Pusztaszeri Marc, Pica Alessia, Janzer Robert
Pathology Department, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Neuropathology. 2006 Apr;26(2):153-7. doi: 10.1111/j.1440-1789.2006.00657.x.
Pineal parenchymal tumors (PPT), including pineoblastomas, are very uncommon, especially in adults. Because of the small number of reported cases, the histological and biological features of these tumors are still being defined, as is their optimal management. Also, the pathological variability of these tumors makes it difficult to draw general conclusions about their behavior. We report the case of a PPT of intermediate differentiation (PPTID) with an excellent outcome. A 3.2 cm tumor in the pineal and tectal region with obstructive hydrocephalus was found in a 28-year-old female complaining of headaches and hypoesthesia of the right side of the face. Stereotactic biopsy revealed a highly cellular tumor composed of small cells that contained little cytoplasm that were arranged in a diffuse pattern. Mitotic activity was low and there was no evidence of necrosis. Immunohistochemical examination demonstrated positive staining for neuron-specific enolase and synaptophysin. There was no expression of neurofilaments or GFAP. Ki-67 proliferation index was 12%. The diagnosis was a PPTID. Treatment consisted of combined neoadjuvant chemotherapy and craniospinal radiotherapy. Six years after treatment, the patient is alive and free of any clinical or radiological signs of relapse. This paper discusses the pathological and biological features of these tumors and the treatment options available.
松果体实质肿瘤（PPT），包括松果体母细胞瘤，非常罕见，尤其是在成人中。由于报告的病例数量较少，这些肿瘤的组织学和生物学特征仍在确定中，其最佳治疗方法也是如此。此外，这些肿瘤的病理变异性使得难以对其行为得出一般性结论。我们报告一例分化中等的松果体实质肿瘤（PPTID），预后良好。一名28岁女性因头痛和右侧面部感觉减退就诊，在松果体和顶盖区发现一个3.2 cm的肿瘤并伴有梗阻性脑积水。立体定向活检显示肿瘤细胞高度密集，由小细胞组成，胞质很少，呈弥漫性排列。有丝分裂活性低，无坏死证据。免疫组化检查显示神经元特异性烯醇化酶和突触素呈阳性染色。神经丝或GFAP无表达。Ki-67增殖指数为12%。诊断为PPTID。治疗包括新辅助化疗和全脑全脊髓放疗联合应用。治疗六年之后，患者存活，无任何临床或影像学复发迹象。本文讨论了这些肿瘤的病理和生物学特征以及可用的治疗选择。
