Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract.

Cancers (Basel)

The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.

Published: December 2024

AI Article Synopsis

  • Desmoplastic small round cell tumors (DSRCTs) are rare and aggressive soft tissue sarcomas primarily found in the gastrointestinal tract, believed to stem from mesenchymal stem cells.
  • Diagnosing DSRCTs is challenging due to their similarity to other tumors, requiring the use of immunohistochemical staining and molecular analysis for accurate identification.
  • Their prognosis varies based on tumor location and stage, often necessitating a multidisciplinary treatment approach that may include surgery, chemotherapy, and radiation therapy.

Article Abstract

Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region. These tumors are believed to originate from multipotent mesenchymal stem cells or primitive progenitor cells. They are composed of small round tumor cells associated with prominent stromal desmoplasia, polyphenotypic differentiation, and gene fusion. Diagnostically, DSRCTs present a significant challenge due to their histological resemblance to other small round cell tumors, such as Ewing sarcoma and rhabdomyosarcoma, necessitating the use of ancillary tests, including immunopanels and molecular analysis, to reach a definitive diagnosis. Immunohistochemical staining, including markers like cytokeratin, vimentin, desmin, and WT1, has proven valuable in differentiating DSRCTs from their mimickers. The prognosis of these tumors is highly dependent on factors such as tumor location and stage at diagnosis, and given their aggressive nature, a multidisciplinary approach may be required that combines surgical resection, chemotherapy, and radiation therapy, among other options. In this review, we provide a synopsis of the pathophysiology of DSRCTs and the latest diagnostic advancements, including the utility of molecular profiling and novel biomarkers.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11639822PMC
http://dx.doi.org/10.3390/cancers16234101DOI Listing

Publication Analysis

Top Keywords

small round
16
round cell
12
cell tumors
12
desmoplastic small
8
gastrointestinal tract
8
tumors
5
round
4
tumors gastrointestinal
4
tract desmoplastic
4
dsrcts
4

Similar Publications

Purpose: The self-shielding radiosurgery system ZAP-X consists of a 3 MV linear accelerator and eight round collimators. For this system, it is a common practice to perform the reference dosimetry using the largest 25 mm diameter collimator at a source-to-axis distance (SAD) of 45 cm with the PTW Semiflex3D chamber placed at a measurement depth of 7 mm in water. Existing dosimetry protocols do not provide correction for these measurement conditions.

View Article and Find Full Text PDF

Purpose: Henneguya sp. is a crucial myxosporean parasite known to cause milky flesh or tapioca disease in the freshwater fish population, leading to heavy mortality. Studies to investigate its host range and to monitor their prevalence in wild and aquacultured fish are necessary.

View Article and Find Full Text PDF

Pelvic 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 PDF

Pelvic 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 PDF

: An automated extrusion-based material deposition is a contemporary and rapid method for pharmaceutical dose-dispensing and preparing (printing) individualized solid dosage forms. The aim of this study was to investigate and gain knowledge of the feasibility of automated extrusion-based material deposition technology in preparing customized prednisolone (PRD)-loaded gel tablets for veterinary applications (primarily for dogs and cats). : The PRD loads of the extrusion-based deposited gel tablets were 0.

View Article and Find Full Text PDF

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!