10-year-old male child with a 2 years history of epistaxis was brought by his parents. Parents informed about inconclusive results of an endoscopic biopsy which was performed before somewhere else. Gradually, the epistaxis subsided but parents observed that the nose bridge of their child was widening. It was accompanied with proptosis and gradual bilateral loss of vision. Doctors admitted the child and a week before admission the child experienced epistaxis one more time.
Apparently, the facial appearance of child had changed abruptly. Nose and eyes had swollen up. Upon examination it was revealed that widening of nasal bridge occurred due to a pinkish tumor which was clearly visible through the anterior nares. As far as the vision of child was concerned, his visual acuity had decreased dramatically. He could count fingers at a distance of only one and two meters from his left and right eyes respectively. Bilateral protosis and conjunctival chemosis were clearly observable. Doctors diagnosed him with Ewing’s Sarcoma.
Ewing’s sarcoma is an extremely rare bone tumor which develops in children and young adults. In this case, the Ewing’s sarcoma originated from the ethmoid sinus and extended into the anterior cranial fossa, bilateral nasal cavities, orbits and maxillary sinus. Due to this pattern of malignancy the patient was presented with complaints concerning the surrounding structures; proptosis, painful opthalmoplegia, decreased visual acuity along with broadening of the nasal bridge, and epistaxis. The most astonishing fact here is that Ewing’s sarcoma commonly involves long bones followed by pelvis and ribs. In merely 4% cases skull is involved. In case of cranial Ewing’s sarcoma most common site is temporal bone followed by frontal, parietal, occipital, sphenoid and ethmiod. Ethmoid has the least probability of being involved in Ewing’s sarcoma and this case has fallen right into that odd probability!
To confirm the extent and status of Ewing’s sarcoma CT scan was performed. The results revealed a well-defined lesion which enhanced on contract. This hyperdense lesion had dimensions 8.2 x 8.6 x 9.2 cm². It was located in basifrontal region and could be seen extending from ethmoid sinus into the bilateral nasal cavities, orbits and maxillary sinus as shown in the figure below.
Soon after admission of patient complete visual loss was reported which compelled emergency surgical decompression. The approach employed in this case was transcranial-transnasal. A near total excision was practiced in this surgery. By using three layers namely fascia lata, split calvarial bone flap and pericranium the anterior cranial fossa floor was repaired. During surgery, greyish-white extradural tumor was observed which was extending towards ethmoid sinuses, nasal cavities, orbits, maxillary sinuses, nasopharynx and lesser wing of sphenoid. Post-operation observation revealed dramatic decrease in proptosis. The patient could count fingers at a distance of 4 meters from right eye and from left eye only with perception of light.
This case was presented as surgical emergency. The treatment constituted of aggressive surgical excision followed chemoradiotherapy. The patient responded really well to the chemoradiotherapy which continued for 10 cycles. During the follow-up of 11 months no radiological or clinical recurrence of sarcoma was observed. The results were quite satisfactory.