REVIEW ARTICLE: Paediatric nasopharyngeal rhabdomyosarcoma: A case series and literature review

Abstract

Rhabdomyosarcoma (RMS) is the most common soft tissue tumour in children, with the head and neck region accounting for 35-40% of cases. Nasopharyngeal RMSs tend to grow rapidly and invade adjacent structures. Both the Intergroup Rhabdomyosarcoma Studies and the European Studies have established that the ideal management of this disease is multimodal, using a combination of surgery, chemotherapy and radiotherapy. This case series examines the role of radiotherapy in the management of paediatric nasopharyngeal RMSs, with particular reference to long-term morbidity and disease-free survival. The cases of five children with nasopharyngeal RMS were reviewed and a systematic review of the literature contained in the PubMed databases was conducted to establish 24 individually detailed cases. Management in all patients was multimodal, using a combination of chemotherapy, radiotherapy as well as surgery. External beam radiotherapy is an integral component of treatment for nasopharyngeal RMSs. With more patients surviving for longer periods, more long-term sequelae of radiotherapy have been reported. Complications include sensorineural deafness, endocrine manifestations following radiation of the pituitary gland, cranial nerve palsies, second malignancies within the radiation field, cataract formation, retinopathy and growth disturbance. Morbidity from radiotherapy may be considerable and depends on the field and dose of radiation. Current advances in radiotherapy are aimed at improving the rate of tumour control and reducing such complications. Recent improvements in imaging and conformal techniques have the potential to reduce the morbidity associated with radiotherapy in this cohort.

Computed tomography and magnetic resonance imaging observations of rhabdomyosarcoma in the head and neck

“…The incidence rate of HNRMS is uncertain between males and females (5–7,14,15); however, the present study exhibited a female predominance (70%). A previous study reported that 43% of RMSs occur prior to reaching five years of age and 78% occur prior to reaching 12 years of age (6), which is consistent with the current study where the median age of patients was 16 years, with 70% of patients <20 years old.…”

“…Calcification and hemorrhaging are rare in HNRMS (2,4–7,15,19) and accordingly, these features were not present radiologically or pathologically in the current study. RMS is an aggressive malignancy, which spreads via three routes; direct extension, lymphatic metastasis and hematogenous metastasis.…”

“…On CT and MRI, the present study clearly demonstrated multi-cavity growth (70%) of HNRMS and three types of direct intracranial extension, including nasocranial, ORB-cranial and nasopharyngeal-cranial communication. The frequency of lymphatic metastasis was 10–20% for HNRMS and is more common in other sites (5,7,15,19,22). In addition, cervical lymph node metastases were detected in two patients (20%, 2/10) with the embryonal subtype, however, no distant metastasis was observed.…”

Oral rehabilitation with implant-based prostheses of two adult patients treated for childhood rhabdomyosarcoma

“…Surgical resection of rhabdomyosarcoma is challenging and can result in large maxillofacial defects with loss of function and esthetics of the surrounding tissues. Radiotherapy may result in facial growth retardation, neuroendocrine dysfunction due to radiation injury of the pituitary gland, visual problems, and hearing loss [2, 3]. In addition, both chemotherapy and radiotherapy have widespread effects on oral tissues.…”

Spontaneous internal jugular vein thrombosis in rhabdomyosarcoma of the nasopharynx

“…Staging is determined by the tumour site, size and metastases. Parameningeal sites including the nasopharynx are at risk of extension to the skull base resulting in poor surgical access and a less favourable prognosis 1. Spontaneous IJV thrombosis is described in malignancy and also in neck trauma from damage to the vessel 2…”