Case Reports in Oncological Medicine

Case Report

A Rare Case of Nasal Sarcoma with BCOR Internal Tandem Duplication Showing Complete Pathologic Response to the VDC-IE Chemotherapy Protocol

Table 1

Retrospective studies and case reports of patients with BCOR-rearranged undifferentiated round cell sarcomas.


Author	Number of cases	Gender/age	Molecular alteration; number of patients	Bone vs. ST	Localized vs. metastatic	Treatment	Necrosis/outcome

Tan et al. [6]	1	M: 21 years old	BCOR::CCNB3: 1	ST	Localized	Surgery and CTX	NR/NR
Suzuki et al. [3]	1	M: 12 years old	BCOR::CCNB3: 1	Bone	Localized	Surgery and CTX	NR/NED at 12 months
Pierron et al. [1]	26	M: 17. F: 9. Mean age: 13.6	BCOR::CCNB3: 26	Bone: 21; ST: 5	Localized: 24; metastatic: 2	Surgery with or without CTX: 12. CTX with or without surgery or RT: 24	Poor necrosis in 13. Not clear for others. AWD:3; NED: 16; DOD: 8
Puls et al. [4]	10	M: 9. F: 1. Mean age: 13.7	BCOR::CCNB3: 10	Bone: 7; ST: 3	Localized: 6; metastatic: 4	CTX+RT: 2. Surgery+CTX: 4. Surgery+CTX + RT: 4	NR/NED: 6; AWD: 1; DOD: 3
Peters et al. [7]	6	M: 6. Age: 7-13 years	BCOR::CCNB3: 6	Bone: 1; ST: 5	Localized: 6	Surgery: 2. Surgery+CTX: 1. Surgery+CTX + RT: 2. CTX + RT: 1	Not applicable. All CTX was adjuvant. NED: 4; DOD: 2
Shibayama et al. [8]	3	M: 3. Mean age: 13.6	BCOR::CCNB3: 3	Bone: 3	Localized: 3	Surgery+CTX: 3	NR/NED: 2; AWD: 1
Ludwig et al. [9]	11	M: 11. Mean age: 12.9	BCOR::CCNB3: 11	Bone: 6; ST: 5	NR	NR	NR/NED: 10; AWD: 1
Yamada et al. [10]	7	M: 2. F: 5. Mean age: 16.5	BCOR::CCNB3: 7	Bone: 6; ST: 1	NR	NR	NR
Matsuyama et al. [11]	11	M: 11. Mean age: 16.4	BCOR::CCNB3: 11	Bone: 4; ST: 7	Localized: 10; metastatic: 1	NR	NR/NED: 7; DOD: 1; NR: 3
Krskova et al. [12]	8	M: 6. F: 2. Mean age: 15.1	BCOR::CCNB3: 8	Bone: 3; ST: 5	Localized: 6; metastatic: 2	CTX + RT: some had surgery	NR/NED: 4; DOD: 3; AWD: 1
Kao et al. [2]	36	M: 31. F: 5. Mean age: 15.1	BCOR::CCNB3: 36	Bone: 20; ST: 14; kidney: 2	Localized: 32; metastatic: 4	Surgery+CTX + RT: 10. Surgery+CTX: 8. Surgery alone: 8. CTX and RT: 1. No data: 9	NR/NED: 13; DOD: 2; DUC: 1; AWD: 6; NR: 14
Rekhi et al. [13]	5	M: 4. F: 1. Mean age: 26	BCOR::CCNB3: 5	Bone: 2; ST: 3	NR	CTX+surgery: 2. CTX + surgery+RT: 1. No data: 2	NR/AWD: 2; DOD: 1; others: NR
Brady et al. [14]	5	M: 4. F: 1. Mean age: 14	BCOR::CCNB3: 5	Bone: 5	NR	Surgery+CTX: 5	NR/NED: 3; DOD: 1; AWD and ongoing treatment: 1
Malik et al. [15]	1	M: 13 years old	BCOR-ITD	Bone	Localized	Surgery+CTX	50% necrosis. Outcome was NR
Yang et al. [16]	2	M: 17 years old. F: 7 months	BCOR-ITD: 1, BCOR::CCNB3: 1.	Bone: 1; ST: one	NR	NR	NR/NR
Antonescu et al. [17]	33	M: 19. F: 14. Age: 19 year. Two were diagnosed at age 1-2 years, other two at 16 and 17 years	BCOR ITD: 28. BCOR- YWHAE-NUTM2B/E fusion: 4. BCOR- YWHAE fusion: 1	Bone: 5; ST: 28	Not clear	Data on 23 patients: surgery+CTX: 14. Surgery +CTX + RT: 4. CTX alone: 5	Necrosis reported for 2 patients: one had >50% necrosis, and the other had 0% necrosis. Outcome data available for 25 patients: DOD: 14; died of other cause: 1; NED: 6; AWD: 4

M: male; F: female; ST: soft tissue; CTX: chemotherapy; RT: radiotherapy; NR: not reported; NED: no evidence of disease; AWD: alive with disease; DOD: died of disease.