【双语病例】颅咽管瘤 Craniopharyngioma

昵称42715024 2018-04-19

展开全文

History: A 12-year-old boy presents with a growth abnormality, having recently fallen off the growth curve and crossing multiple percentile lines. Further workup revealed a low T4 with normal thyroid stimulating hormone (TSH) and low insulin-like growth factor 1 (IGF-1) levels.

病史：12岁男孩，表现为生长异常，近期生长曲线下降。进一步检查发现，甲状腺素（T4）低，促甲状腺素（TSH）正常，胰岛素生长因子（IGF-1）低。

A brain MRI was obtained. Midline sagittal T1-weighted image, coronal T1- and T2-weighted images, axial fluid-attenuated inversion-recovery (FLAIR) and gradient-recall echo (GRE) images, and contrast-enhanced axial, coronal and sagittal T1-weighted images are shown below.

行脑MRI扫描，中线位置矢状T1WI、冠状T1WI/T2WI、轴位FLAIR/GRE、增强扫描（轴/冠/矢状）如下所示。

Findings

There is a sellar and suprasellar mass that predominantly demonstrates intermediate to high T1-weighted signal and high T2-weighted signal. A focal heterogeneous area of low T2-weighted signal is seen superiorly, with associated susceptibility, which likely represents calcification. The mass demonstrates patchy, nodular enhancement, particularly superiorly. There is mild associated mass effect, with compression of the pituitary gland inferiorly, and mild upward mass effect on the chiasm.

Differential diagnosis

Craniopharyngioma
Rathke cleft cyst
Pituitary adenoma
Meningioma
Teratoma
Arachnoid cyst

Diagnosis: Craniopharyngioma

影像表现：

蝶鞍及鞍上肿物，主要表现为T1WI等高信号，T2WI高信号，病灶上部信号不均，可见局灶性T2WI低信号区，具有相关磁敏感性，可能代表钙化。肿块内可见斑片状、结节样强化，主要位于病灶上部。病变有轻度的占位效应，垂体受压位于病灶下方，视交叉受压略上移。

鉴别诊断：

颅咽管瘤
Rathke裂囊肿
垂体腺瘤
脑膜瘤
畸胎瘤
蛛网膜囊肿

最后诊断：颅咽管瘤

Key points

Craniopharyngioma

Pathophysiology and epidemiology

Craniopharyngiomas are tumors arising from remnants of the craniopharyngeal duct.
They typically arise in a sellar/suprasellar location; however, they can arise anywhere along the infundibulum (from the third ventricle to the pituitary gland).
They have a bimodal age distribution, with a peak in ages 10 to 14, and a second peak in the fourth to sixth decades.
The two histological subtypes are adamantinomatous and papillary.

Adamantinomatous subtypes tend to affect primarily children, are predominantly cystic and lobulated, tend to encase vessels, and have calcification.
Papillary subtypes tend to affect primarily adults, are predominantly solid and more spherical, and are less likely to encase vessels or have calcification.

病理生理及流行病学：

颅咽管瘤一种起源于颅咽管残余的上皮细胞的肿瘤；

典型发病部位在蝶鞍及鞍上区，也可发生于沿漏斗走行区的任何部位（从三脑室至垂体）。

发病年龄有“双峰”分布的特征，一个高峰为10-14岁，另一个高峰为40-60岁。

两种组织学亚型：成釉质细胞型和鳞状乳头型。

成釉质型多见于儿童，主要表现为囊性、分叶状，可包埋血管，可见钙化；

鳞状乳头型多见于成人，主要表现为实性、球形，包埋血管及钙化少见。

Imaging appearance

Imaging hallmarks include calcification, cyst formation, and solid or nodular enhancement, with variations between subtypes as noted above.
The lesions are usually hyperintense on T2-weighted images, and they range from hypo- to hyperintense on T1-weighted images. T1 signal may be related to protein or cholesterol content.
Solid components tend to enhance.

影像表现：

特征性表现包括：钙化、囊变、实性或结节样强化，如上所述，上述特征因病理分型不同而有所变化。

病变在T2WI上常表现为高信号，T1WI上从低到高信号均可见，与肿瘤内含蛋白或胆固醇成分有关。

增强扫描实性部分有强化。

Clinical considerations

Patient presentation is variable and may include headaches, visual changes, endocrine abnormalities, and cognitive or motor deficits.

Management is with surgical resection, with the approach depending on the size and extent of the lesion.

Recurrence is common with the adamantinomatous subtype, but less common with the papillary subtype.

相关临床认识：

患者临床症状不同，可表现为头痛、视力改变、内分泌异常、认知或运动异常。

治疗方法为手术切除，具体术式依据肿瘤的大小及范围而定。

肿瘤复发在成釉质细胞型更为常见，而在鳞状乳头型相对少见。