Korean J Ophthalmol.  2006 Mar;20(1):70-75. 10.3341/kjo.2006.20.1.70.

A Case of Alveolar Rhabdomyosarcoma of the Ethmoid Sinus Invading the Orbit in an Adult

• Moon HS
• Kwon SW
• Lee JH

• KMID: 1099076
• DOI: http://doi.org/10.3341/kjo.2006.20.1.70

Abstract

PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult. METHODS: A 48-year-old male patient presented at our clinic complaining of proptosis that had persisted for 2 weeks in his left eye. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle. Endoscopic intranasal biopsy revealed alveolar RMS. Conservative debulking and orbital wall decompression were performed. RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS. Since ipsilateral cervical and spinal metastasis was detected, systemic treatment was administered simultaneously. CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors. Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.

Keyword

Adult rhabdomyosarcoma; Ethmoid; Orbital tumor

MeSH Terms

Tomography, X-Ray Computed
Rhabdomyosarcoma, Alveolar/*pathology/radiography
Paranasal Sinus Neoplasms/*pathology/radiography
Orbital Neoplasms/*pathology/radiography
Neoplasm Invasiveness
Middle Aged
Male
Magnetic Resonance Imaging
Humans
*Ethmoid Sinus
Diagnosis, Differential
Biopsy

Figure

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  Fig. 1 Coronal (A) and axial (B,C) CT scans show a well-circumscribed, homogeneous mass in the left ethmoid sinus with extension into the left orbit and compression of the left medial rectus muscle. The tumor shows marked enhancement with contrast agents.

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  Fig. 2 Orbital magnetic resonance imaging (MRI) scans show rhabdomyosarcoma in left ethmoid sinus with low signal intensity on T1-weighted image (A,B) and moderate enhancement on enhanced scan (C). On a T2-weighted image, the lesion is isointense to extraocular muscles and hypointense to orbital fat (D). The lesion extends into the left orbit with compression of the left medial rectus muscle, and has intracranial extension with compression of the left side frontal lobe.

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  Fig. 3 Preoperative photography of a 48-year-old man suffering diplopia, with marked limitation of ocular movement, chemosis, proptosis in the left eye.

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  Fig. 4 Gross examination showed a circumscribed reddish tumor measuring 1.5×1.2×1.0 cm.

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  Fig. 5 Photomicrograph of isolated rhabdomyosarcoma. (A) Low magnification reveals diffuse small round cells (Hematoxylin-eosin, original magnification ×40). (B) High magnification reveals round cells (rhabdomyoblast) with eosinophilic cytoplasm and eccentric nuclei (arrow) (Hematoxylin-eosin, original magnification ×400). (C) Immunohistochemistry gave positive results with desmin, confirming the diagnosis of rhabdomyosarcoma (original magnification ×400). (D) Immunohistochemistry with smooth muscle actin (original magnification ×400).

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  Fig. 6 5-month postoperative photograph showing improvement of ocular movement and enophthalmos in the left eye.

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