Article ; Online: Abdominopelvic desmoplastic small round cell tumor with metastasis: A case report and literature review.
2024 Volume 103, Issue 14, Page(s) e37664
Abstract: Rationale: Desmoplastic small round cell tumor (DSRCT) is a rare and rapidly metastasizing soft tissue sarcoma, distinguished by its unique cell morphology and pleomorphic differentiation.: Patient concerns: This report describes the case of an 18- ... ...
| Abstract | Rationale: Desmoplastic small round cell tumor (DSRCT) is a rare and rapidly metastasizing soft tissue sarcoma, distinguished by its unique cell morphology and pleomorphic differentiation. Patient concerns: This report describes the case of an 18-year-old male diagnosed with abdominopelvic DSRCT exhibiting metastases to the peritoneum, liver, pleura, bone, and muscle. The patient primarily presented with symptoms of incomplete intestinal obstruction and an abdominal mass. Diagnoses: Colonoscopy revealed lumen stenosis caused by external compression mass. Contrast-enhanced computed tomography and 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed multiple lesions in the abdominopelvic cavity. A needle biopsy of an abdominal wall lesion established it as a malignant tumor, origin unknown. Immunohistochemical staining post-surgery showed positive results for Cytokeratin (CK), CK7, Desmin, Vimentin, Caudal type homeobox 2 (CDX2), and Ki-67. Fluorescence in situ hybridization analysis revealed an Ewing sarcoma breakpoint region 1/EWS RNA binding protein 1 (EWSR1) rearrangement, and next-generation sequencing identified an EWSR1-Wilms tumor protein 1 (WT1) gene fusion. Interventions: The patient underwent laparoscopic exploratory surgery, which encompassed biopsy, ascites drainage, adhesion lysis, reinforcement of weakened sections of the small intestinal walls, and repositioning of twisted intestines. Postoperatively, the treatment protocol included fasting, rehydration, gastrointestinal decompression, and parenteral nutrition. However, the patient did not received chemotherapy. Outcomes: The patient declined further treatment and deceased in early November. Lessons: This case highlights the nonspecific nature of DSRCT symptoms. In clinical practice, it is crucial to meticulously evaluate unexplained intestinal obstruction in young patients, considering DSRCT as a differential diagnosis to avoid delays in diagnosis. |
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| MeSH term(s) | Male ; Humans ; Adolescent ; Desmoplastic Small Round Cell Tumor/diagnosis ; Desmoplastic Small Round Cell Tumor/therapy ; In Situ Hybridization, Fluorescence ; Soft Tissue Neoplasms ; Intestinal Obstruction ; Oncogene Proteins, Fusion/genetics |
| Chemical Substances | Oncogene Proteins, Fusion |
| Language | English |
| Publishing date | 2024-04-05 |
| Publishing country | United States |
| Document type | Review ; Case Reports ; Journal Article |
| ZDB-ID | 80184-7 |
| ISSN | 1536-5964 ; 0025-7974 |
| ISSN (online) | 1536-5964 |
| ISSN | 0025-7974 |
| DOI | 10.1097/MD.0000000000037664 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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