[Skip to Content]

Claim CME

Pathology and Laboratory Medicine

Orbital Rhabdomyosarcoma With Unusual Histopathology in a Young Adult

To identify the key insights or developments described in this article

1 Credit CME

JAMA Ophthalmology

Published Online: June 16, 2022

Ophthalmic Images

Article Information and Disclosures

Tessnim R. Ahmad, MD¹;

Meleha Ahmad, MD¹;

Robert Kersten, MD¹

Author Affiliations

¹Department of Ophthalmology, Wayne & Gladys Valley Center for Vision, University of California, San Francisco, San Francisco

Read article on JAMA Network

Read Case Claim CME

A 22-year-old man presented with 1 month of worsening left upper eyelid ptosis and diplopia. Best-corrected visual acuity was 20/20 OU. Examination was notable for left eye proptosis, hypoglobus, supraduction deficit, and ptosis with reduced levator function. Magnetic resonance imaging showed a 2.2-cm enhancing extraconal superior orbital mass, which was excised via an anterior orbitotomy through an eyelid crease incision (Figure, A). Histopathology showed primitive, malignant cells arranged in sheets and nests, forming an alveolilike pattern, and an absence of strap cells, suggestive of alveolar rhabdomyosarcoma (Figure, B). However, myogenin staining was patchy, and the tumor lacked FOXO1 fusion, consistent with embryonal rhabdomyosarcoma.¹ The patient underwent chemotherapy and radiotherapy for microscopic residual disease. Rhabdomyosarcoma is the most common orbital malignancy in childhood with a mean age at onset of 7 to 8 years; less than 1% of reported cases occur in adults.² FOXO1 fusion–negative status, primary site in the orbit, and embryonal histopathology are favorable prognostic factors.³

Claim CME

Sign in to take quiz and track your certificates

Buy This Activity

Article Information

Corresponding Author: Robert Kersten, MD, Department of Ophthalmology, Wayne & Gladys Valley Center for Vision, University of California, San Francisco, 490 Illinois St, Floor 5, San Francisco, CA 94158 (robert.kersten@ucsf.edu).

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information, Melike Pekmezci, MD, University of California, San Francisco, for the pathologic images, and Vinil Shah, MD, University of California, San Francisco, for the radiologic images. No one received financial compensation for their contribution.

References

WHO Classification of Tumours Editorial Board. Soft Tissue and Bone Tumours. 5th ed. International Agency for Research on Cancer; 2020.

Weiss SW , Goldblum JR . Rhabdomyosarcoma. In: Weiss SW , Goldblum JR , eds. Enzinger and Weiss’s Soft Tissue Tumors. CV Mosby; 2001:785-835.

Hibbitts E , Chi YY , Hawkins DS , et al. Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors: a report from the Children’s Oncology Group. Cancer Med. 2019;8(14):6437-6448. doi:10.1002/cam4.2504 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.