A case of progressive exophthalmos in a 5-year-old girl

Spindle cell sarcoma is a rare tumor, with little presentation in the literature. Soft tissue tumors constitute a heterogeneous group, requiring a multidisciplinary approach.

5-year-old schoolboy who goes to his pediatrician for exophthalmos. His mother refers a progressive proptosis in the left eye of 6 months of evolution. No loss of visual acuity, in the last weeks intermittent pain with eye movements. On examination proptosis of the left eye with intermittent tearing and vascular lesion in the temporal portion of the same eye, he presented restriction of left eye movements. He was referred to the hospital emergency department where a head CT scan (Figure 1,2) was performed, revealing a unilateral left retroorbital lesion measuring 28 x 14 mm, causing proptosis, mass effect on retroocular structures and loss of ocular sphericity. Given the findings in the imaging tests, it was decided surgical intervention by means of frontotemporal craniotomy, subsequently receiving complementary treatment with local radiotherapy. Due to negative molecular pathology studies, the result was finally interpreted as undifferentiated 'adult-type' spindle cell sarcoma.

Although undifferentiated spindle cell sarcoma is a rare pathology, it should be included in the differential diagnosis of retro-orbital pathologies. The objective of this case is to review the main characteristics of spindle cell sarcoma, given its rapid and aggressive form of presentation, in a rare tumor.