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.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1177/10935266241312435 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts.
Pediatr Dev Pathol
January 2025
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St. Louis, MO, USA.
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+.
View Article and Find Full Text PDFPelvic Desmoplastic Small Round Cell Tumor on 18F-PSMA PET/CT.
Clin Nucl Med
November 2024
From the Department of Nuclear Medicine, the First Medical Centre, Chinese PLA General Hospital, Beijing, China.
Desmoplastic small round cell tumor (DSRCT) as a subtype of sarcoma is rare and aggressive. We present the case of a 23-year-old man with a histologically confirmed DSRCT who underwent 18F-PSMA PET/CT before prostate biopsy and surgical resection of the tumor. It is well known that PSMA PET has a high accuracy for detecting clinically significant prostate adenocarcinoma and is useful in guiding prostate biopsy.
View Article and Find Full Text PDFPelvic Desmoplastic Small Round Cell Tumor on 18F-PSMA PET/CT.
Clin Nucl Med
February 2025
From the Department of Nuclear Medicine, the First Medical Centre, Chinese PLA General Hospital, Beijing, China.
Desmoplastic small round cell tumor (DSRCT) as a subtype of sarcoma is rare and aggressive. We present the case of a 23-year-old man with a histologically confirmed DSRCT who underwent 18F-PSMA PET/CT before prostate biopsy and surgical resection of the tumor. It is well known that PSMA PET has a high accuracy for detecting clinically significant prostate adenocarcinoma and is useful in guiding prostate biopsy.
View Article and Find Full Text PDFPediatric Soft Tissue Sarcoma in Limb-Girdle Muscular Dystrophy: Molecular Findings and Clinical Implications.
Am J Case Rep
December 2024
Department of Molecular Medicine and Surgery, Center for Molecular Medicine, Karolinska Institute, Stockholm, Sweden.
BACKGROUND Limb-girdle muscular dystrophy recessive 1 (LGMDR1) is an autosomal recessive degenerative muscle disorder characterized by progressive muscular weakness caused by pathogenic variants in the CAPN3 gene. Desmoplastic small round cell tumors (DSRCT) are ultra-rare and aggressive soft tissue sarcomas usually in the abdominal cavity, molecularly characterized by the presence of a EWSR1::WT1 fusion transcript. Mouse models of muscular dystrophy, including LGMDR1, present an increased risk of soft tissue sarcomas.
View Article and Find Full Text PDFDesmoplastic Small Round Cell Tumor: a study of CT, MRI, PET/CT multimodal imaging features and their correlations with pathology.
BMC Med Imaging
December 2024
Department of Radiology, The First Affiliated Hospital of Xiamen University, 55 Zhenhai Road, Xiamen, 361000, People's Republic of China.
Purpose: Exploring the computed tomography (CT), magnetic resonance imaging (MRI), and fluorodeoxyglucose positron emission tomography (FDG-PET)/CT Multimodal Imaging Characteristics of Desmoplastic Small Round Cell Tumor (DSRCT) to enhance the diagnostic proficiency of this condition.
Methods: A retrospective analysis was performed on clinical data and multimodal imaging manifestations (CT, MRI, FDG-PET/CT) of eight cases of DSRCT. These findings were systematically compared with pathological results to succinctly summarize imaging features and elucidate their associations with both clinical and pathological characteristics.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!