Pineal parenchymal tumor of intermediate differentiation:diagnostic pitfalls and discussion of treatment options of a rare tumor entity
Pineal parenchymal tumor of intermediate differentiation:diagnostic pitfalls and discussion of treatment options of a rare tumor entity
复制标题
松果体实质中间分化肿瘤：罕见肿瘤实体的诊断陷阱和治疗选择的讨论
作者：
C. Senft;A. Raabe;E. Hattingen;D. Sommerlad;V. Seifert;K. Franz
松果体区域的肿瘤并不常见，约占欧洲和美国成人颅内肿瘤的0.4-1%。从组织病理学上看，它们是一组异质性很强的肿瘤。在真正的松果体肿瘤中，中间分化的松果体实质肿瘤（PPTIDs）是最不常见的类型。在本文中，我们报告了一个病人的情况与意想不到的和难以诊断的PPTID。在第三脑室后部发现一个2.2 × 2.2 cm中线肿块，伴有连续阻塞性脑积水，患者为44岁男性，表现为复视和步态障碍。肿瘤与松果体没有明确的联系。鉴别诊断包括所有脑室内和中线肿瘤，因此采取活检。初步组织病理学诊断为生殖细胞瘤或原始神经外胚层肿瘤，组织样本由参考神经病理研究所重新检查。最终诊断为PPTID。然后通过经脑室/经巩膜入路切除肿瘤。肿瘤标本的组织病理学检查证实了PPTID的诊断。术后，患者接受伽玛刀放射治疗。在1年的随访中，没有肿瘤再生的迹象。一般的松果体实质肿瘤，尤其是pptid的诊断是很麻烦的。它们的组织病理学特征仍在定义中，不同肿瘤实体的生物学行为也是如此。因此，包括手术、放射治疗和化疗在内的治疗选择仍然存在争议。我们建议手术切除PPTID，最好是在肿瘤大小允许的情况下全部切除。
Tumors of the pineal region are uncommon, comprising approximately 0.4–1% of all intracranial tumors in adults in European and American series. Histopathologically, they are a very heterogeneous group of tumors. Of genuine pineal tumors, pineal parenchymal tumors of intermediate differentiation (PPTIDs) are the least frequently found type. In this paper, we report on the case of a patient with an unexpected and difficult-to-diagnose PPTID. A 2.2 × 2.2-cm midline mass within the posterior part of the third ventricle with consecutive obstructive hydrocephalus was found in a 44-year-old man presenting with diplopia and gait disturbances. There was no clear connection of the tumor to the pineal gland. Differential diagnosis included all intraventricular and midline tumors, therefore a biopsy was taken. Preliminary histopathological diagnosis was germinoma or primitive neuroectodermal tumor, and the tissue sample was reexamined by a referential neuropathological institute. Final diagnosis was PPTID. The tumor was then resected through a transventricular/transchoroidal approach. Histopathological examination of tumor specimen confirmed the diagnosis of a PPTID. Postoperatively, the patient received gamma-knife radiosurgery. At 1-year follow-up, there are no signs of tumor regrowth. Diagnosis of pineal parenchymal tumors in general and PPTIDs in particular can be troublesome. Their histopathological features are still being defined, as is the biological behavior of the different tumor entities. Thus, treatment options including surgery, radiation therapy, and chemotherapy remain controversial. We recommend surgical removal of PPTID, preferably in toto whenever the size of the tumor permits that kind of excision.