[Epithelioid sarcoma of the orbit].

BACKGROUND

Sarcomas count among the very rare malignancies of the orbit. Epithelioid sarcomas typically occur in the subcutaneous tissue of younger patients; an affected orbit constitutes a rarity.

OBJECTIVES

Only three cases of this extremely rare disease of the orbit, which is associated with marked aggressiveness and a very poor prognosis, have been analysed in the worldwide current literature.

PATIENT AND METHODS

We present a case report together with a literature review. A rapidly progressing painful proptosis with visual loss occurred in a 30-year-old patient. Imaging and invasive diagnostic procedures were initiated as a consequence of this.

RESULTS

Diagnostic imaging revealed an infiltrative process of the right orbit. Following endonasal decompression of the orbit and probe acquisition, histological and immunohistochemical tests showed the presence of a proximal type epithelioid sarcoma. Therapy consisted of orbit exenteration together with implantation of titanium miniplates for orbital prosthesis. During the course of disease, osseous metastases developed. The patient died during palliative chemotherapy, 14 months after the initial diagnosis.

CONCLUSION

Epithelioid sarcomas are extremely rare, difficult to treat tumours in the head and neck region. The associated mortality rates are high. Aggressive surgical therapy with intensive follow-up is recommended. The prognosis depends upon the resection status.