Intra-abdominal desmoplastic small round cell tumor with liver metastasis: MDT of a case and literatures review_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

ZHOU Kai ^1,2 , HE Xin ³ , CHEN Weixia ⁴ , QIU Meng ⁵ , ZHONG Xiaofei ⁶ , LIAO Mingheng ¹ ,  CEN Ying ² ,  WU Hong ¹

1. Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;
2. Department of Plastic and Burn Surgery, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;
3. Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;
4. Department of Radiology, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;
5. Department of Oncology, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;
6. Department of Ultrasound, West China Hospital of Sichuan University, Chengdu 610041, P. R. China;

Corresponding author：

WU Hong, Email: wuhong7801@163.com

Keywords：

desmoplastic small round cell tumor; liver metastasis; Ewing sarcoma; multi-disciplinary treatment

DOI：

10.7507/1007-9424.201808052

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Objective To explore the clinicopathologic features and treatment of desmoplastic small round cell tumor (DSRCT). Methods By summarizing the diagnosis and treatment of a DSRCT patient with liver metastasis, who was admitted to Department of Liver Surgery in West China Hospital in October 2017, and exploring its clinicopathologic features and treatment by reviewing literatures. Results This patient was generally in good condition, after the multi-disciplinary discussion between the imaging physician, the oncologist, and the liver surgeon, it was considered that there were indications of operation, and after communicating with the patient’ families, actively chose surgical treatment. The performance was successful, and this patient was treated with adjuvant chemotherapy postoperatively. The operative time for this patient was 5 hours, and blood loss was 600 mL. There was no complication occurred, such as bleeding, bile leakage, and intestinal fistula, and discharged on 8 days after surgery. This patient was followed up for 10 months, without tumor recurrence and metastasis occurred. The results of literatures showed that, DSRCT was more common in young male population, the mean age was 12–27 years old, the longest median survival time was 39.2 months, and 3-year survival rate was 20.8%–71%. Those patients who received surgery, had longer median survival time. Conclusions DSRCT is a rare aggressive soft tissue sarcoma, which is usually diagnosed with multiple organ metastases. The treatment is mainly multi-mode treatment based on surgical resection combined with radiotherapy and chemotherapy, but the overall prognosis is poor.

Citation： ZHOU Kai, HE Xin, CHEN Weixia, QIU Meng, ZHONG Xiaofei, LIAO Mingheng, CEN Ying, WU Hong. Intra-abdominal desmoplastic small round cell tumor with liver metastasis: MDT of a case and literatures review. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2018, 25(10): 1242-1248. doi: 10.7507/1007-9424.201808052 Copy

1.	Saab R, Khoury JD, Krasin M, et al. Desmoplastic small round cell tumor in childhood: the St. Jude Children’s Research Hospital experience. Pediatr Blood Cancer, 2007, 49(3): 274-279.
2.	Hayes-Jordan A, LaQuaglia MP, Modak S. Management of desmoplastic small round cell tumor. Semin Pediatr Surg, 2016, 25(5): 299-304.
3.	Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, et al. Multimodality treatment of desmoplastic small round cell tumor: chemotherapy and complete cytoreductive surgery improve patient survival. Clin Cancer Res, 2018, [Epub ahead of print].
4.	Biermann JS, Chow W, Reed DR, et al. NCCN Guidelines Insights: Bone Cancer, Version 2.2017. J Natl Compr Canc Netw, 2017, 15(2): 155-167.
5.	Bisogno G, Ferrari A, Rosolen A, et al. Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor. Bone Marrow Transplant, 2010, 45(5): 907-911.
6.	Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol, 1989, 9(2): 177-183.
7.	Gerald WL, Ladanyi M, de Alava E, et al. 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(9): 3028-3036.
8.	Butt SU, Bull JMH, Scott A. Desmoplastic small round-cell tumor in a young indigenous Australian man: a case report. J Glob Oncol, 2016, 3(1): 79-81.
9.	Gerald WL, Rosai J, Ladanyi M. Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. Proc Natl Acad Sci U S A, 1995, 92(4): 1028-1032.
10.	Worley BS, van den Broeke LT, Goletz TJ, et al. Antigenicity of fusion proteins from sarcoma-associated chromosomal translocations. Cancer Res, 2001, 61(18): 6868-6875.
11.	Rachfal AW, Luquette MH, Brigstock DR. Expression of connective tissue growth factor (CCN2) in desmoplastic small round cell tumour. J Clin Pathol, 2004, 57(4): 422-425.
12.	Lee SB, Kolquist KA, Nichols K, et al. The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. Nat Genet, 1997, 17(3): 309-313.
13.	Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma, 2012, 2012: 714986.
14.	Lae ME, Roche PC, Jin L, et al. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol, 2002, 26(7): 823-835.
15.	Bulbul A, Fahy BN, Xiu J, et al. Desmoplastic small round blue cell tumor: a review of treatment and potential therapeutic genomic alterations. Sarcoma, 2017, 2017: 1278268.
16.	Chen J, Wu Z, Sun B, et al. Intra-abdominal desmoplastic small round cell tumors: CT and FDG-PET/CT findings with histopathological association. Oncol Lett, 2016, 11(5): 3298-3302.
17.	Thomas R, Rajeswaran G, Thway K, et al. Desmoplastic small round cell tumour: the radiological, pathological and clinical features. Insights Imaging, 2013, 4(1): 111-118.
18.	Vicens RA, Patnana M, Le O, et al. Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists. Abdom Imaging, 2015, 40(2): 436-456.
19.	Ostermeier A, McCarville MB, Navid F, et al. FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up. Pediatr Radiol, 2015, 45(9): 1308-1315.
20.	Kushner BH, Laquaglia MP, Gerald WL, et al. Solitary relapse of desmoplastic small round cell tumor detected by positron emission tomography/computed tomography. J Clin Oncol, 2008, 26(30): 4995-4996.
21.	Völker T, Denecke T, Steffen I, et al. Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol, 2007, 25(34): 5435-5441.
22.	Caraway NP, Fanning CV, Amato RJ, et al. Fine-needle aspiration of intra-abdominal desmoplastic small cell tumor. Diagn Cytopathol, 1993, 9(4): 465-470.
23.	Crapanzano JP, Cardillo M, Lin O, et al. Cytology of desmoplastic small round cell tumor. Cancer, 2002, 96(1): 21-31.
24.	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.
25.	Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol, 2011, 23(4): 385-389.
26.	Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med, 2003, 348(8): 694-701.
27.	Pinnix CC, Fontanilla HP, Hayes-Jordan A, et al. Whole abdominopelvic intensity-modulated radiation therapy for desmoplastic small round cell tumor after surgery. Int J Radiat Oncol Biol Phys, 2012, 83(1): 317-326.
28.	Hayes-Jordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol, 2014, 21(1): 220-224.
29.	Honoré C, Amroun K, Vilcot L, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol, 2015, 22(4): 1073-1079.
30.	朱容萱, 王坚, 朱莉菲, 等. 促结缔组织增生性小圆细胞肿瘤的临床特征及预后因素分析. 上海交通大学学报: 医学版, 2018, 37(3): 281-287.
31.	Hayes-Jordan A, Green H, Fitzgerald N, et al. Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatr Surg, 2010, 45(5): 1000-1006.
32.	邢镨元, 石远凯, 冯奉仪, 等. 促纤维组织增生性小圆细胞肿瘤的临床特征及治疗模式探讨. 中华肿瘤杂志, 2010, 32(2): 139-142.
33.	Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol, 1996, 14(5): 1526-1531.
34.	Goodman KA, Wolden SL, La Quaglia MP, et al. Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys, 2002, 54(1): 170-176.
35.	Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res, 2013, 3(1): 14.

1. Saab R, Khoury JD, Krasin M, et al. Desmoplastic small round cell tumor in childhood: the St. Jude Children’s Research Hospital experience. Pediatr Blood Cancer, 2007, 49(3): 274-279.
2. Hayes-Jordan A, LaQuaglia MP, Modak S. Management of desmoplastic small round cell tumor. Semin Pediatr Surg, 2016, 25(5): 299-304.
3. Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, et al. Multimodality treatment of desmoplastic small round cell tumor: chemotherapy and complete cytoreductive surgery improve patient survival. Clin Cancer Res, 2018, [Epub ahead of print].
4. Biermann JS, Chow W, Reed DR, et al. NCCN Guidelines Insights: Bone Cancer, Version 2.2017. J Natl Compr Canc Netw, 2017, 15(2): 155-167.
5. Bisogno G, Ferrari A, Rosolen A, et al. Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor. Bone Marrow Transplant, 2010, 45(5): 907-911.
6. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol, 1989, 9(2): 177-183.
7. Gerald WL, Ladanyi M, de Alava E, et al. 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(9): 3028-3036.
8. Butt SU, Bull JMH, Scott A. Desmoplastic small round-cell tumor in a young indigenous Australian man: a case report. J Glob Oncol, 2016, 3(1): 79-81.
9. Gerald WL, Rosai J, Ladanyi M. Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. Proc Natl Acad Sci U S A, 1995, 92(4): 1028-1032.
10. Worley BS, van den Broeke LT, Goletz TJ, et al. Antigenicity of fusion proteins from sarcoma-associated chromosomal translocations. Cancer Res, 2001, 61(18): 6868-6875.
11. Rachfal AW, Luquette MH, Brigstock DR. Expression of connective tissue growth factor (CCN2) in desmoplastic small round cell tumour. J Clin Pathol, 2004, 57(4): 422-425.
12. Lee SB, Kolquist KA, Nichols K, et al. The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. Nat Genet, 1997, 17(3): 309-313.
13. Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma, 2012, 2012: 714986.
14. Lae ME, Roche PC, Jin L, et al. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol, 2002, 26(7): 823-835.
15. Bulbul A, Fahy BN, Xiu J, et al. Desmoplastic small round blue cell tumor: a review of treatment and potential therapeutic genomic alterations. Sarcoma, 2017, 2017: 1278268.
16. Chen J, Wu Z, Sun B, et al. Intra-abdominal desmoplastic small round cell tumors: CT and FDG-PET/CT findings with histopathological association. Oncol Lett, 2016, 11(5): 3298-3302.
17. Thomas R, Rajeswaran G, Thway K, et al. Desmoplastic small round cell tumour: the radiological, pathological and clinical features. Insights Imaging, 2013, 4(1): 111-118.
18. Vicens RA, Patnana M, Le O, et al. Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists. Abdom Imaging, 2015, 40(2): 436-456.
19. Ostermeier A, McCarville MB, Navid F, et al. FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up. Pediatr Radiol, 2015, 45(9): 1308-1315.
20. Kushner BH, Laquaglia MP, Gerald WL, et al. Solitary relapse of desmoplastic small round cell tumor detected by positron emission tomography/computed tomography. J Clin Oncol, 2008, 26(30): 4995-4996.
21. Völker T, Denecke T, Steffen I, et al. Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol, 2007, 25(34): 5435-5441.
22. Caraway NP, Fanning CV, Amato RJ, et al. Fine-needle aspiration of intra-abdominal desmoplastic small cell tumor. Diagn Cytopathol, 1993, 9(4): 465-470.
23. Crapanzano JP, Cardillo M, Lin O, et al. Cytology of desmoplastic small round cell tumor. Cancer, 2002, 96(1): 21-31.
24. 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.
25. Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol, 2011, 23(4): 385-389.
26. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med, 2003, 348(8): 694-701.
27. Pinnix CC, Fontanilla HP, Hayes-Jordan A, et al. Whole abdominopelvic intensity-modulated radiation therapy for desmoplastic small round cell tumor after surgery. Int J Radiat Oncol Biol Phys, 2012, 83(1): 317-326.
28. Hayes-Jordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol, 2014, 21(1): 220-224.
29. Honoré C, Amroun K, Vilcot L, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol, 2015, 22(4): 1073-1079.
30. 朱容萱, 王坚, 朱莉菲, 等. 促结缔组织增生性小圆细胞肿瘤的临床特征及预后因素分析. 上海交通大学学报: 医学版, 2018, 37(3): 281-287.
31. Hayes-Jordan A, Green H, Fitzgerald N, et al. Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatr Surg, 2010, 45(5): 1000-1006.
32. 邢镨元, 石远凯, 冯奉仪, 等. 促纤维组织增生性小圆细胞肿瘤的临床特征及治疗模式探讨. 中华肿瘤杂志, 2010, 32(2): 139-142.
33. Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol, 1996, 14(5): 1526-1531.
34. Goodman KA, Wolden SL, La Quaglia MP, et al. Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys, 2002, 54(1): 170-176.
35. Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res, 2013, 3(1): 14.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Intra-abdominal desmoplastic small round cell tumor with liver metastasis: MDT of a case and literatures review

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