Orbital pleomorphic sarcoma: A case report

Abstract

Aims/Purpose: To report a case involving an undifferentiated pleomorphic sarcoma localized in the orbit.Methods: Case report and literature review.Results: Undifferentiated pleomorphic sarcomas (UPS) are one of the least common types within all malignant sarcomas, with their incidence reaching 5–10% of the cases. Within this group of UPS, tumours involving the head and neck have an even lower incidence (approximately 3 to 5%). We report the case of an 86 year old male with a history of cardiovascular disease, presenting with a painless lesion in his right eye. The orbit CT reported a soft tissue lesion in contact with the right ocular globe, lacrimal gland and extraconal fat. At examination the patient registered best corrected visual acuity right eye (OD) no light perception right eye and left eye (OS) 20/50. At slit lamp examination OD is not evaluable due to corneal compromise. OS anterior segment and posterior pole with no alterations. Immunohistochemistry was negative for P40, desmine, CD34, SOX‐10 and cytokeratin cocktail. S100 had weak and patchy staining. These results led to a giant cell pleomorphic sarcoma diagnosis, histological grade 3. The patient underwent urgent orbital exenteration, with an adequate postsurgical evolution and oncology follow up.Conclusions: The diagnosis of an UPS is based on exclusion, due to their heterogeneous nature, with histological characteristics that overlap with other malignant mesenchymal neoplasms, mainly with metastatic sarcomatoid carcinoma and melanoma. These tumours are characterized by their rapid growth and potential for metastasis, especially in the lungs. Treatment includes surgical resection, sometimes combined with adjuvant chemotherapy. Strict periodic checkups are essential, since local recurrence is frequent (between 19 to 31%), with most cases occurring within the first 24 months.