Subscribe to RSS
Advanced Orofacial Rhabdomyosarcoma: A Retrospective Study of 31 Cases
16 June 2015
28 September 2015
19 February 2016 (online)
Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma encountered in childhood and adolescence. Early diagnosis of pediatric cases is critical to improving outcomes, especially when socioeconomic status and geographical access to specialist services can reduce opportunities for early cancer detection and treatment.
Objective The objective of this study is to determine factors that can delay referral and treatment in specialist pediatric oncology center upon our population specificities.
Methods This retrospective study involved 31 children between 2003 and 2013. Children affected by histologically confirmed RMS occurring as a primary lesion in the orofacial area were included.
Results The median age was 8 ± 4.22 years (range: 3 months – 15 years). The male to female ratio was 1.8:1. Most of the patients had advanced stage disease at presentation (81.7% group had 3–4 pretreatment staging) with parameningeal involvement in 80.6% of the cases. The 2-year event-free survival rate was 17.7 ± 7.8% for all the patients. Delay of admission to our unit and abandonment of treatment seem to be important factors for the dismal prognosis.
Conclusion Patient's location, socioeconomic status and health care coverage have had an impact on longer delays in seeking care and on follow-up. More studies are needed for implementation of a better management practices and a better supportive care upon specificities of our population.
- 1 Weber RS, Benjamin RS, Peters LJ, Ro JY, Achon O, Goepfert H. Soft tissue sarcomas of the head and neck in adolescents and adults. Am J Surg 1986; 152 (4) 386-392
- 2 Chigurupati R, Alfatooni A, Myall RW, Hawkins D, Oda D. Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases. Oral Oncol 2002; 38 (5) 508-515
- 3 Pappo AS, Shapiro DN, Crist WM, Maurer HM. Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol 1995; 13 (8) 2123-2139
- 4 Wiener ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg 1994; 3 (3) 203-206
- 5 Al-Khateeb T, Bataineh AB. Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002; 93 (5) 580-585
- 6 Fletcher CDM, Unni KK, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2002: 142-154
- 7 Antillon F, Castellanos M, Valverde P , et al. Treating Pediatric soft tissue sarcomas in a country with limited resources: the experience of the Unidad Nacional de Oncologia Pediatrica in Guatemala. Pediatr Blood Cancer 2008; 51 (6) 760-764
- 8 Maurer HM, Beltangady M, Gehan EA , et al. The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 1988; 61 (2) 209-220
- 9 Lawrence Jr W, Anderson JR, Gehan EA, Maurer H . Children's Cancer Study Group. Pediatric Oncology Group. Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Cancer 1997; 80 (6) 1165-1170
- 10 Sbeity S, Abella A, Arcand P, Quintal MC, Saliba I. Temporal bone rhabdomyosarcoma in children. Int J Pediatr Otorhinolaryngol 2007; 71 (5) 807-814
- 11 Peters E, Cohen M, Altini M, Murray J. Rhabdomyosarcoma of the oral and paraoral region. Cancer 1989; 63 (5) 963-966
- 12 Fatusi OA, Ajike SO, Olateju SO, Adebayo AT, Gbolahan OO, Ogunmuyiwa SA. Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population. Int J Oral Maxillofac Surg 2009; 38 (3) 256-260
- 13 Ndom P. Challenges of anticancer chemotherapy in Africa. Can J Urol 2008; 15 (1) 3909-3911