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International Journal of Clinical Case Reports 2014, Vol. 4, No. 2, 1-5
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4
only if major morbidity can be avoided (Raney, 1983).
In the IRS IV protocol, newer agents like etoposides,
ifosfamide and melphalan were added to standard
treatment of vincristine, actinomycin D, and
cyclophosphamide.
The introduction of multimodal treatment (multi-drug
chemotherapy in conjunction with radiotherapy and/or
surgery) is associated with increase in survival form
approximately 25% before formation of
multi-institutional trials such as the IRS to 70% with
significant improvement in terms of remission rates in
cases of non-metastatic rhabdomyosarcoma in
children which was evident in our patient.
With regards to the present multimodality treatment
regime, patients are categorized according to their risk,
which takes into account the location of the tumor,
histological and surgical results. Low-risk patients are
those who have the best prognosis, whereas
intermediate-risk or high-risk patients have an
increased risk of having relapses and incurable disease.
To separate the features into meaningful categories,
patients are assigned to both a surgicopathologic
clinical group. All patients with metastatic disease
(group IV, stage 4) are considered high risk, except
children and adolescents younger than 14 years with
embryonal rhabdomyosarcoma (ERMS) (Raney,
1983). Although all patients require chemotherapy,
regimens vary depending on the stage and group. In
our case, being in group III and stage I, we used a
multimodal approach according to IRS IV protocol as
pointed out earlier. This gave us a better result as the
boy is back in school doing well.
Whereas PM-RMS patients with intra cranial
extension may experience CNS failure, they are also
at risk for local, regional, and distant failure. Apart
from Intra cranial extension, other high-risk features
of rhabdomyosarcoma are also applicable to PM-RMS:
older age, alveolar histology, and lymph node
involvement. Our patient is a young boy and presented
with no lymphnode involvement.
3 Conclusion
Advances into the multimodality management have
dramatically improved survival in PM-RMS. The
timing, volume, and dose of radiation and
chemotherapy are critical in the treatment of this
disease. As in other malignant tumours of the
paediatric population, an optimal balance between
cure and toxicity is paramount for both chemotherapy
and radiotherapy. The fact that survival improved
from approximately 25% before formation of
multi-institutional trials such as the IRS to 70% is a
remarkable testimony to the effectiveness of
multimodal
treatment
and collaborative
multidisciplinary efforts dedicated to the care of
children with rhabdomyosarcoma. We recommend
thorough examination and investigation including
nasopharyngoscopy for patients with recurrent
epistaxis.
Authors’ contributions
GB did the initial work-up, did literature review and
wrote the initial manuscript. CN and RB did the
operation and contributed in the literature review. JN
and EK helped in the literature review and writing of
the manuscript. DA helped in the preparation of the
histopathological slides and special staining.
References
Abbas A.S., 2005, Rhabdomyosarcoma of the middle ear and
mastoid: a case report and review of the literature, Ear
nose and Throat Journal, 84(12): 780-784
Bale P.M., P.R., and Steves M.M., 1986, Pathology and
behaviour of juvenile rhabdomyosarcoma, F. M (Ed.)
Pathology of neoplasia in children and adolescents,
pp.196-222
Carl M., and G.G. Sotti, 1997, soft tissue sarcoma, Paediatric
oncology, 1: 380-416
Carol J.M., and Richard J.H., eds., 2010, Paediatric head and
neck malignancies, Cummings Otolaryngology Head &
Neck Surgery, pp.2835-2849
Flamant F., Rodary C., Rey A., Praquin M.T., Sommelet D.,
Quintana E., Theobald S., Brunat-Mentigny M., Otten J.,
Voûte P.A., Habrand J.L., Martelli H., Barrett A.,
Terrier-Lacombe M.J., and Oberlin O., 1998, Treatment of
non metastatic rhabdomyosarcoma in childhood and
adolescence, results of the second study of the
international Society of paediatric otolaryngology:
MMT84, European journal of cancer, 34, 1050-1062
http://dx.doi.org/10.1016/S0959-8049(98)00024-0
Hu J., Qiu. J.H., and Liu S.L., 2002, Embryonal