A rare case in an 11-year old child with bilateral primary orbital rhabdomyosarcoma with rapid onset of bilateral proptosis and temporal lobe abscess secondary to chronic suppurative otitis media

Gupta, B. S.; Tomar, Shubham

doi:10.18203/2320-6012.ijrms20205335

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“…In contrast, the presentation of bilateral protrusion of the eyes is usually caused by the primary orbital RMS itself, although such cases are rare. Gupta and Tomar [ 13 ] reported a similar case of an 11-year-old Hindu boy who exhibited signs of bilateral proptosis with conjunctival chemosis and keratinization. In that case, however, the immunohistochemical analysis demonstrated embryonal characteristics, which is consistent with most cases of primary orbital RMS.…”

Section: Discussion/conclusionmentioning

confidence: 99%

Bilateral Proptosis as an Unusual Presentation of Adult-Onset Alveolar-Type Rhabdomyosarcoma of Ethmoid Sinus: A Case Report

et al. 2022

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Rhabdomyosarcoma (RMS) exhibits varying degrees of clinical manifestations, and one of the determining factors is its primary site of origin. Ocular proptosis is an infrequent presentation of parameningeal RMS. The growing tumor in spacious environments such as air-filled sinuses can obscure its early detection, leading to late disease intervention. Among the four subtypes (embryonal, leiomyomatous, sclerosing and spindle cell, and alveolar), the predominant type of RMS in the paranasal sinuses is alveolar. The incidence in adult-onset RMS is relatively low compared with those of children. We herein present a rare case of a 23-year-old man with an unusual presentation of bilateral proptosis from alveolar RMS of the ethmoid sinus. In contrast to our patient, most reported cases of ocular involvement in RMS turned out to be unilateral and responded poorly to treatment. Despite the aggressive behavior of the adult-onset alveolar subtype in comparable reports, our case shows an excellent outcome. Negative <i>FOXO1</i> fusion status has been recognized in recent studies as a molecular feature inclined toward a favorable outcome in alveolar RMS. The integration of molecular prognostic factors to risk stratification could be advantageous in determining different prognoses and proper management for an individual patient.

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Section: Discussion/conclusionmentioning

confidence: 99%