Clinical features and outcome regarding head and neck non-rhabdomyosarcoma soft tissue sarcoma in children

doi:10.3969/j.issn.1006-7795.2019.06.020

Abstract

Abstract: Objective The purpose for this study was to explore the clinical presentation, treatment, survival and risk factors of head and neck non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric patients. Methods The clinical data of head and neck NRSTS patients diagnosed between Oct.2012 and Feb.2018 were retrospectively studied. Clinical features, treatment and outcomes were analyzed. Results Eleven patients were enrolled in this study, including 7 boys and 4 girls, with a median age of 5.6 years. Including malignant rhabdoid tumors(n=3),synovial sarcoma(n=2), fibrosarcoma(n=3), malignant neurilemmoma(n=1), undifferentiated small round cell sarcomas with CIC-DUX4 fusion (n=1), and undifferentiated small round cell sarcomas (n=1). The primary site of the tumor including neck (n=4), scalp and facial soft tissue (n=3), oral cavity and oropharynx(n=3), and fossae infratemporalis (n=1). The diameter of the tumor ≥ 5 cm(n=6),<5 cm(n=5). Six patients had local lymph node metastasis, and 3 patients had distant metastasis. All patients(n=11) received systemic chemotherapy, radiation therapy was carried out in 3 patients, 4 patients received ¹²⁵I particle implantation, 4 patients did not receive any adjuvant radiation. Surgery was performed in 5 patients, and 6 patients only received biopsy. The follow-up time was 10-78 month, and median follow-up time was 19 month. The estimated mean survival time was (64.7±6.9) month (95%CI:51.1-78.2%). The 1-year overall survival was (88.9±10.5)%. Conclusion In this series, most of the pediatric head and neck NRSTS are nonmetastatic, and the short-term outcome is favorable. ¹²⁵I particle implantation is effective for local control, and improved systemic therapies are needed for patients with metastatic disease.

Key words: children, head and neck, non-rhabdomyosarcoma soft tissue sarcoma, malignant rhabdoid tumor, undifferentiated small round cell sarcomas with CIC-DUX4 fusion

CLC Number:

R739.91

Duan Chao, Wang Shengcai, Jin Mei, Zhang Dawei, Zhao Wen, Wang Xisi, Zhao Qian, Tai Jun, Zhang Jie, He Lejian, Zhang Jianguo, Ni Xin, Ma Xiaoli. Clinical features and outcome regarding head and neck non-rhabdomyosarcoma soft tissue sarcoma in children[J]. Journal of Capital Medical University, 2019, 40(6): 921-926.

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URL: https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/10.3969/j.issn.1006-7795.2019.06.020

https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/Y2019/V40/I6/921

References

[1] 樊征夫, 李舒, 方志伟, 等. 儿童非横纹肌肉瘤软组织肉瘤的美国儿童肿瘤协作组危险度评估与综合治疗单中心研究[J]. 中华实用儿科临床杂志, 2016, 31(7):535-539.
[2] Federico S M, Gilpin D, Samant S, et al. Clinical features and outcomes of young patients with head and neck non-rhabdomyosarcoma soft tissue sarcomas[J]. Head Neck, 2015, 37(1):76-83.
[3] Pang A, Carini M, Maki R G. Contemporary therapy for adcanced soft-tissue sarcoma in adults:A review[J]. JAMA Onco, 2016, 2(7):941-947.
[4] Tudor-Green B, Gomez R, Brennan P A. Current update on the diagnosis and management of head and neck soft tissue sarcoma[J]. J Oral Pathol Med, 2017, 46(9):674-679.
[5] Million L, Donaldson S S. Resectable pediatric nonrhabdoyosarcoma soft tissue sarcoma:which patients benefit from adjuvant radiation therapy and how much?[J]. ISRN Oncol, 2012, 2012:341408.
[6] Spunt S L, Pappo A S. Childhood non-rhabdomyosarcoma soft tissue sarcomas are not adult type tumors[J]. J Clin Oncol, 2006,24(12):1958-1959.
[7] Pappo A S, Rao B N, Jenkins J J, et al. Metastatic non-rhabdomyosarcomatous soft-tissue sarcomas in children and adolescents:the St. Jude Children's Research Hospital experience[J]. Med Pediatr Oncol, 1999,33(2):76-82.
[8] Tinkle C L, Fernandez-Pineda I, Sykes A, et al. Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients:Results from a prospective study using limited-margin radiotherapy[J]. Cancer, 2017,123(22):4419-4429.
[9] Cahlon O, Brennan M F, Jia X, et al. A postoperative nomogram for local recurrence risk in extremity soft tissue sarcomas after limb-sparing surgery without adjuvant radiation[J]. Ann Surg, 2012,255(2):343-347.
[10] Waxweiler T V, Rusthoven C G, Proper M S, et al. Non-rhabdomyosarcoma soft tissue sarcoma in Children:A suveilance, epidemiology, and end results analysis validating COG risk stratification[J]. Int J Radiat Oncol Bio Phys, 2015, 92(2):339-348.
[11] Alektiar K M, Brennan M F, Singer S. Local control comparison of adjuvant brachytherapy to intensity-modulated radiotherapy in primary high-grade sarcoma of the extremity[J]. Cancer, 2011,117(14):3229-3234.
[12] Childs S K, Kozak K R, Friedmann A M, et al. Proton radiotherapy for parameningeal rhabdomyosarcoma:clinical outcomes and late effects[J]. Int J Radiat Oncol Biol Phys, 2012,82(2):635-642.
[13] Wasif N, Smith C A, Tamurian R M, et al. Influence of physician specialty on treatment recommendations in the multidisciplinary management of soft tissuesarcoma of the extremities[J]. JAMA Surg, 2013, 148(7):632-639.
[14] Felderhof J M, Creutzberg C L, Putter H, et al. Long-term clincal outcome of patients with soft tissue sarcomas treated with limb-sparing surgery and postoperative radiotherapy[J]. Acta Oncol, 2013,52(4):745-752.
[15] Daigeler A, Harati K, Goertz O, et al. Prognostic factors and surgical tactics in patients with locally recurrent soft tissue sarcomas[J]. Handchir Mikrochir Plast Chir,2015, 47(2):118-127.
[16] Duan C, Zheng L,Zhang D W,et al.Treatment of malignant rhabdoid tumors of the head and neck with combined chemotherapy and 125I particle implantation[J].Pediatr Invest,2018,2(1):53-55.
[17] Rehders A, Hosch S B, Scheunemann P, et al. Benefit of surgical treatment of lung metastasis in soft tissue sarcoma[J]. Arch Surg, 2007, 142(1):70-75.
[18] Paulino A C, Ritchie J, Wen B C. The value of postoperative radiotherapy in childhood nonrhabdomyosarcoma soft tissue sarcoma[J]. Pediatr Blood Cancer, 2004, 43(5):587-593.
[19] 肖绍文, 刘长青,方志伟,等.软组织肉瘤术后放疗392例临床观察[J].中华肿瘤防治杂志,2018,25(8):578-580,585.
[20] Kawai A, Woodruff J, Healey J H, et al. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma[J]. N Engl J Med, 1998, 338(3):153-160.
[21] Adem C, Gisselsson D, Dal Cin D, et al. ETV6 rearrangements in patients with infantile fibrosarcomas and congenital mesoblastic nephromas by fluorescense in situ hybridization[J]. Mod Pathol, 2001,14(12):1246-1251.
[22] Choi E Y, Thomas D G, McHugh J B, et al. Undifferentiated small round cell sarcoma with t(4;19)(q35;q13.1) CIC-DUX4 fusion:a noval highly aggressive soft tissue tumor with distinctive histopathology[J]. Am J Surg Pathol, 2013,37(9):1379-1386.