Primary pineal melanocytic tumor - Case report
Primary pineal melanocytic tumor - Case report
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DOI：
10.3171/jns.2001.94.3.0523
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发表时间：
2001-03-01
影响因子：
4.1
通讯作者：
Kojima, H
中科院分区：
文献类型：
--
作者：
Suzuki, T;Yasumoto, Y;Kojima, H
原发于松果体的黑素细胞病变是非常罕见的。作者报告了一例原发性松果体黑色素细胞瘤，并扩散到右侧海马，患者为一名50岁女性，表现为记忆障碍。磁共振成像（MR）显示一个肿块，在T-1加权像上呈高信号，在T-2加权像上呈低信号。松果体肿瘤经枕骨经小脑幕入路次全切除，病人接受全脑照射。组织学检查结果显示，肿瘤主要由不典型细胞组成，黑色素缺乏，并有坏死灶。肿瘤的强色素沉着区域含有与黑色素细胞瘤相似的分化良好的细胞。一项超微结构研究证实了成熟型黑素小体的证据。该患者在手术和放疗后11个月死亡（症状发作后1.7年）。尸检结果显示肿瘤通过软脑膜间隙和心室壁侵入实质。肿瘤被诊断为恶性，最后得出结论，肿瘤中的非典型细胞可能是罪魁祸首。这个松果体黑色素细胞肿瘤表现出广泛的分化，从高度恶性黑色素瘤到分化良好的黑色素细胞瘤，这可能是患者相对较长的生存期的原因。该肿瘤的生物学行为和形态学特征与其他松果体实质病变相似。
A primary melanocytic lesion arising from the pineal gland is very rare. The authors report a case of primary pineal melanocytic tumor with dissemination to the right hippocampus in a 50-year-old woman who presented with memory disturbance. Magnetic resonance (MR) imaging revealed a mass that was hyperintense on T-1-weighted and hypointense on T-2-weighted MR images. The pineal tumor was removed subtotally via the occipital transtentorial approach, and the patient underwent whole-brain irradiation. Results of histological examination revealed that the tumor predominantly consisted of atypical cells with scanty melanin pigment and some necrotic foci. The strongly pigmented areas of the tumor contained well-differentiated cells similar to those of melanocytoma. An ultrastructural study demonstrated evidence of a mature type of melanosome.The patient died 11 months after surgery and radiotherapy (1.7 years after the onset of symptoms). The autopsy findings demonstrated tumor invasion into the parenchyma through the leptomeningeal space and the ventricular wall. The tumor was diagnosed as being malignant, and it was finally concluded that the atypical cells in the tumor were probably responsible. This pineal melanocytic tumor exhibited a wide spectrum of differentiation, ranging from highly malignant melanoma to well-differentiated melanocytoma, which may have contributed to the patient's relatively long survival period. The biological behavior and morphological characteristics of this tumor appear to be similar to those of other pineal parenchymal lesions.