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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

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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Figure 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.

Mentions: A 48 year old male patient presented at our clinic complaining of proptosis and lid swelling in his left eye that had persisted for 2 weeks. The patient had no significant past history or family history associated with ocular disease, trauma, or tumors. Although no palpable mass was noted, exophthalmometry measurements were 20 mm on the left, and 18 mm on the right with a base of 104 mm. Motility was slightly limited in adduction. Results of ocular examination including visual acuity, color vision, and funduscopy were normal. The thyroid function test was also normal. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with adjacent invasion of the left orbit and compression of the left medial rectus muscle (Fig. 1). Magnetic resonance imaging (MRI) showed that the signal intensity of the mass was low on a T1-weighted image with contrast enhancement and isointense to muscles on a T2-weighted image. The lesion extended into the left orbit with compression of the left medial rectus muscle, and there was also intracranial extension with compression of left side frontal lobe (Fig. 2).


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)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 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.
Mentions: A 48 year old male patient presented at our clinic complaining of proptosis and lid swelling in his left eye that had persisted for 2 weeks. The patient had no significant past history or family history associated with ocular disease, trauma, or tumors. Although no palpable mass was noted, exophthalmometry measurements were 20 mm on the left, and 18 mm on the right with a base of 104 mm. Motility was slightly limited in adduction. Results of ocular examination including visual acuity, color vision, and funduscopy were normal. The thyroid function test was also normal. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with adjacent invasion of the left orbit and compression of the left medial rectus muscle (Fig. 1). Magnetic resonance imaging (MRI) showed that the signal intensity of the mass was low on a T1-weighted image with contrast enhancement and isointense to muscles on a T2-weighted image. The lesion extended into the left orbit with compression of the left medial rectus muscle, and there was also intracranial extension with compression of left side frontal lobe (Fig. 2).

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