基本HTML版本
International Journal of Clinical Case Reports 2014, Vol. 4, No. 2, 1-5
http://ijccr.biopublisher.ca
1
Paediatric Parameningeal Rhabdomyosarcoma: A Case Report Post-multimodal
Treatment
Gustave Buname
1
, Richard Byaruhanga
1
, Emily Kakande
1
, Justine Namwagala
1
, David Alele
2
,
Christopher Ndoleriire
1
1. Department of Ear, Nose and Throat, Makerere University, Kampala, Uganda
2. Department of pathology, Makerere university, Kampala, Uganda
Corresponding author email:
bishop4jesus@gmail.com
,
International Journal of Clinical Case Reports, 2014, Vol.4, No.2 doi: 10.5376/ijccr.2014.04.0002
Received: 06 Feb., 2014
Accepted: 27 Mar., 2014
Published: 7 Jun., 2014
© 2014 Buname et al. This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use,
distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Buname et al., 2014, Paediatric Parameningeal Rhabdomyosarcoma: A Case Report Post-multimodal Treatment, International Journal of Clinical Case Reports,
Vol.4, No.2, 1-5 (doi: 10.5376/ijccr.2014.04.0002)
Abstract
Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood, representing 5% of all childhood cancers. We
are reporting an interesting case of parameningeal rhabdomyosarcoma in a child who underwent multimodal treatment. A 7 year old
boy presented to our clinic with a history of bad breath, nasal obstruction and recurrent epistaxis from the left nostril for 3 months.
On examination he had mild left proptosis with normal eye vision and movements, reddish left nasal mass with a smooth surface.
Paranasal CT scan showed slightly enhancing soft tissue mass 72×77 mm in the nasal cavity that deviated the nasal septum to the
right, extending to the nasopharynx posteriorly and to the maxillary and ethmoidal sinuses with another mass 11×16 mm extension
into the orbit. Biopsy was taken and histology showed embryonal rhabdomyosarcoma.An extensive tumour debulking was done
folwed by chemotherapy (vincristine,dactinomycin and cyclophosphamide) and 50 grays of radiotherapy. A surveillance CT scan a
month after treatment revealed over 90% tumour reduction. All patients with metastatic disease (group IV, stage 4) are considered
high risk, except children and adolescents younger than 14 years with embryonal rhabdomyosarcoma. Advances into the
multimodality management have dramatically improved survival in PM-RMS from approximately 25% to 75%.
Keywords
Rhabdomyosarcoma; Multimodal treatment; CT scan
Introduction
Rhabdomyosarcoma is the most common sarcoma of
childhood and the most common neoplasm in
childhood after neuroblastoma and nephroblastoma
(Carl, 1997; Carol, 2010). It was first described by
Webber in 1854 (Abbas, 2005). Rhabdomyosarcomas
of the head and neck account for approximately 41%
of all paediatric rhabdomyosarcomas, commonly
occurring in the orbit, the nasopharynx, and the ear
(Hu, 2002; Sautter, 2004). Within the head and neck
region, parameningeal rhabdomyosarcoma (PM-RMS)
that is tumour arising from the nasal cavity, paranasal
sinuses,
infratemporal fossa, nasopharynx,
or
mastoid/middle ear represents 16% of all cases and is
associated with early recurrence and poor prognosis
(Sautter, 2004; Turner, 2011). Histologically, it is
classified into embryonal, alveolar, pleomorphic,
and mixed histological subtypes. Embryonal
rhabdomyosarcoma is the most
common
histological variant seen in childhood (Sautter,
2004).
Surgical resection was considered to be the main
mode of treatment before the introduction of first
Intergroup Rhabdomyosarcoma Study (IRS) in 1972
(Raney, 1983). Second line treatment was radiation
therapy, although this resulted in major morbidity and
was associated with poor prognosis in majority of the
patients (Newton, 1988). However, the management
and outcome has greatly improved with the
introduction of multimodal treatment (multi-drug
chemotherapy in conjunction with radiotherapy and/or
surgery) with significant improvement in terms of
remission rates in cases of non-metastatic
rhabdomyosarcoma in children (Flammant, 1998).We
present a case of a 7 years old boy with parameningeal
rhabdomyosarcoma who underwent all the three
treatment modalities.