A desmoplastic small round cell tumor(DSRCT)is a very rare malignant tumor that mainly occurs in the intra-abdominal cavity in young adults.This neoplasm has an extremely poor prognosis, with a clinical course characterized by rapid progression and metastasis.We present a 31-year-old man who presented with chief complaints of dysphagia, ataxic gait, and hoarseness.He first underwent surgical resection of a tumor in the medulla oblongata; however, the lesion was suspected to be a metastatic neoplasm.Following a thorough medical examination, the patient was diagnosed with retroperitoneal DSRCT with multiple metastatic lesions.He received multidisciplinary treatment including debulking surgery for the primary lesion; radiotherapy for metastatic lesions in the brain, abdomen, and cervical lymph nodes; hepatic artery embolization for liver metastasis; and systemic chemotherapy.The patient died of progressive disease 17 months after the initial diagnosis.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Pediatric 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.
Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract.
Cancers (Basel)
December 2024
The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
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.
View Article and Find Full Text PDFReview of Imaging Peritoneal Disease and Treatment.
Can Assoc Radiol J
December 2024
Abdominal Division, Joint Department of Medical Imaging, Toronto, ON, Canada.
Article Synopsis
- Peritoneal disease is divided into benign and malignant categories, with malignant cases further classified into primary and secondary types, including conditions like peritoneal mesothelioma and peritoneal carcinomatosis.
- Differentiating between benign and malignant peritoneal conditions is crucial, as this determines the course of treatment and helps avoid unnecessary procedures.
- For patients with secondary malignant diseases like peritoneal carcinomatosis, cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) may offer a chance for cure, and radiologists play a key role in assessing disease extent and potential surgical issues.
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!