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Extended resection and intensity-modulated radiotherapy managed a rare primary orbit epithelioid sarcomaTreatment approach for a rare orbit tumor in a young man

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Key Takeaway
Note that extended resection and intensity-modulated radiotherapy were effective for this rare orbit epithelioid sarcoma.

This case report describes a rare instance of classic primary epithelioid sarcoma of the left orbit in a 25-year-old male. The diagnosis was confirmed through histology and immunohistochemistry showing a loss of nuclear INI1. This specific pathology is noted as a key reference for clinical management and pathological diagnosis of this tumor type, particularly within Asian populations.

The patient underwent extended tumor resection combined with local intensity-modulated radiotherapy. Follow-up at 7 months postoperatively showed no evidence of local recurrence or distant metastasis. The case highlights the role of immunohistochemistry in confirming the specific subtype of epithelioid sarcoma.

A primary limitation is the single-case study size, which means the findings cannot be generalized to a broader patient population. Larger series are required to confirm the efficacy of this management approach for orbit tumors. Clinical application should be interpreted with caution due to the rarity of the condition and limited sample size.

How this fits prior evidence

This case report addresses a gap in clinical documentation regarding orbital presentations of epithelioid sarcoma. It extends prior evidence regarding diagnostic challenges in epithelioid sarcoma by providing specific details on immunohistochemical confirmation (loss of nuclear INI1) and local management for an orbital tumor, whereas previous reports focused on soft-tissue masses of the thigh.

When a 25-year-old man presented with a tumor in his left orbit, doctors faced the challenge of treating an uncommon condition known as epithelioid sarcoma. This specific type of cancer is rare, making it difficult to establish standard treatment paths for every patient.

To manage the growth, the medical team performed an extended tumor resection followed by local intensity-modulated radiotherapy. These combined treatments were aimed at removing the primary tumor while targeting the area with precise radiation. Follow-up checks conducted seven months after the surgery showed no signs of the cancer returning or spreading to other parts of the body.

While this case provides a helpful roadmap for managing this rare tumor in the Asian population, it is important to note that this was a single-case report. Because only one patient was treated this way, more studies involving larger groups are needed to confirm if these results can be expected for every patient with similar symptoms.

What this means for you:
Surgery and targeted radiation successfully managed a rare orbit tumor in a 25-year-old male patient.

Common questions

What is epithelioid sarcoma of the orbit?

It is a rare type of cancer that can occur in the area around the eye. In this specific case, the tumor was confirmed through histology and special staining that showed a loss of nuclear INI1, which helps doctors identify this specific condition.

What treatment was used for this patient?

The patient underwent an extended tumor resection, which is a surgical removal of the growth. This was followed by local intensity-modulated radiotherapy to target the area with radiation and ensure the cancer was managed effectively.

Did the cancer return after treatment?

No local recurrence or distant metastasis was detected during the follow-up period of 7 months postoperatively. This indicates that the combination of surgery and targeted radiation was successful for this specific patient.

Study Details

Study typeSystematic review
EvidenceLevel 1
PublishedJun 2026
View Original Abstract ↓
BackgroundEpithelioid sarcoma is an exceedingly rare malignant soft tissue neoplasm with unclear histogenesis and distinctive epithelioid morphology. Primary epithelioid sarcoma of orbit represents an extremely rare clinical entity, with only isolated case reports documented globally. Notably, no formal, peer-reviewed case report of primary epithelioid sarcoma of orbit has been published in Asia to date.Case presentationA 25-year-old male patient presented with slowly progressive, painless proptosis of the left eye. Orbital MRI revealed an irregular mass in the superomedial extraconal space of the left orbit, with an initial radiological impression of vascular tumor or inflammatory lesion. Histologically, the tumor displayed characteristic multinodular growth, composed of a mixture of epithelioid and spindle cells with necrotizing granuloma-like changes. Skeletal muscle invasion and vascular tumor emboli were observed. Immunohistochemically, tumor cells co-expressed epithelial markers (broad-spectrum CK, EMA), mesenchymal marker (Vimentin) and CD34, with loss of nuclear INI1 (SMARCB1) expression. Whole-body PET-CT performed postoperatively excluded distant metastasis, and a diagnosis of classic primary epithelioid sarcoma of the left orbit was confirmed. The patient underwent extended tumor resection combined with local intensity-modulated radiotherapy. No local recurrence or distant metastasis was detected at 7 months postoperatively.ConclusionsPrimary epithelioid sarcoma of the orbit is diagnostically challenging, and pathological examination remains the gold standard for diagnosis. The combination of characteristic histological features and a distinct immunophenotype (loss of INI1) is critical for definitive diagnosis and differential diagnosis. We report the first case of primary epithelioid sarcoma of orbit in Asia, which enriches the clinicopathological data of this rare tumor in the Asian population and provides a key reference for its clinical management and pathological diagnosis. However, as a single-case study, larger series are needed to confirm generalizability.
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