Rare Case of Sellar and Suprasellar Metastasis from Ewing’s Sarcoma of Tibia

Abstract

We hereby report a case of metastatic Ewing's sarcoma presenting with rapid-onset total ophthalmoplegia, optic atrophy, and right temporal hemianopia. Comprehensive ophthalmic and neurological evaluation with targeted radioimaging revealed a tumor mass centered over the sella, compressing optic chiasma, extending to involve the left cavernous sinus and the left orbital apex. Whole-body imaging revealed the evidence of multifocal lung and mediastinal metastasis with focal lytic defect in the left femoral head. Histopathological evaluation of transnasal punch biopsy from the nasopharyngeal extension of the tumor revealed small round-cell tumor with strong CD99 positivity, supporting the diagnosis of Ewing's sarcoma. Rapid, aggressive extensions of the metastatic tumor into vital structures despite the initiation of chemoradiation of the extensive intracranial tumor led to unexpected demise of the patient. Our case is an unusual case of Ewing's sarcoma metastasis manifesting as a sellar mass and mimicking a pituitary adenoma radiologically, with a rapid progression within 2 weeks to cause massive extension of tumor into suprasellar, infrasellar, and left parasellar area, indicative of highly malignant nature of the tumor.

Primary Ewing sarcoma of the sphenoid bone with infrasellar spread in a 17-year-old patient: case report and literature review

“…Выполнена правосторонняя ампу тация нижней конечности ниже колена. Через 3 мес у девочки возник неврологический дефицит: в левом глазу отсутствовало световосприятие, наблюдались аксиальный проптоз (2 мм), тотальные птоз, офталь моплегия, афферентный дефект зрачка с полной атрофией зрительного нерва и потерей роговичного рефлекса [24]. По данным КТ было выявлено объ емное образование размерами 34 × 26 мм в селлярной и супраселлярной областях, сдавливающее оптиче скую хиазму, предположительно -макроаденома гипофиза.…”

Headache with cranial nerve palsy – a diagnostic and therapeutic challenge in three cases

“…A hepatocelluláris carcinoma clivust, sinus cavernosust és a sziklacsont csúcsát érintő metasztázisai okozhatnak kombinált III, IV, VI agyideg bénulást [37]. Leírtak a tíbiából kiinduló metasztatikus Ewing-szarkómát, amely komplett ophtalmoplegiával, optikus ideg atrophiával, illetve temporalis hemianopiával hívta fel magára a figyelmet [38]. Megemlítendőek még a chondromák és chondrosarcomák, amelyek a sinus cavernosus érintettségével változatos agyidegbénulásokat eredményezhetnek [39,40].…”

Commentary

“…In the case presented by Gupta et al .,[9] a 13-year-old female presented to an outpatient facility with headache, ptosis, diplopia, and ophthalmoplegia within 3 months of diagnosis and resection of a ES. This case aptly describes a presentation of cavernous sinus syndrome (CSS) wherein multiple cranial nerve palsies result in ophthalmoplegia, ptosis, and facial sensory loss due to the involvement of adjacent cranial nerves.…”

“…In addition, the optic nerve (cranial nerve two) lies just above and outside the cavernous sinus, superior, and lateral to the pituitary gland on each side, which as in the case presented by Gupta et al ., was also involved. [9]…”