Clinical Characteristics and the Prognosis of Childhood Rhabdomyosarcoma in 60 Patients treated at a Single Institute
Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma in children. The aim of study was to retrospectively review the treatment results of childhood rhabdomyosarcoma and identify prognostic factors. 60 children with rhabdomyosarcoma treated between 1996 and 2002 in Shafa Hospital were reviewed. The data were analyzed for clinico-epidemiological factors. Age, gender, race, histology type, primary site, tumor size and intergroup rhabdomyosarcoma study (IRS) group were evaluated. The primary site of involvement was orbit in 6 cases (10%) head and neck nonparameningial in 12 cases (20%), parameningial region in 12 cases (20%). The histological findings were as follows: 12 cases (72.5%) for embryonal, 6 cases (10%) for alveolar and 11 cases (17. 5%) for botryoid type. With respect to the IRS III (15%) were group II, 32 (52.5%) were group III and 24 cases (40%) were group IV. The 5-year survival rate was 47.9%. Primary tumor site (P=0.0003), and histology (P=0.05) were associated significantly with survival after recurrence. Among the variables, age, gender, regional lymph node involvement, and IRS group did not affect 5-year survival but the type and time of recurrence (P=0.0002), and its relation with therapy (P=0.0001) were associated with survival. This study showed that overall survival for rhabdomyosarcoma is dependent on histological subtype, primary site, disease group, duration of disease before treatment. The outcome for infant with RMS is less satisfactory than older children and the patients aged 1-9 years had the best 5 year survival.
Pizzo PA, Poplack DG. Principle and Practice of PediatricmOncology. 5th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005. p. 971-2.
Boué DR, Parham DM, Webber B, Crist WM, Qualman SJ. Clinicopathologic study of ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV. Pediatr Dev Pathol 2000;3(3):290-300.
Gurney JG, Young Jr JL, Roffers SD, Smith MA, Bunin GR. Soft tissue sarcomas. In: Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR, editors. Cancer Incidence and survival among children and Adolesceents: United States SEER program 1975-1995. Bethesda, MD: National Cancer Institute, SEER Program; 1999. p. 111.
Lanzkowsky PH. Manual of Pediatric Hematology and Oncology. 4th ed. Boston: Elsevier Academic Press; 2005. p. 561-2.
Stiller CA, Parkin DM. International variations in the incidence of childhood soft-tissue sarcomas. Paediatr Perinat Epidemiol 1994;8(1):107-19.
Maurer HM, Gehan EA, Beltangady M, Crist W, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, et al. The Intergroup Rhabdomyosarcoma Study-II. Cancer 1993;71(5):1904-22.
James DH Jr, Hustu O, Wrenn EL Jr, Johnson WW. Childhood malignant tumors. Concurrent chemotherapy with dactinomycin and vincristine sulfate. JAMA 1966;197(12):1043-5.
Wolden SL, Anderson JR, Crist WM, Breneman JC, Wharam MD Jr, Wiener ES, Qualman SJ, Donaldson SS.Indications for radiotherapy and chemotherapy after complete resection in rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Studies I to III. J Clin Oncol 1999;17(11):3468-75.
Kamen BA, Frenkel E, Colvin OM. Ifosfamide: should the honeymoon be over? J Clin Oncol 1995;13(2):307-9.
Green DM. Evaluation of single-dose vincristine, actinomycin D, and cyclophosphamide in childhood solid tumors. Cancer Treat Rep 1978;62(10):1517-20.
Raney RB Jr. Inefficacy of cisplatin and etoposide as salvage therapy for children with recurrent or unresponsive soft tissue sarcoma. Cancer Treat Rep 1987;71(4):407-8.
Peto R, Pike MC, Armitage P, Breslow NE, Cox DR, Howard SV, Mantel N, McPherson K, Peto J, Smith PG.Design and analysis of randomized clinical trials requiring prolonged observation of each patient. II. analysis and examples. Br J Cancer 1977;35(1):1-39.
Ruymann FB, Grovas AC. Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas. Cancer Invest 2000;18(3):223-41.
Abd El-Aal HH, Habib EE, Mishrif MM. Rhabdomyosarcoma: the experience of the pediatric unit of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) (from January 1992 to January 2001). J Egypt Natl Canc Inst 2006;18(1):51-60.
Shouman T, El-Kest I, Zaza K, Ezzat M, William H, Ezzat I. Rhabdomyosarcoma in childhood: a retrospective analysis of 190 patients treated at a single institution. J Egypt Natl Canc Inst 2005;17(2):67-75.
Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol 2001;19(12):3091-102.
Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995;13(3):610-30.
Suita S, Noguchi S, Takamatsu H, Mizote H, Nagasaki A, Inomata Y, Hara T, Okamura J, Miyazaki S, Kawakami K, Eguchi H, Tsuneyoshi M; Committee for Pediatric Solid Malignant Tumors in the Kyushu Area. Clinical characteristics and the prognosis of rhabdomyosarcoma - a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan. Eur J Pediatr Surg 2005;15(6):409-13.
Akyüz C, Sancak R, Büyükpamukçu N, Atahan L, Göğüş S, Kutluk T, Büyükpamukçu M. Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years. Turk J Pediatr 1998;40(4):491-501.
Nakada K. The clinical features and prognosis of rhabdomyosarcoma: follow-up studies on pediatric tumors from the Japanese Pediatric Tumor Registry 1971-1980. Part II. Committee of Malignant Tumors, Japanese Society of Pediatric Surgeons. Jpn J Surg 1990;20(5):503-9.
Callender TA, Weber RS, Janjan N, Benjamin R, Zaher M, Wolf P, el-Naggar A. Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children. Otolaryngol Head Neck Surg 1995;112(2):252-7.
Mazzoleni S, Bisogno G, Garaventa A, Cecchetto G, Ferrari A, Sotti G, Donfrancesco A, Madon E, Casula L, Carli M; Associazione Italiana di Ematologia e OncologiaPediatrica Soft Tissue Sarcoma Committee. Outcomes and prognostic factors after recurrence in children and adolescents with nonmetastatic rhabdomyosarcoma. Cancer 2005;104(1):183-90.
Rodeberg D, Paidas C. Childhood rhabdomyosarcoma. Semin Pediatr Surg 2006;15(1):57-62.
Flamant F, Rodary C, Rey A, Praquin MT, Sommelet D, Quintana E, Theobald S, Brunat-Mentigny M, Otten J, Voûte PA, Habrand JL, Martelli H, Barrett A, Terrier- Lacombe MJ, Oberlin O. Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84. Eur J Cancer 1998;34(7):1050-62.
Ferrari A, Casanova M, Bisogno G, Zanetti I, Cecchetto G, De Bernardi B, Riccardi R, Tamaro P, Meazza C, Alaggio R, Ninfo V, Carli M; Italian Cooperative Group. Rhabdomyosarcoma in infants younger than one year old: areport from the Italian Cooperative Group. Cancer 2003;97(10):2597-604.
Bradford WB, Jose BO, Butler D, Lindberg RD, Paris K, Spanos WJ Jr, Patel CC, Bertolone SJ. Rhabdomyosarcoma in children: a ten year review. J Ky Med Assoc 1998;96(10):399-402.
La Quaglia MP, Heller G, Ghavimi F, Casper ES, Vlamis V, Hajdu S, Brennan MF. The effect of age at diagnosis on outcome in rhabdomyosarcoma. Cancer 1994;73(1):109-17.
Arndt CA, Donaldson SS, Anderson JR, Andrassy RJ, Laurie F, Link MP, Raney RB, Maurer HM, Crist WM. What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer 2001;91(12):2454-68.
Simon JH, Paulino AC, Smith RB, Buatti JM. Prognostic factors in head and neck rhabdomyosarcoma. Head Neck 2002;24(5):468-73.
Neville HL, Andrassy RJ, Lobe TE, Bagwell CE, Anderson JR, Womer RB, Crist WM, Wiener ES. Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997). J Pediatr Surg 2000;35(2):317-21.
Pappo AS, Anderson JR, Crist WM, Wharam MD, Breitfeld PP, Hawkins D, Raney RB, Womer RB, Parham DM, Qualman SJ, Grier HE. Survival after relapse in children and adolescents with rhabdomyosarcoma: A reportmfrom the Intergroup Rhabdomyosarcoma Study Group. J Clin Oncol 1999;17(11):3487-93.
Copyright (c) Acta Medica Iranica
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.