J Korean Ophthalmol Soc.  2010 Oct;51(10):1403-1408.

Recurrent Alveolar Soft-Part Sarcoma With Concurrent Involvement of the Orbit and Multiple Sites of the Body

• Kim JW
• Cho YK
• La TY

Abstract

PURPOSE
To report a rare case of recurrent alveolar soft-part sarcoma (ASPS) with concurrent involvement of the orbit and multiple sites of the body that was removed successfully by surgery.
CASE SUMMARY
A 37-year-old woman presented with diplopia at the left lateral gaze and proptosis of the left eye. Two years earlier, the patient had a mass excision of the right gluteus maximus and the left orbit, and ASPS was histopathologically diagnosed at that time. In addition, the patient had been treated with chemotherapy and radiation therapy. On radiologic examination, recurrent tumor of the left orbit was found and surgically removed. The histopathologic examination showed that tumor cells were arranged in an alveolar pattern divided by fibrous septa and contained abundant granules in the cytoplasm, typically consistent with ASPS. Postoperatively, the symptoms of diplopia and proptosis improved.
CONCLUSIONS
ASPS can occur and recur in the orbit as well as systemically. In addition, at the time of surgical removal of ASPS in the orbit, the surgeon should be particularly cautious of massive bleeding.

Keyword

Bleeding; Body; Orbit; Recurrent alveolar soft part sarcoma

MeSH Terms

Adult
Cytoplasm
Diplopia
Exophthalmos
Eye
Female
Hemorrhage
Humans
Orbit
Sarcoma, Alveolar Soft Part
Viperidae

Figure

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  Figure 1. (A) Pelvic MRI shows a large lobulated hypervascular soft tissue mass in the right gluteus maximus muscle about 11.8×9.3 cm in diameter. (B) Brain CT shows bone destructions and formations of the left orbit and sphenoid bone with contrast enhancing of a soft tissue mass (arrow) in the left orbit.

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  Figure 2. Chest CT shows multiple small nodules (arrows) in both lungs.

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  Figure 3. (A) Preoperative brain MRI shows left orbital roof destruction with intraorbital invasion of a soft tissue mass. (B) Post-operative brain MRI shows that the part of the left Laserassistedlateral orbital wall, and intraorbital mass were removed.

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  Figure 4. Patient shows proptosis of the left eye with periorbital ecchymosis and conjunctival injection (A: Frontal view, B: Chin up view).

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  Figure 5. Preoperative facial CT and 3 dimensional CT shows well enhanced soft tissue mass in the left orbit and zygomatic bone area. CT also shows mass invasion adjacent to the orbital apex and unclear outline of the left lateral rectus muscle. (A: Transverse section without contrast enhancement (CE), B: Transverse section with CE, C: Coronal section with CE, D: 3 dimensional view).

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  Figure 6. (A) Through lateral canthotomy and conjunctival incision, a dark red-colored, capsulated soft tissue mass (arrow) was found underneath. (B) An almost totally exposed mass is seen. (C) Removed mass was 4×3×2.5 cm in size. (D) Photograph of post-operative 1 month shows improved proptosis.

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  Figure 7. The tumor cells were arranged in an alveolar pattern divided by fibrous septa (A, H & E stain, ×100) and contained abundant granular cytoplasm (B, H & E stain, ×400). PAS positive intracytoplasmic crystal-like granules were observed character-istically (C, PAS stain, ×400).

Reference

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