, sex-cord stromal tumor, small round blue cell tumor",

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language = "English",

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T1 - Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts

AU - Nayman, Eric I.

AU - Brathwaite, Carole

AU - Pedroso, Felipe

AU - Fader, Maggie E.

AU - Obeidin, Farres

AU - Dehner, Louis P.

PY - 2025

Y1 - 2025

N2 - A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+. The tumor had the EWSR1::WT1 fusion. Numerous peritoneal nodules less than 1 cm were noted on repeat laparoscopy 3 months later. These had similar features including the ciliated epithelial cysts. Our case illustrates yet another potential variant pattern of DSRCT with Mullerian-like ciliated epithelial cysts.

AB - A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+. The tumor had the EWSR1::WT1 fusion. Numerous peritoneal nodules less than 1 cm were noted on repeat laparoscopy 3 months later. These had similar features including the ciliated epithelial cysts. Our case illustrates yet another potential variant pattern of DSRCT with Mullerian-like ciliated epithelial cysts.

KW - EWSR1::WT1 fusion

KW - Mullerian cysts

KW - WT1

KW - desmoplastic small round cell tumor

KW - sex-cord stromal tumor

KW - small round blue cell tumor

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SN - 1093-5266

JO - Pediatric and Developmental Pathology

JF - Pediatric and Developmental Pathology

ER -

Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts

Abstract

Keywords

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