Clinicopathological characteristics and outcomes of 42 cases of ocular adnexal sarcoma.

Abstract

Purpose: To study the clinicopathological features, treatment, and prognosis of ocular adnexal sarcomas diagnosed at a tertiary comprehensive hospital in China during 13 years.

Methods: A case series of patients with histopathological diagnoses of ocular adnexal sarcomas at a tertiary general hospital over 13 years. Medical records of demographic details, clinicopathological features, treatment, and prognosis at the time of presentation were reviewed.

Results: Forty-two cases of ocular adnexal sarcomas were found throughout the study period. The mean age was 37.1 years, with 27 males and 15 females. The most common clinical manifestation was exophthalmos (61.9%), and the median time to diagnosis was 6 months. These tumors most often occurred in the orbit (83.3%). The most common TNM stage at presentation was T2N0M0 (63.6%). Fifteen different histological types were identified; rhabdomyosarcoma accounted for the highest percentage (21.4%), followed by liposarcoma and malignant solitary fibrous tumor (16.7%). The diagnosis of each subtype depended on histomorphologic characteristics, supplemented by immunohistochemistry and molecular pathology. Most patients (56.8%) underwent multimodality therapy. Thirty-seven (88.1%) patients completed follow-up, while five patients were lost to follow-up. Of these 37 patients, 24 (64.9%) survived and 13 (35.1%) died; 19 (51.4%) experienced local recurrence, and 13 (35.1%) developed metastasis.

Conclusions: Ocular adnexal sarcomas are rare and histologically diverse, with rhabdomyosarcoma predominating in children and liposarcoma and malignant solitary fibrous tumor in adults. Middle-aged and elderly patients presenting with unilateral proptosis accompanied by visual impairment, especially in cases of cranio-orbital communication associated with headache, should raise suspicion for the possibility of malignant solitary fibrous tumor to avoid delayed diagnosis. Among liposarcomas, atypical lipomatous tumor/highly differentiated liposarcoma is more common. Accurate pathological diagnosis is crucial and requires a combination of histomorphologic, immunohistochemical, and molecular evaluation.