Zorludemir S, Scheithauer B W, Hirose T, Van Houten C, Miller G, Meyer F B
Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Am J Surg Pathol. 1995 May;19(5):493-505.
Since clear cell meningioma has only recently been recognized as a morphologic entity, its pathobiology has not been studied. Fourteen examples occurring in seven females and six males, ages 9 to 82 years (mean 29 years), were examined; one was associated with type 2 neurofibromatosis. Of these cases, seven (50%) were spinal-intradural (six lumbar, one thoracic), three (21%) arose in the posterior fossa (cerebellopontine angle), three (21%) were supratentorial, and one (7%) was centered upon the foramen magnum. In one case (8%), two tumors were considered to be independent primaries. One tumor (8%) appeared to show no dural attachment. Thirteen tumors were subject to complete study. All were composed of sheets of clear, glycogen-rich, polygonal cells forming only a few vague whorls. Hyalinization, both stromal and perivascular, was often extensive. Mitoses were rare in primary tumors. Immunohistochemistry showed vimentin and epithelial membrane antigen staining to be reactive in 100%. Stains for S-100 protein and CAM 5.2 were negative. Progesterone and estrogen receptor staining was observed in 77% and 0%, respectively. Ultrastructural study showed abundant cytoplasmic glycogen, a few cytoplasmic lumina, intermediate filaments, interdigitation of cell membranes, and desmosomal junctions. The means, medians, and ranges of proliferating cell nuclear antigen (PCNA) and MIB-1 antigen labeling indices for nonrecurring and recurring tumors were 10.4%, 8.8%, 0.8-23.4% and 11%, 1.4%, 0.1-50.3%, as compared with 7.4%, 6.7%, 2.9-17.2% and 13.3%, 13.4%, 3.3-25.7%, respectively. Twelve successful DNA ploidy studies showed that 11 tumors (85%) were diploid and one was tetraploid; percentage S-phase determinations varied from 4 to 9% (mean 6.0%). Recurrence was noted in eight patients (61%) (five of whom had multiple recurrences); there was local discontinuous spread in two cases (15%) and widespread cranial to spinal metastasis in one case (8%). Three patients (23%) are dead of disease. In summary, clear cell meningiomas are morphologically unique, show no sex predilection, affect primarily the lumbar region and cerebellopontine angle, and despite their benign appearance, may be inordinately aggressive, particularly intracranial examples. No close association was noted between recurrence or clinical outcome and such factors as mitotic activity, PCNA proliferation indices, percent S-phase determination, or DNA ploidy status. In contrast, MIB-1 proliferation indices were appreciably higher among recurring tumors.
由于透明细胞型脑膜瘤直到最近才被确认为一种形态学实体，其病理生物学尚未得到研究。我们检查了发生在7名女性和6名男性中的14个病例，年龄在9至82岁之间（平均29岁）；其中1例与2型神经纤维瘤病相关。在这些病例中，7例（50%）为脊髓硬膜内肿瘤（6例位于腰椎，1例位于胸椎），3例（21%）起源于后颅窝（桥小脑角），3例（21%）为幕上肿瘤，1例（7%）以枕骨大孔为中心。在1例（8%）中，两个肿瘤被认为是独立的原发肿瘤。1例肿瘤（8%）似乎未显示与硬脑膜相连。13个肿瘤进行了完整的研究。所有肿瘤均由成片的透明、富含糖原的多边形细胞组成，仅形成少数模糊的漩涡状结构。基质和血管周围的玻璃样变常常广泛存在。原发性肿瘤中罕见有丝分裂。免疫组织化学显示波形蛋白和上皮膜抗原染色均呈100%阳性反应。S-100蛋白和CAM 5.2染色均为阴性。孕激素和雌激素受体染色分别见于77%和0%的病例。超微结构研究显示有丰富的细胞质糖原、少数细胞质腔、中间丝、细胞膜的交错和桥粒连接。非复发性和复发性肿瘤的增殖细胞核抗原（PCNA）和MIB-1抗原标记指数的均值、中位数和范围分别为10.4%、8.8%、0.8 - 23.4%和11%、1.4%、0.1 - 5
