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Eye

Orbit other than optic nerve

Rhabdomyosarcoma-orbit

Author: Nat Pernick, M.D.

Last author update: 1 November 2015

Last staff update: 29 December 2020

Copyright: 2004-2024, PathologyOutlines.com, Inc.

PubMed Search: Eye rhabdomyosarcoma[title] orbit

Table of Contents

Cite this page: Pernick N. Rhabdomyosarcoma-orbit. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeorbitrms.html. Accessed December 23rd, 2024.

Definition / general

Most common orbital sarcoma in childhood
Usually embryonal and alveolar subtypes (alveolar more aggressive)
Often rapid onset of unilateral proptosis
May occur after radiation therapy for retinoblastoma, close to previously irradiated fields
Tumors in retinoblastoma patients may have rosette-like structures
References: Am J Surg Pathol 1998;22:1351 (postradiation therapy for bilateral retinoblastoma)

Radiology images

AFIP images

CT shows large tumor of orbital floor invading maxillary sinus

Clinical images

AFIP images

Botryoid tumor beneath the conjunctiva

Whole mount images

AFIP images

Large tumor has infiltrated orbit

Microscopic (histologic) description

Syncytium of strap cells with abundant eosinophilic cytoplasm
Also closely packed small round cells with scanty cytoplasm, coarse nuclear chromatin and increased mitotic activity
Many have minimal rhabdomyoblastic differentiation except in occasional mature strap cells with cross striations
Alveolar pattern has fibrovascular septa resembling lung alveoli

Microscopic (histologic) images

AFIP images