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Orbital Rhabdomyosarcoma With Unusual Histopathology in a Young Adult

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1 Credit 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

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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

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
1.
WHO Classification of Tumours Editorial Board.  Soft Tissue and Bone Tumours. 5th ed. International Agency for Research on Cancer; 2020.
2.
Weiss  SW , Goldblum  JR . Rhabdomyosarcoma. In: Weiss  SW , Goldblum  JR , eds.  Enzinger and Weiss’s Soft Tissue Tumors. CV Mosby; 2001:785-835.
3.
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/cam4.2504PubMedGoogle ScholarCrossref
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