Incarcerated metastasis as the first manifestation of a rare sarcoma
Authors:
M. Pekař 1,2; Z. Janda 3; K. Franková 4
Authors‘ workplace:
Centrum cévní a miniinvazivní chirurgie, Nemocnice AGEL Třinec-Podlesí
1; Fyziologický ústav, Lékařská fakulta, Masarykova Univerzita, Brno
2; Chirurgie, Nemocnice AGEL Ostrava-Vítkovice
3; Patologie, Nemocnice AGEL Nový Jičín
4
Published in:
Rozhl. Chir., 2023, roč. 102, č. 1, s. 28-31.
Category:
Case Report
doi:
https://doi.org/10.33699/PIS.2023.102.1.28–31
Overview
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.
Keywords:
Hernia – metastasis – sarcoma – desmoplastic small round cell tumor
Sources
1. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol. 1989;9(2):177– 183. doi:10.3109/15513818909022347.
2. Li X, Yu J, Fang S, et al. Desmoplastic small round cell tumor: a case report and review of the literature. World J Surg Oncol. 2014;12(1):9. doi:10.1186/1477-7819-12-9.
3. Lettieri CK, Garcia-Filion P, Hingorani P. Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database. J Cancer Epidemiol. 2014:680126. doi:10.1155/2014/680126.
4. Subbiah V, Lamhamedi-Cherradi S-E, Cuglievan B, et al. Multimodality treatment of desmoplastic small round cell tumor: Chemotherapy and complete cytoreductive surgery improve patient survival. Clin Cancer Res. 2018;24(19):4865–4873. doi:10.1158/1078-0432.CCR-18-0202.
5. Honoré C, Delhorme JB, Nassif E, et al. Can we cure patients with abdominal desmoplastic small round cell tumor? Results of a retrospective multicentric study on 100 patients. Surg Oncol. 2019;29:107–112. doi:10.1016/j.suronc.2019.04.002.
6. Hendricks A, Boerner K, Germer CT, et al. Desmoplastic small round cell tumors: A review with focus on clinical management and therapeutic options. Cancer Treat Rev. 2021;93:102140. doi:10.1016/j. ctrv.2020.102140.
7. Livaditi E, Mavridis G, Soutis M, et al. Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience. Eur J Pediatr Surg. 2006;16(6):423–427. doi:10.1055/s-2006-924736.
8. Bulbul A, Noel Fahy B, Xiu J, et al. Desmoplastic small round blue cell tumor: A review of treatment and potential therapeutic genomic alterations. Sarcoma 2017:1278268. doi:10.1155/2017/1278268.
9. Loktev A, Shipley JM. Desmoplastic small round cell tumor (DSRCT): emerging therapeutic targets and future directions for potential therapies. Expert Opin Ther Targets 2020;24(4):281–285. doi:10.1080/ 14728222.2020.1738392.
10. Anderson WJ, Doyle LA. Updates from the 2020 World Health Organization classification of soft tissue and bone tumours. Histopathology 2021;78(5):644– 657. doi:10.1111/his.14265.
11. Adámková Krákorová D. Systémová léčba sarkomů měkkých tkání dospělých vyjma GIST. Onkologie 2010;4(5):308–310.
12. Wei G, Shu X, Zhou Y, et al. Intra-abdominal desmoplastic small round cell tumor: current treatment options and perspectives. Front Oncol. 2021;11:705760. doi:10.3389/fonc.2021.705760.
13. Lal DR, Su WT, Wolden SL, et al. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40(1):251–255. doi:10.1016/j.jpedsurg. 2004.09.046.
14. Biswas G et al. Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer 2005;42(2):78. doi: 10.4103/0019-509X.16696.
15. Veselý K. Histopatologická diagnostika nádorů měkkých tkání. Onkologie 2010;4(5):293–296.
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