Malignant granular cell tumor of orbit: pathological features and literature review

Abstract

Abstract Orbital granular cell tumor is a rare tumor, and malignant granular cell tumor of the orbit is rarely reported.This paper reports a case of malignant granular cell tumor in the right eye of a 57-year-old man. The onset of the disease was right eye pain with subacute dry eye, and progressive right eye movement disorder, right eye deviation, and decreased visual acuity.The gross examination showed an unshaped nodule of 3.5cm×2cm×1cm. The section was gray and medium in quality.Microscopically, the boundary of the tumor was still clear, slightly nodular, with abundant cytoplasm, epithelioid in some areas, and spindle shaped tumor cells in some areas. The nuclei were enlarged with vesicular nuclei, prominent nucleoli, mitotic figures > 2/10 HPF, and tumor necrosis was seen locally .Immunohistochemical staining showed that tumor cells were positive for S100, SOX10, CD56, CD68, Calretinin, weakly positive for TFE-3, and H3K27Me3 and INI-1 were not absent.Other indicators MiTF, HMB45, MelanA, PCK were negative, and Ki67 proliferation index was 10% in the hot spot area.Orbital malignant granulosa cell tumor is very rare. Understanding its pathological features and correct diagnosis is very important for the treatment and prognosis evaluation of patients.