PEKAŘ, Matej, Zdeněk JANDA and Kristýna FRANKOVÁ. Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review. CUREUS. LONDON: SPRINGERNATURE, 2024, vol. 16, No 6, p. 1-7. ISSN 2168-8184. Available from: https://dx.doi.org/10.7759/cureus.61729.
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Basic information
Original name Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review
Authors PEKAŘ, Matej (703 Slovakia, guarantor, belonging to the institution), Zdeněk JANDA (203 Czech Republic) and Kristýna FRANKOVÁ (203 Czech Republic).
Edition CUREUS, LONDON, SPRINGERNATURE, 2024, 2168-8184.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30204 Oncology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 1.200 in 2022
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.7759/cureus.61729
UT WoS 001259101000034
Keywords in English Small Round Cell Tumor; Incarcerated Epigastric Linea Alba Hernia
Tags 14110515, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 12/7/2024 12:22.
Abstract
Desmoplastic small round cell tumor is a very rare soft tissue sarcoma with a bleak prognosis and short patient survival. The most common occurrence is in 20-30-year-old men. Our study presents the case report of a 40-year-old patient who was diagnosed with this sarcoma. The first symptom of the illness was an incarcerated epigastric hernia with sarcoma metastasis resembling an intestinal loop in an ultrasound image. The fluorescence in situ hybridization (FISH) method showed a fusion of the EWS and WT1 genes. Systemic palliative chemotherapy using the VDC-IE (vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide) regimen was chosen instead of further surgery due to the disease's generalization. However, the therapy failed to halt the disease progression and was thus terminated after 18 months. The patient's overall survival was 19 months. The rare character of this disease complicates the diagnostics in clinical practice. Nevertheless, rare sarcomas should be considered in patients with non-specific abdominal symptoms, including patients with incarcerated ventral hernia.
Links
MUNI/A/1547/2023, interní kód MUName: Analýza (dys)funkce: od molekul k živému organismu
Investor: Masaryk University, Analysis of (dys)function: from molecules to the living organism
PrintDisplayed: 17/7/2024 09:30