rhabdomyosarcoma ( rms ) is a malignant tumor of striated muscle origin and derives from primitive mesenchyme that retains its capacity for skeletal muscle differentiation .
the combined use of chemoradiotherapy and surgery improves the survival rate significantly , namely up to 5 years .
we report on a 42-year - old male patient who , without a significant clinical history , presented to the ent service complaining of nasal obstruction , exophthalmos , edema and ipsilateral facial congestion . ct and mri were performed .
ct showed an aggressive , noncalcified , solid mass centered in the left nasoethmoidal region invading the left side of the frontal sinus , left maxillary sinus and the left orbit .
t2-weighted mri showed a mildly hypointense , solid mass centered in the left nasoethmoidal region invading the left side of the frontal sinus .
the tumor was also invading the left orbit with lateral displacement of the medial rectus muscle and globe ( fig .
3 ) . intracranial invasion and heterogeneous avid enhancement of the mass was seen in the postcontrast fat - saturated images ( fig .
9 ) , s-100 , chromogenin and synaptophysin and negative for nse ( neuron - specific enolase ) .
the case was presented and discussed at the head and neck tumor board and a decision was made to initiate chemoradiation therapy consisting of 4 cycles of ifosfamide , doxoruvicine , vincristin and mesna .
conventional radiotherapy treatment was started with a total dose of 63 gy , administered in 1.8-gy doses during 7 weeks ( fig .
rms is a malignant tumor with striated muscle differentiation deriving from primitive mesenchyme   that retains its capacity for skeletal muscle differentiation .
rms was first described in the english literature in 1937 and in 1992 in children as a tumor mainly composed of bundles of cells with myogenic differentiation by immunohistochemical and ultrastructural analysis .
since then and until 2007 , 21 cases have been described in the english literature .
this sarcoma is one of the most common soft tissue sarcomas in newborns , children , and young adults .
the annual incidence of rms in the usa is 4.6 per million in subjects under 20 years of age .
rms may occur in all age groups but is more prevalent in the first and second decades of life , with a peak between 2 and 6 years of age , representing approximately 48% of all pediatric cancers .
although head and neck tumors are rare in children , approximately 60% of all pediatric rms cases occur in the head and neck [ 9 , 10 , 11 ] .
rms has different grades of striated muscle cell differentiation and it may occur in any part of the body .
four different histopathological types have been described : embryonal , alveolar , pleomorphic and undifferentiated , with embryonal and alveolar being the two most common histopathological types described in childhood .
the embryonal type represents 70% of all cases , is mainly seen in children under the age of 12 and carries the best prognosis .
the alveolar type is more frequent in the extremities , affecting an older age group .
it generally shows the chromosomal translocation t2:13 ; p3514 , carrying a more ominous prognosis than the other types of rms . the pleomorphic variety is less frequent and occurs more often in an older population .
approximately 40% of all newly diagnosed rms arise in head and neck structures including parameningeal sites ( 16% of all cases , and almost half of all head and neck cases ) , the orbit or eyelid ( 10% of all cases ) , and other nonorbit , nonparameningeal sites ( 10% of all cases ) .
the parameningeal sites include the nose , nasopharynx , paranasal sinuses , middle ear , mastoid , infratemporal fossa and pterygopalatine fossa .
rms of the temporal bone carries a poor prognosis due to its proximity to the brain and vital structures .
jaffe et al . found a 2-year survival rate of 0% in a review of 20 cases from the literature .
the nonorbital and nonparameningeal forms include the scalp , parotid gland , oropharynx , larynx and oral cavity .
the tongue , palate and cheeks are the most common oral sites . of the 35% of all rms that occur in the head and neck , 1012% present in the oral cavity .
rms can have a syndromic presentation such as their association with beckwith - wiedemann syndrome ( 10% of all cases ) .
metastasis occurs by hematogenous or lymphatic spread , most commonly to the lungs , bones and brain .
the prognosis is influenced by the anatomic location at the time of presentation , patient 's age , completeness of resection , extent of metastasic disease and tumor histology .
the combined use of chemoradiotherapy and surgery has significantly improved the survival rate of head and neck rms to 5 years in the last 30 years .
a study indicates that approximately 65% of the children diagnosed with rms survive with combined therapy . in the pediatric parameningeal rms cases ,
the treatment of choice is chemoradiotherapy , with surgery having a limited role due to the relative inaccessibility of the lesions and associated surgical morbidity .
improved and innovative operative techniques of craniofacial surgical reconstruction have resulted in satisfactory functional and cosmetic results . in conclusion
, rms is a rare head and neck tumor that occurs in the adult population and has a poor prognosis despite aggressive therapy .
 abstractwe report a patient who presented to the ent service complaining of nasal obstruction , exophthalmos , edema and ipsilateral facial congestion . 
 imaging studies revealed an aggressive noncalcified solid mass centered in the left nasoethmoidal region and heterogeneous avid enhancement following contrast media injection . 
 subsequently , a biopsy confirmed the presence of solid alveolar rhabdomyosarcoma . 
 the patient was treated with chemoradiation therapy for 7 weeks . due to the advanced stage of the disease , 
 the patient was enrolled in a palliative care and pain control program . 
Introduction
Case Presentation
Discussion
Disclosure Statement