A Rare Cause of Ascites: Desmoplastic Small Round Cell Tumour

Abstract

Desmoplastic small round cell tumour (DSRCT) is a malignant pathologic entity with a mesothelial origin. It is a rare neoplasm of small round cells located in the abdomen, mainly affecting male adolescents and young adults. Herein, we report a 17-year-old man presented with ascite and growing very fast abdominal mass. Laparoscopy was performed showing thickening of omentum and peritoneum and normal internal organs. Histopathological examination of the peritoneal samples concluded to desmoplastic small round cell tumor. The patient was treated according to the cyclophosphamide, vincristine and adriamisin. He responded chemotherapy and reduced intraabdominal, pelvic ascites and intraabdominal masses.

Keywords

• Ascites, Desmoplastic small round cell tumour, Young patient

Asidin Nadir Bir Nedeni: Dezmoplastik Küçük Yuvarlak Hücreli Tümör

Abstract

Dezmoplastik küçük yuvarlak hücreli tümör mezotelyal orjinli malign patolojilidir. Abdomende oldukça nadirdir ve başlıca genç erkeklerde görülür. Oldukça malign seyirlidir. Burada çok hızlı büyüyen abdominal kitle ve asidi olan 17 yaşında erkek hasta sunduk. Laparaskopide periton ve omentunmda kalınlaşma saptandı ve iç organlar normaldi. Histopatolojide periton örneklerinde dezmoplastik küçük yuvarlak hücreler saptandı. Hasta siklofosfamid, vinkristin ve adriamisin ile tedavi edildi. Kemoterapiye cevap verdi ve hastanın intraabdominal ve pelvik asidi ile inraabdominal kitlesi azaldı.

Keywords

• Asit, Dezmoplastik küçük yuvarlak hücreli tümör, Genç hasta

References

1. Quaglia MP, Brennan MF. The clinical approach to desmoplastic small round cell tumor. Surg Oncol 2000; 9: 77-81.
2. Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 2011; 23: 385-9.
3. Jordan AH, Pappo A. Management of desmoplastic small round-cell tumors in children and young adults. J Pediatr Hematol Oncol 2012; 2: 73-5.
4. Roganovich J, Bisogno G, Cecchetto G, D'Amore ES, Carli M. Paratesticular desmoplastic small round cell tumor: case report and review of the literature. J Surg Oncol 1999; 71: 269-72.
5. Taş A, Akbal E, Akgedik S, Köklü S. Mucinous cystadenoma of the peritoneum: A rare cause of ascites. Dig Liver Dis 2010; 42: 910-1.
6. Heikkila AJ, Prebtani AP. Desmoplastic small round cell tumour in a 74 year old man: an uncommon cause of ascites. Diagn Pathol 2011; 6: 55. Gönderilme Tarihi: 02.08.2012