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 and Kristýna FRANKOVÁ

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30204 Oncology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

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.

Links

MUNI/A/1547/2023, interní kód MU

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

Investor: Masaryk University, Analysis of (dys)function: from molecules to the living organism

Citovat

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.

@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 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. \textit{CUREUS}. LONDON: SPRINGERNATURE, 2024, vol.~16, No~6, p.~1-7. ISSN~2168-8184. Available from: https://dx.doi.org/10.7759/cureus.61729.

Detailed Information on Publication Record