Response to immunotherapy in a patient with advanced epithelioid sarcoma of adrenal gland: A case report

Abstract

Epithelioid sarcoma (ES) is a rare and highly invasive soft tissue malignant tumor with uncertain histogenesis. The localization of ES in the adrenal gland is rather unusual. The present study reported a case of stage IV ES in the adrenal gland of a 28-year-old male. The tissue biopsy of adrenal and lung lesions revealed epithelioid cells. Immunohistochemistry indicated that the tumor cells were strongly positive for cytokeratin (CK)8, CK, epithelial membrane antigen (EMA), CD34 and programmed cell death protein 1 ligand (PD-L1) but negative for nuclear integrase interactor 1 expression. The next-generation sequencing technology was applied using peripheral blood, indicating a low tumor mutation burden of 4.11 mutations (Muts)/Mb and somatic mutations in SMARCB1 . After diagnosis, the patient underwent unsuccessful palliative chemotherapy and radiotherapy. However, application of immune checkpoint inhibitors (ICIs) achieved partial remission and the overall survival reached 25 months. ICI monotherapy may be a feasible treatment for patients with ES with a strong expression of PD-L1.

Epithelioid sarcoma

“…Case reports of the efficacy of pembrolizumab in advanced ES in adults have also been published. Pembrolizumab was used in the second line of palliative therapy, after chemotherapy with doxorubicin-ifosfamide [77]. In the study of nivolumab, a 24-year-old male ES metastatic lung patient had PR after 4 immunotherapy cycles, but the response was not durable as the patient progressed after the next 4 cycles [78].…”

Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report

“…With regard to immunotherapy using immune checkpoint inhibitors (ICIs), there have been some case reports demonstrating a response ranging from partial remission to complete remission. These case reports used anti-PD1 ICIs such as pembrolizumab or nivolumab, or anti-CTLA-4 ICIs such as ipilimumab [31,32]. However, another retrospective analysis from a tertiary care center in India found that immunotherapy in two patients did not have any objective response [33].…”