Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Abstract

Abstract. Ewing's sarcoma (ES) is an aggressive tumour that may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is the case report of a ES of the ethmoid sinus with intracranial and orbital extension in a 33-year-old male patient who presented with anosmia, epistaxis, reduction of visual acuity in the left eye and headache. On otorhinolaryngological clinical examination and biopsy via flexible endoscope, the lesion was misdiagnosed as ethmoid sinus carcinoma. The subsequent magnetic resonance imaging (MRI) of the brain revealed a large mass (6x7 cm) eroding the ethmoid and sphenoid sinuses, extending beyond the orbits and occupying the anterior cranial fossa with a maximum extension of ~5 cm. The patient underwent surgical resection and the microscopic examination of the specimen established the diagnosis of ES (immunohistochemically positive for CD99, neuron-specific enolase, CD56, synaptophysin, pancytokeratin, low-molecular weight cytokeratins and vimentin. The periodic acid Schiff stain exhibited strong intracytoplasmic block positivity and fluorescence in situ hybridization revealed a t(22;11) translocation. First-line chemotherapy was administered for 3 cycles; however, on restaging MRI, local disease progression was diagnosed. The patient received radiotherapy and second-line chemotherapy for 6 cycles. At 15 months after the diagnosis, the patient remains recurrence-free and maintains a good functional status and quality of life.

Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease

“…In 2011, Hafezi et al reviewed a total of 63 cases of any age by merging a multicenter series of 14 cases with 49 cases collected by review of the English literature. Subsequently, an additional 11 cases of sinonasal Ewing's sarcoma have been published. Therefore, the overall number is 74, with only 22 patients older than 18 years.…”

Ewing’s sarcoma of sinonasal tract: a rare case

“…The less common, extra skeletal, form involves soft tissue and rarely manifests in head and neck region. 6 Genetically, t (11,22) chromosome translocation is a hall mark of diagnosis. 6 Head and neck ES usually presents in patients younger than 30 yrs of age with a peak incidence in those aged 10 to 15 yrs.…”

Sinonasal FUS-ERG-Rearranged Ewing’s Sarcoma Mimicking Glomangiopericytoma

“…Ewing’s sarcoma is a rare and aggressive tumor that typically presents in the skeletal form but may also present less frequently in the extraskeletal form. Ewing’s sarcoma in the extraskeletal form in the head and neck is rare, and even more rare in the sinonasal tract [1]. Only case reports of sinonasal Ewing’s sarcoma have been reported in the literature [2].…”