Desmoplastic small round cell tumor: a case report and review of the literature

World Journal of Surgical Oncology - Tập 12 - Trang 1-4 - 2014
Xiang Li1, Jing Yu1, Shibao Fang1, Xiaoming Xing2, Jie Zhao2
1Department of Ultrasound, Affiliated Hospital of Medical College Qingdao University, Qingdao, China
2Department of Pathology, Affiliated Hospital of Medical College, Qingdao University, Qingdao, China

Tóm tắt

Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. It affects predominantly young males. In the current report, a 14-year-old male patient was admitted to the hospital for evaluation of abdominal distension, and abdominal pain. Imaging examination revealed a high prevalence of multiple intraperitoneal and liver parenchymal cystic and solid tumors. After an explorative surgery, the pathological findings confirmed the presentation of desmoplastic small round cell tumor. Diagnosis of desmoplastic small round cell tumor could easily have been overlooked since there was no specific evidence for this condition available in the clinical and imaging examinations. In the present study, ultrasound examination detected solid cystic masses, which suggested the presence of necrosis and hemorrhage. Immunohistochemistry and cytogenetic studies confirmed the diagnosis of desmoplastic small round cell tumor in this patient.

Tài liệu tham khảo

Gerald WL, Rosai J: Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol. 1989, 9: 177-183. 10.3109/15513818909022347. Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J: Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol. 1991, 15: 499-513. 10.1097/00000478-199106000-00001. Zhu ZX, Wang J: Softtissue tumor pathology. 2008, Beijing: People's Medical Publishing House Amato RJ, Ellerhorst JA, Ayala AG: Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases. Cancer. 1996, 78: 845-851. 10.1002/(SICI)1097-0142(19960815)78:4<845::AID-CNCR22>3.0.CO;2-U. Hassan I, Shyyan R, Donohue JH, Edmonson JH, Gunderson LL, Moir CR, Arndt CA, Nascimento AG, Que FG: Intraabdominal desmoplastic small round cell tumors: a diagnostic and therapeutic challenge. Cancer. 2005, 104: 1264-1270. 10.1002/cncr.21282. Barnoud R, Sabourin JC, Pasquier D, Ranchere D, Bailly C, Terrier-Lacombe MJ, Pasquier B: Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors. Am J Surg Pathol. 2000, 24: 830-836. 10.1097/00000478-200006000-00008. Gerald WL, Ladanyi M, de Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP, Rosai J: Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998, 16: 3028-3036. Yang JL, Xu WP, Wang J, Zhu XZ, Zhang RY: [Desmoplastic small round cell tumor: a clinicopathologic study of 15 cases]. Zhonghua Bing Li Xue Za Zhi. 2005, 34: 650-655. Cummings OW, Ulbright TM, Young RH, Dei Tos AP, Fletcher CD, Hull MT: Desmoplastic small round cell tumors of the paratesticular region. A report of six cases. Am J Surg Pathol. 1997, 21: 219-225. 10.1097/00000478-199702000-00013. Bismar TA, Basturk O, Gerald WL, Schwarz K, Adsay NV: Desmoplastic small cell tumor in the pancreas. Am J Surg Pathol. 2004, 28: 808-812. 10.1097/01.pas.0000126782.94781.dc. Adsay V, Cheng J, Athanasian E, Gerald W, Rosai J: Primary desmoplastic small cell tumor of soft tissues and bone of the hand. Am J Surg Pathol. 1999, 23: 1408-1413. 10.1097/00000478-199911000-00012. Hayes-Jordan A, Anderson PM: The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol. 2011, 23: 385-389. 10.1097/CCO.0b013e3283477aab. Mingo L, Seguel F, Rollan V: Intraabdominal desmoplastic small round cell tumour. Pediatr Surg Int. 2005, 21: 279-281. 10.1007/s00383-005-1401-1. Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F, Merchant TE, Boulad F, Cheung NK, Bonilla MA, Crouch G, Kelleher JF, Steinherz PG, Gerald WL: Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol. 1996, 14: 1526-1531.