Serra KAMER, Emin DARENDELILER, Yavuz ANACAK, Banu ATALAR, Fulya AĞAOĞLU, Ayça İRIBAŞ, Esra KORKMAZ KIRAKLI, Arzu ERGEN, Fazilet ÖNDER DINÇBAŞ

Adult Rhabdomyosarcoma: Clinical Features and Radiotherapy Outcomes—The Turkish Oncology Group (TOG) Bone and Soft Tissue Sarcoma Study Group

OBJECTIVEAlthough rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma diagnosed in childhood,it represents only 2%–5% of adult soft tissue sarcomas. The aim of the present study was to better understand the clinical characteristics, treatment approaches, and outcomes of patients with adult RMS whoreceived radiotherapy (RT) as a component of their multidisciplinary management since there are scarcedata on adult RMS due to its rarity.METHODSThe medical records of patients with adult RMS who were ≥18 years old and treated with RT betweenJanuary 1995 and August 2016 in four different radiation centers were evaluated in terms of clinicalcharacteristics, treatment, and follow-up data retrospectively.RESULTSThere were 28 patients. The median age at diagnosis was 28 (19–53) years. The most common site of involvement was the head and neck (25%), and parameningeal region involvement was prominent (92%)among them. In general, unfavorable site of involvement was markedly higher than favorable ones (82%vs. 18%). Alveolar and pleomorphic subtypes compromised 75% of the cases. Fifteen patients had surgery, 26 chemotherapy, 10 radical intent of RT, 9 adjuvant, 3 preoperative, and 6 palliative. The follow-uptime was from 3 to 235 (median 18) months, disease-free survival was between 2 and 48 (median 12)months, and 5-year overall survival (OS) was 25% (median OS 20 (4–235) months). There were significant differences in terms of survival according to histopathological subtypes (p: 0.017), risk groups(p

PDF

___

1. Malempati S, Hawkins DS. Rhabdomyosarcoma: review of the Children’s Oncology Group (COG) SoftTissue SarcomaCommittee experience and rationale for current COG studies. Pediatr Blood Cancer 2012;59(1):5–10.
2. Ferrari A, Dileo P, Casanova M, Bertulli R, Meazza C, Gandola L, et al. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a singleinstitution. Cancer 2003;98(3):571–80.
3. Maurer HM, Beltangady M, Gehan EA, Crist W, Hammond D, Hays DM, et al. The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 1988;61(2):209–20.
4. Maurer HM, Gehan EA, Beltangady M, Crist W, Dickman PS, Donaldson SS, et al. The Intergroup Rhabdomyosarcoma Study-II. Cancer 1993;71(5):1904–22.
5. Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995;13(3):610–30.
6. Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, et al. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol 2001;19(12):3091–102.
7. Raney RB, Maurer HM, Anderson JR, Andrassy RJ, Donaldson SS, Qualman SJ, et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols. Sarcoma 2001;5(1):9–15.
8. Frustaci S, Gherlinzoni F, De Paoli A, Bonetti M, Azzarelli A, Comandone A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 2001;19(5):1238–47.
9. Little DJ, Ballo MT, Zagars GK, Pisters PW, Patel SR, El-Naggar AK, et al. Adult rhabdomyosarcoma: outcome following multimodality treatment. Cancer 2002;95(2):377–88.
10.Spalteholz M, Gulow J. Pleomorphic rhabdomyosarcoma infiltrating thoracic spine in a 59-year-old female patient: Case report. GMS Interdiscip Plast Reconstr Surg DGPW 2017;6:Doc11.
11.Khosla D, Sapkota S, Kapoor R, Kumar R, Sharma SC. Adult rhabdomyosarcoma: Clinical presentation, treatment, and outcome. J Cancer Res Ther 2015;11(4):830–4.
12.Van Gaal JC, De Bont ES, Kaal SE, Versleijen-Jonkers Y, van der Graaf WT. Building the bridge between rhabdomyosarcoma in children, adolescents and young adults: the road ahead. Crit Rev Oncol Hematol 2012;82(3):259–79.
13.Leonard L. Gunderson, editors. Pediatric Soft Tissue Sarcomas. In: Clinical Radiation Oncology. 4th ed. Philadelphia: Elseiver: 2016. p.1408.
14.Enneking W, editor. Staging of musculoskeletal neoplasms. Springer Verlag; 1984.
15.Gerber NK, Wexler LH, Singer S, Alektiar KM, han ML, Shi W, et al. Adult rhabdomyosarcoma survival improved with treatment on multimodality protocols. Int J Radiat Oncol Biol Phys 2013;86(1):58–63.
16.Hawkins WG, Hoos A, Antonescu CR, Urist MJ, Leung DH, Gold JS, et al. Clinicopathologic analysis of patients with adult rhabdomyosarcoma. Cancer 2001;91(4):794–803.
17.La Quaglia MP, Heller G, Ghavimi F, Casper ES, Vlamis V, Hajdu S, et al. The effect of age at diagnosis on outcome in rhabdomyosarcoma. Cancer 1994;73(1):109– 17.
18.Lloyd RV, Hajdu SI, Knapper WH. Embryonal rhabdomyosarcoma in adults. Cancer 1983;51(3):557–65.
19.Miettinen M. Rhabdomyosarcoma in patients older than 40 years of age. Cancer 1988;62(9):2060–5.
20.Seidal T, Kindblom LG, Angervall L. Rhabdomyosarcoma in middle-aged and elderly individuals. APMIS 1989;97(3):236–48.
21.Kattan J, Culine S, Terrier-Lacombe MJ, Théodore C, Droz JP. Paratesticular rhabdomyosarcoma in adult patients: 16-year experience at Institut Gustave-Roussy. Ann Oncol 1993;4(10):871–5.
22.Esnaola NF, Rubin BP, Baldini EH, Vasudevan N, Demetri GD, Fletcher CD, et al. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg 2001;234(2):215–23.
23.Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and endresults program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 2009;27(20):3391–7.
24.Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001;23(4):215–20.
25.Egas-Bejar D, Huh WW. Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. Adolesc Health Med Ther 2014;5:115–25.
26.Ulutin C, Bakkal BH, Kuzhan O. A Cohort Study of Adult Rhabdomyosarcoma: A Single Institution Experience. World Journal of Medical Sciences 2008:3(2);54–9.
27.Schürch W, Bégin LR, Seemayer TA, Lagacé R, Boivin JC, Lamoureux C, et al. Pleomorphic soft tissue myogenic sarcomas of adulthood. A reappraisal in the mid-1990s. Am J Surg Pathol 1996;20(2):131–47.
28.Wharam MD Jr, Foulkes MA, Lawrence W Jr, Lindberg RD, Maurer HM, Newton WA Jr, et al. Soft tissue sarcoma of the head and neck in childhood: nonorbital and nonparameningealsites. A report of the Intergroup Rhabdomyosarcoma Study (IRS)-I. Cancer 1984;53(4):1016–9.
29.Gaffney EF, Dervan PA, Fletcher CD. Pleomorphic rhabdomyosarcoma in adulthood. Analysis of 11 cases with definition of diagnostic criteria. Am J Surg Pathol 1993;17(6):601–9.
30.Borinstein SC, Steppan D, Hayashi M, Loeb DM, Isakoff MS, Binitie O, et al. Consensus and controversies regarding the treatment of rhabdomyosarcoma. Pediatr Blood Cancer 2018;65(2).
31.Breneman JC, Lyden E, Pappo AS, Link MP, Anderson JR, Parham DM, et al. Prognostic factors and clinical outcomes in children and adolescents with metastaticrhabdomyosarcoma-a report from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol 2003;21(1):78–84.
32.Nakhleh RE, Swanson PE, Dehner LP. Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults. A clinical, pathologic, and immunohistochemical study of 12 cases. Cancer 1991;67(4):1019–24.