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

Mentions: A fiberoptic endoscopic biopsy of the nasal cavity revealed alveolar RMS. Metastatic work-up revealed cervical lymph node involvement, indicating a stage IV RMS based on the Intergroup Rhabdomyosarcoma Study (IRS) TNM Pretreatment Staging System.5 Since distant metastasis was detected, radiation therapy and chemotherapy were scheduled; however, approximately one week later the visual acuity of the left eye had decreased to 20/40, and there was increased proptosis (5.5 mm) and severe limitation of ocular movement (Fig. 3). Surgery was considered necessary to preserve vision and ameliorate the symptoms, therefore conservative debulking and orbital wall decompression were performed through a transconjunctival incision in the inferior fornix under general anesthesia.


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)

Preoperative photography of a 48-year-old man suffering diplopia, with marked limitation of ocular movement, chemosis, proptosis in the left eye.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Preoperative photography of a 48-year-old man suffering diplopia, with marked limitation of ocular movement, chemosis, proptosis in the left eye.
Mentions: A fiberoptic endoscopic biopsy of the nasal cavity revealed alveolar RMS. Metastatic work-up revealed cervical lymph node involvement, indicating a stage IV RMS based on the Intergroup Rhabdomyosarcoma Study (IRS) TNM Pretreatment Staging System.5 Since distant metastasis was detected, radiation therapy and chemotherapy were scheduled; however, approximately one week later the visual acuity of the left eye had decreased to 20/40, and there was increased proptosis (5.5 mm) and severe limitation of ocular movement (Fig. 3). Surgery was considered necessary to preserve vision and ameliorate the symptoms, therefore conservative debulking and orbital wall decompression were performed through a transconjunctival incision in the inferior fornix under general anesthesia.

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