Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-8555, Japan.
Pathol Int. 2010 Mar;60(3):241-4. doi: 10.1111/j.1440-1827.2009.02509.x.
Extraspinal ependymoma is a rare tumor, occurring most commonly in the sacrococcygeal region, and only a small number of cases have been reported to arise in the uterine ligament. Herein is reported a case of extraspinal ependymoma arising in the broad ligament of a 27-year-old woman. The lesion was 14 cm in diameter with an intra-abdominal implant in the omentum. On cut section the tumor was found to be solid, and demonstrated hemorrhaging, necrosis, myxoid foci, and central cystic spaces. Microscopically the tumor was composed of a proliferation of short spindle or polygonal cells arranged in short fascicles or in a solid sheet-like fashion with occasional perivascular pseudorosettes, together with myxoid areas and variable histological architectures exhibiting cribriform, pseudopapillary, and variable-sized cystic patterns. On immunohistochemistry most tumor cells were positively reactive to glial fibrillary acidic protein (GFAP), CD99, estrogen receptor, and progesterone receptor. The patient has remained disease-free for 6 months after the adjuvant chemoradiotherapy. Extraspinal ependymoma should be considered as a differential diagnosis when examining unusual intrapelvic tumors, especially in young female patients. The identification of characteristic histological features such as perivascular pseudorosettes and immunohistochemical expression of GFAP are helpful for confirming the diagnosis.
椎管外髓内瘤是一种罕见的肿瘤，最常发生于尾骨-骶骨区域，仅有少数病例报道发生于子宫韧带。本文报告了一例发生于阔韧带的椎管外髓内瘤，患者为 27 岁女性。病变直径 14cm，在大网膜上有腹腔内种植。切面见肿瘤实性，伴出血、坏死、黏液样灶和中央囊性区。镜下肿瘤由短梭形或多边形细胞增生组成，呈短束状或实性片状排列，偶见血管周围假菊形团，伴黏液样区和不同组织学结构，呈筛状、假乳头状和不同大小的囊性模式。免疫组化染色显示大多数肿瘤细胞弥漫阳性表达胶质纤维酸性蛋白（GFAP）、CD99、雌激素受体和孕激素受体。患者在辅助放化疗后 6 个月无疾病进展。当检查不常见的盆腔内肿瘤时，应考虑椎管外髓内瘤作为鉴别诊断，特别是年轻女性患者。血管周围假菊形团等特征性组织学特征和 GFAP 的免疫组化表达有助于确诊。
