Neglected primary Ewing's sarcoma of ethmoid presenting as surgical emergency
A 10-year-old male child was brought by his parents with history of recurrent epistaxis for 2 years for which endoscopic biopsy was performed elsewhere. The biopsy was inconclusive. His epistaxis subsided but parents noted gradual widening of nasal bridge, proptosis, and decrease in the vision in both eyes. A week prior to admission he had another bout of epistaxis.\nExamination revealed bilateral severe proptosis with conjunctival chemosis and complete ophthalmoplegia in left and partial in right eye []. His visual acuity was counting fingers at a distance of 2 metres in right eye and 1 metre in the left eye. Nasal bridge was widened with pinkish tumor visible through the anterior nares. Rest of examination was unremarkable.\nCT scan revealed a well-defined hyperdense lesion 8.2 × 8.6 × 9.2 cm3 in basifrontal region extending through the ethmoid sinuses into bilateral nasal cavities, orbits, and maxillary sinuses and eroding the bone. It was enhancing homogenously on contrast [].\nPatient had sudden onset, complete visual loss shortly after admission and hence was taken up for emergency surgical decompression. He was operated by transcranial-transnasal approach and greyish white, moderately vascular predominantly extradural tumor was noted in basifrontal region which was encroaching into bilateral ethmoid sinuses, nasal cavities, orbits, maxillary sinuses up to nasopharynx, and extending posteriorly up to lesser wing of sphenoid with erosion of adjacent bone. Dura was involved by the tumor and only a small part of tumor was extending intradurally. Bilateral external carotid control was taken in the neck. A near total excision was achieved followed by repair of anterior cranial fossa floor in three layers using fascia lata, split calvarial bone flap, and pericranium. Postoperatively, proptosis decreased dramatically [] and visual acuity improved to finger counting at 4 metres in right eye with perception of light in left eye.\nMicroscopic examination revealed the tissue composed of cellular lesion; cells are round to polygonal with scant to moderate amount of cytoplasm and very frequent mitosis with small foci of necrosis. IHC with LCA, Tdt, CK was negative and CD99 positive in tumor cells suggestive of Ewing's sarcoma.\nHe was subjected to chemo-radiotherapy subsequently. Following four cycles of chemotherapy with vincristine (1 mg), doxorubicin (70 mg), cyclophosphamide (1100 mg) intervened with ifosfomide (D1-D4, 1.7 g), and etoposide (D1-D4, 90 mg). After four cycles of chemotherapy, intensity modulated radiotherapy on linear accelator (Clinac-iX-3665), 1.8 Greys per fraction, 31 fractions (total dose- 55.8 Greys) was given. He was continued on chemotherapy (described above) for 10 cycles. He is planned to get chemotherapy for two more cycles. Patient was doing well at follow up of 11months and there was no apparent clinical or radiological [] evidence of local recurrence or systemic metastasis (No hot spot on bone scan).