Desmoplastic Small Round Cell Tumor as a Rare Cause of an
Incarcerated Epigastric Linea Alba Hernia: A Case Report and
Literature Review

J 2024

Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review

PEKAŘ, Matej, Zdeněk JANDA a Kristýna FRANKOVÁ

Základní údaje

Originální název

Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review

Autoři

PEKAŘ, Matej (703 Slovensko, garant, domácí), Zdeněk JANDA (203 Česká republika) a Kristýna FRANKOVÁ (203 Česká republika)

Vydání

CUREUS, LONDON, SPRINGERNATURE, 2024, 2168-8184

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30204 Oncology

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 1.200 v roce 2022

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.7759/cureus.61729

UT WoS

001259101000034

Klíčová slova anglicky

Small Round Cell Tumor; Incarcerated Epigastric Linea Alba Hernia

Štítky

14110515, rivok

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 12. 7. 2024 12:22, Mgr. Tereza Miškechová

Anotace

V originále

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.

Návaznosti

MUNI/A/1547/2023, interní kód MU

Název: Analýza (dys)funkce: od molekul k živému organismu

Investor: Masarykova univerzita, Analýza (dys)funkce: od molekul k živému organismu

Citovat

PEKAŘ, Matej, Zdeněk JANDA a 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, roč. 16, č. 6, s. 1-7. ISSN 2168-8184. Dostupné z: https://dx.doi.org/10.7759/cureus.61729.

@article{2408137,
   author = {Pekař, Matej and Janda, Zdeněk and Franková, Kristýna},
   article_location = {LONDON},
   article_number = {6},
   doi = {http://dx.doi.org/10.7759/cureus.61729},
   keywords = {Small Round Cell Tumor; Incarcerated Epigastric Linea Alba Hernia},
   language = {eng},
   issn = {2168-8184},
   journal = {CUREUS},
   title = {Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review},
   url = {https://www.cureus.com/articles/260497-desmoplastic-small-round-cell-tumor-as-a-rare-cause-of-an-incarcerated-epigastric-linea-alba-hernia-a-case-report-and-literature-review#!/},
   volume = {16},
   year = {2024}
}

TY  - JOUR
ID  - 2408137
AU  - Pekař, Matej - Janda, Zdeněk - Franková, Kristýna
PY  - 2024
TI  - Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review
JF  - CUREUS
VL  - 16
IS  - 6
SP  - 1-7
EP  - 1-7
PB  - SPRINGERNATURE
SN  - 21688184
KW  - Small Round Cell Tumor
KW  - Incarcerated Epigastric Linea Alba Hernia
UR  - https://www.cureus.com/articles/260497-desmoplastic-small-round-cell-tumor-as-a-rare-cause-of-an-incarcerated-epigastric-linea-alba-hernia-a-case-report-and-literature-review#!/
N2  - 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.
ER  -

PEKAŘ, Matej, Zdeněk JANDA a Kristýna FRANKOVÁ. Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review. \textit{CUREUS}. LONDON: SPRINGERNATURE, 2024, roč.~16, č.~6, s.~1-7. ISSN~2168-8184. Dostupné z: https://dx.doi.org/10.7759/cureus.61729.

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