An Unusual Cause of Proptosis: Orbital Metastasis of Paratesticular Leiomyosarcoma

Abstract

Paratesticular leiomyosarcomas are uncommon malignant tumors that originate from smooth muscle. Paratesticular leiomyosarcoma shows aggressive tumor behavior due to a high incidence of local recurrence and distant metastasis. The orbit metastasis is also rare and associated with a poor prognosis. Clinical suspicion plays an essential role in the diagnosis of orbital leiomyosarcoma due to uncommon presentation. Comprehensive neuroimaging and histopathological work-up warranted for definitive diagnosis and management. To date, surgical resection remains the best curative treatment. However, leiomyosarcomas are usually friable and unencapsulated, which makes surgery challenging. Also, whether adjunctive chemotherapy will alter the long-term prognosis remains to be determined. Herein, we report the case of a young male who presented with proptosis and decreased vision, subsequently diagnosed with metastatic orbital leiomyosarcoma from a primary paratesticular tumor. We performed orbital enucleation with negative margins. The patient is currently under systemic, systemic pazopanib chemotherapy.

Keywords

• unilateral proptosis
• orbital metastasis
• paratesticular leiomyosarcoma
• ocular enucleation
• neuroimaging