Malpica Anais, Moran Cesar A
Department of Pathology, M D Anderson Cancer Center, Houston, TX 77030-4009, USA.
Ann Diagn Pathol. 2002 Oct;6(5):281-7. doi: 10.1053/adpa.2002.35739.
Two cases of primary primitive neuroectodermal tumors of the cervix are presented. The two female patients are 35 and 51 years of age who presented with abnormal uterine bleeding of several weeks' duration. On gynecologic examination, a mass in the cervical area was palpated and a biopsy was obtained. The initial biopsy was interpreted as possible small cell carcinoma in both women. A radical hysterectomy was performed in both patients. Grossly, in both cases, the uterine cervix showed an ill-defined tumor involving the ectocervix and endocervix, measuring 3.0 and 4.0 cm in greatest dimension, respectively, and showing areas of necrosis and hemorrhage. Histologic sections showed the presence of a malignant neoplasm arranged in cords and with a vague nesting pattern. Areas of hemorrhage and necrosis were also present. The neoplastic cells were characterized by having indistinct cell borders, small round to oval nuclei, and inconspicuous nucleoli. Mitotic figures were easily identified. In one patient, the tumor had metastasized to lymph nodes. Immunohistochemical studies revealed the neoplastic cells to be positive for antibodies for CD99 and focally for synaptophysin, while keratin, chromogranin, smooth muscle actin, desmin, and neurofilament protein were negative. Both patients received adjuvant chemotherapy and remain alive 5 and 18 months after initial diagnosis, respectively. The present cases highlight the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix.
本文报告了两例宫颈原发性原始神经外胚层肿瘤。两名女性患者年龄分别为35岁和51岁，均表现为持续数周的子宫异常出血。妇科检查时，在宫颈区域触及肿块并进行了活检。最初的活检结果在两名女性中均被解释为可能是小细胞癌。两名患者均接受了根治性子宫切除术。大体上，在这两例病例中，子宫颈均显示出边界不清的肿瘤，累及宫颈外口和宫颈内口，最大直径分别为3.0 cm和4.0 cm，并可见坏死和出血区域。组织学切片显示存在呈条索状排列且具有模糊巢状结构的恶性肿瘤。也有出血和坏死区域。肿瘤细胞的特征为细胞边界不清、核小而圆至椭圆形、核仁不明显。易见有丝分裂象。在一名患者中，肿瘤已转移至淋巴结。免疫组化研究显示肿瘤细胞CD99抗体阳性，突触素局灶性阳性，而细胞角蛋白、嗜铬粒蛋白、平滑肌肌动蛋白、结蛋白和神经丝蛋白均为阴性。两名患者均接受了辅助化疗，分别在初次诊断后5个月和18个月时仍存活。本病例突出了在宫颈小细胞肿瘤的鉴别诊断中考虑原始神经外胚层肿瘤的重要性。
