%0 Journal Article
%T Pediatric Sclerosing Rhabdomyosarcomas: A Review
%A Amandeep Kumar
%A Manmohan Singh
%A Mehar C. Sharma
%A Sameer Bakshi
%A Bhawani S. Sharma
%J ISRN Oncology
%D 2014
%R 10.1155/2014/640195
%X Sclerosing RMS (SRMS) is a recently described subtype of RMS that has not yet been included in any of the classification systems for RMSs. We did pubmed search using keywords ¡°sclerosing, and rhabdomyosarcomas¡± and included all pediatric cases (age ¡Ü 18 years) of SRMSs in this review. We also included our case of an eleven-year-old male child with skull base SRMS and discuss the clinical, histopathological, immunohistochemical, and genetic characteristics of these patients. Till now, only 20 pediatric cases of SRMSs have been described in the literature. Pediatric SRMS more commonly affects males at a mean age of 9 years. Extremeties and head/neck regions were most commonly affected. Follow-up details were available for 16 patients with mean follow-up of 25.3 months. Treatment failure rate was 43.75%. Overall amongst these 16 patients, 10 were alive without disease, 4 were alive with disease, and two died. Thus, overall and disease-free survival amongst these 16 patients were 87.5% and 62.5%, respectively. The literature regarding clinical behaviour and outcome of pediatric patients with SRMSs is patchy. Detailed molecular/genetic analysis and clinicopathological characterization with longer follow-ups of more cases may throw some light on this possibly new subtype of RMS. 1. Introduction Rhabdomyosarcoma (RMS) is the commonest soft tissue tumor of childhood [1] with predilection for head and neck, genitourinary system, and extremities [2]. Though head and neck region is the preferred site for occurrence of RMSs, primary intracranial RMSs are rare tumors with an incidence of around 3% [3]. These are rarely considered in the differential diagnoses of intracranial tumors. Pediatric RMSs are classified by International Classification for Childhood Sarcomas into embryonal (ERMS), alveolar (ARMS), botryoid, and spindle cell subtypes [4]. Sclerosing RMS (SRMS), a recently described type of RMS, was first reported by Mentzel and Katenkamp in 2000 [5]. Since then 39 cases have been described in the literature, out of which 20 are pediatric cases. We hereby describe a case of right middle cranial fossa SRMS with extracranial extension in an eleven-year-old male child and discuss the literature pertinent to pediatric SRMSs. 2. Case Illustration 2.1. Clinical Presentation An eleven-year-old male child presented with complaints of headache for the past 6 months. Patient also had recurrent vomiting of 1 month duration. He also complained of numbness over the right side of face. On examination, patient was conscious and oriented. Sensory loss was present over right
%U http://www.hindawi.com/journals/isrn.oncology/2014/640195/