Uskřinutá metastáza jako první manifestace vzácného sarkomu

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Title in English Incarcerated metastasis as the first manifestation of a rare sarcoma
Authors

PEKAŘ Matej JANDA Zdeněk FRANKOVÁ Kristýna

Year of publication 2023
Type Article in Periodical
Magazine / Source Rozhledy v Chirurgii
MU Faculty or unit

Faculty of Medicine

Citation
web https://perspinsurg.com/rvch/article/view/821
Doi http://dx.doi.org/10.33699/pis.2023.102.1.28-31
Keywords Hernia; metastasis; sarcoma; desmoplastic small round cell tumor
Description Desmoplastic small round cell tumor belongs to a group of soft tissue sarcomas. It is a rare disease, since its discovery in 1989, only hundreds of cases have been described in the literature. Due to the rare incidence of the tumor, this disease remains unknown in common medical practice. It occurs most often in men at young age. Its prognosis is serious and the average survival of patients ranges from 1.5 to 2.5 years. Treatment options include surgical resection, chemo-, radio- and targeted therapy. Our work presents a case report of a 40 year-old patient sufferred from this sarcoma. The first manifestation of the disease was an incarcerated epigastric hernia with omentum and sarcoma metastasis. A resection of the incarcerated part of the omentum was performed with a biopsy from another intra-abdominal lesion. Biopsy specimens were sent for histopathological evaluation. For the generalization of the disease, further surgery was not indicated and systemic palliative chemotherapy in the VDC-IE regimen was chosen. At the time of submitting the manuscript, the patient has survived 6 months since the surgical procedure.
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