论文部分内容阅读
研究背景发生于垂体的混合性神经节细胞瘤-垂体腺瘤临床罕见,由于其缺乏特征性影像学表现,易误诊为垂体腺瘤,是术前诊断鞍区肿瘤的难点。本文回顾分析1例鞍区混合性神经节细胞瘤-生长激素垂体腺瘤患者的诊断与治疗经过,结合文献对此类临床少见垂体肿瘤的临床病理学特征进行分析,以期提高诊断与鉴别诊断能力。方法与结果女性患者,28岁,临床主要表现为反复头痛伴视物模糊8月余,以及肢端肥大和闭经表现。头部CT和MRI显示鞍内和鞍上不规则占位性病变,呈T1WI等或稍低信号、T2WI稍高信号,增强扫描病灶呈明显不均匀强化,边界清晰,压迫视交叉和第三脑室底部。手术全切除肿瘤。组织学形态观察,肿瘤组织分为两部分结构,一部分为不规则簇状排列的神经节细胞样细胞分布于神经纤维背景中,另一部分为片状排列或局部乳头状结构的圆形和卵圆形细胞,两部分结构相互混杂。免疫组织化学染色,神经节细胞样细胞区域肿瘤细胞胞质突触素(Syn)呈弥漫性强阳性,腺垂体激素呈阴性;圆形细胞区域肿瘤细胞胞质Syn呈弥漫性阳性,约30%肿瘤细胞生长激素呈阳性,其余神经垂体激素呈阴性。最终病理诊断为(鞍区)混合性神经节细胞瘤-生长激素垂体腺瘤(WHOⅠ级)。术后未予放射治疗,随访1年,临床症状明显改善,肿瘤未复发。结论鞍区混合性神经节细胞瘤-垂体腺瘤临床罕见,鉴于目前大多支持该肿瘤是起源于垂体干/祖细胞、具有内分泌细胞和神经元双向分化的独立肿瘤实体,故建议采用“伴神经节细胞分化的垂体腺瘤”的诊断术语,应注意与两种独立肿瘤形成的碰撞瘤相鉴别。
Research Background Occur in the pituitary mixed ganglioneuroma - pituitary adenoma is rare clinical, because of its lack of characteristic imaging findings, easily misdiagnosed as pituitary adenoma, preoperative diagnosis of sellar tumors is difficult. This paper retrospectively analyzed one case of mixed ganglioneuroblastoma - growth hormone pituitary adenoma diagnosis and treatment of patients, combined with the literature on the clinical features of these rare clinical pituitary tumors were analyzed in order to improve the diagnostic and differential diagnosis ability . Methods and Results Female patients, 28 years old, the main clinical manifestations of repeated headache with blurred vision more than August, and acral hypertrophy and amenorrhea manifestations. Head CT and MRI showed irregular space occupying lesion in the saddle and saddle, showing T1WI or slightly lower signal, T2WI slightly higher signal, enhanced scanning lesions were significantly uneven enhancement, clear boundary, oppression of the optic chiasm and the third ventricle bottom. Surgical removal of the tumor. Histological observation showed that the tumor tissue was divided into two parts. One part was irregularly arranged ganglion cell-like cells distributed in the nerve fiber background, and the other part was round and oval Cells, two parts of the structure mixed with each other. Immunocytochemical staining showed that the cytoplasmic synaptophysin (Syn) in the ganglion cell-like cell region was strongly diffuse and the adenohypophyseal hormone was negative. The cytoplasmic Syn in the round cell area was diffusely positive, and about 30% Tumor cell growth hormone was positive, the rest of the neurohypophysial pituitary hormone was negative. The final pathology was diagnosed as a mixed ganglioneuroblastoma (GHG), a growth-hormone pituitary adenoma (WHO grade I). No radiation after surgery, followed up for 1 year, clinical symptoms improved significantly, the tumor did not relapse. Conclusions Mixed ganglioneuroma-pituitary adenoma is rare in the sellar region. Given that most of the current support for this tumor is an independent tumor entity that originates from pituitary stem / progenitor cells and has bidirectional differentiation of endocrine cells and neurons, it is recommended to use “companion Ganglion cell differentiation of pituitary adenomas, ”the diagnosis of terminology, should pay attention to the formation of two separate tumor cells and the differential diagnosis.