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Epithelioid sarcoma of the orbit.

Alkatan HM, Chaudhry I, Al-Qahtani A - Ann Saudi Med (2011 Mar-Apr)

Bottom Line: The classical "distal" form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk.In the orbit, only a single report of two cases, which had a typical histopathologic appearance, has been previously published.We present the third case of orbital primary epithelioid sarcoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, Oculoplastic and Orbital Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. jkatan@kkesh.med.sa

ABSTRACT
Epithelioid sarcoma is an aggressive and rare malignancy first recognized by Enzinger in 1970. It is known most commonly to affect the distal upper extremities in young adults. The classical "distal" form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk. The "proximal" variant of this tumor is deep seated, tends to occur in older patients and predominantly develops in the pelvis, perineum, and genital tract. In the orbit, only a single report of two cases, which had a typical histopathologic appearance, has been previously published. We present the third case of orbital primary epithelioid sarcoma.

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Focally positive tumor cells (cytokeratin, ×400)
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Figure 5: Focally positive tumor cells (cytokeratin, ×400)

Mentions: The histopathology showed a multinodular pattern of growth with large pleomorphic epithelioid cells, abundant cytoplasm, vesicular nuclei, and prominent nucleoli (Figure 1). The tumor nodules showed central necrosis with pseudogranulomatous appearance. Mitotic figures were frequent (Figure 2). There was strong staining for vimentin (Figure 3) and CD34 (Figure 4), in addition to moderate positivity with cytokeratin (Figure 5). Tumor cells were negative to desmin and myogenin.


Epithelioid sarcoma of the orbit.

Alkatan HM, Chaudhry I, Al-Qahtani A - Ann Saudi Med (2011 Mar-Apr)

Focally positive tumor cells (cytokeratin, ×400)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3102481&req=5

Figure 5: Focally positive tumor cells (cytokeratin, ×400)
Mentions: The histopathology showed a multinodular pattern of growth with large pleomorphic epithelioid cells, abundant cytoplasm, vesicular nuclei, and prominent nucleoli (Figure 1). The tumor nodules showed central necrosis with pseudogranulomatous appearance. Mitotic figures were frequent (Figure 2). There was strong staining for vimentin (Figure 3) and CD34 (Figure 4), in addition to moderate positivity with cytokeratin (Figure 5). Tumor cells were negative to desmin and myogenin.

Bottom Line: The classical "distal" form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk.In the orbit, only a single report of two cases, which had a typical histopathologic appearance, has been previously published.We present the third case of orbital primary epithelioid sarcoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, Oculoplastic and Orbital Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. jkatan@kkesh.med.sa

ABSTRACT
Epithelioid sarcoma is an aggressive and rare malignancy first recognized by Enzinger in 1970. It is known most commonly to affect the distal upper extremities in young adults. The classical "distal" form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk. The "proximal" variant of this tumor is deep seated, tends to occur in older patients and predominantly develops in the pelvis, perineum, and genital tract. In the orbit, only a single report of two cases, which had a typical histopathologic appearance, has been previously published. We present the third case of orbital primary epithelioid sarcoma.

Show MeSH
Related in: MedlinePlus