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A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult.

Moon HS, Kwon SW, Lee JH - Korean J Ophthalmol (2006)

Bottom Line: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Gachon Medical School, Gil Medical Center, Incheon, Korea.

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.

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Related in: MedlinePlus

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

Mentions: Gross examination revealed a well-circumscribed, ovoid, reddish tumor, which measured 1.5×1.2×1.0 cm (Fig. 4). Microscopic examination revealed diffuse small spindle-shaped to round cells with abundant eosinophilic cytoplasm and eccentric nuclei. There was no evidence of cross-striation. Immunohistochemical analysis was positive for desmin, S-100, smooth muscle actin (SMA), and CD56, consistent with a diagnosis of alveolar RMS (Fig. 5). After surgery, the patient experienced some return of visual function and ocular motility (Fig. 6). The patient was treated with chemotherapy including adriamycin, combined with irradiation of the orbit and ethmoid sinus of 5800 cGy over a period of 6 weeks. The chemotherapy was discontinued because the patient developed pancytopenia. A bone scan indicated progressive metastasis to the spine and the patient died 7 months after the initial diagnosis.


A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult.

Moon HS, Kwon SW, Lee JH - Korean J Ophthalmol (2006)

5-month postoperative photograph showing improvement of ocular movement and enophthalmos in the left eye.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2908821&req=5

Figure 6: 5-month postoperative photograph showing improvement of ocular movement and enophthalmos in the left eye.
Mentions: Gross examination revealed a well-circumscribed, ovoid, reddish tumor, which measured 1.5×1.2×1.0 cm (Fig. 4). Microscopic examination revealed diffuse small spindle-shaped to round cells with abundant eosinophilic cytoplasm and eccentric nuclei. There was no evidence of cross-striation. Immunohistochemical analysis was positive for desmin, S-100, smooth muscle actin (SMA), and CD56, consistent with a diagnosis of alveolar RMS (Fig. 5). After surgery, the patient experienced some return of visual function and ocular motility (Fig. 6). The patient was treated with chemotherapy including adriamycin, combined with irradiation of the orbit and ethmoid sinus of 5800 cGy over a period of 6 weeks. The chemotherapy was discontinued because the patient developed pancytopenia. A bone scan indicated progressive metastasis to the spine and the patient died 7 months after the initial diagnosis.

Bottom Line: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Gachon Medical School, Gil Medical Center, Incheon, Korea.

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.

Show MeSH
Related in: MedlinePlus