介绍:
《On call 36小时》女主角的脊髓星形细胞瘤是什么？
Spinal astrocytoma
脊髓星形细胞瘤
Spinal astrocytomas are the second most common spinal cord tumour overall, representing 40% of intramedullary tumours . They account for 60% of paediatric intramedullary tumours, making them the most common spinal cord tumour in children .
脊髓星形细胞瘤是（成人）脊髓肿瘤中第二常见的肿瘤（第一是室管膜瘤），占髓内肿瘤的40%。脊髓星形细胞瘤占小儿髓内肿瘤的60%，是儿童最常见的脊髓肿瘤。
Epidemiology
流行病学
The peak incidence of spinal astrocytomas occurs in the third decade, with the mean age at presentation being ~ 30 years. Males are somewhat affected more commonly than females (M:F = 3:2) .
脊髓星形细胞瘤的发病高峰大约在三十几岁的时候，平均发病年龄为30岁。男性比女性更容易受到影响(男性：女性=3：2)。
Associations
相关性
There is an increased incidence in neurofibromatosis type 1.
1型神经纤维瘤病的发病率增加。
Clinical presentation
临床表现
Clinical presentation is similar to that of other intramedullary spinal tumours, with pain, weakness and sensory changes common. Bowel and bladder dysfunction are uncommon.
临床表现与其他髓内脊柱肿瘤相似，常见疼痛、无力和感觉改变，少见肠和膀胱功能不全。
Pathology
病理
Astrocytomas (both intracranial and spinal) arise from astrocytic glial cells. Spinal cord astrocytomas generally have a lower histologic grade than astrocytomas in the brain (see grading of astrocytomas):
星形细胞瘤(颅内和脊髓)起源于星形胶质细胞。脊髓星形细胞瘤的组织学分级通常低于颅内星形细胞瘤(详见星形细胞瘤分级)。
adults
olow-grade: 75%
oanaplastic astrocytoma: 25%
oglioblastoma: ~1%
paediatric
oin children <3 years, 80% are grade I or II
成人
o低级别：75%
o间变性星形细胞瘤：25%
o多形性胶母细胞瘤: ~1%
儿童
o3岁以下的儿童患者，80%是I 级或II级
All astrocytomas are characterised by hypercellularity and the absence of a surrounding capsule. In contrast to cord ependymomas, a cleavage plane is not present in most intramedullary spinal astrocytomas.
所有星形细胞瘤的特征都是多细胞性和乏包膜。与脊髓室管膜瘤相比，髓内星形细胞瘤不存在卵裂平面。
High grade tumours are more likely to demonstrate extensive leptomeningeal spread, seen in up to 60% of spinal cord glioblastomas .
高级别的肿瘤更有可能表现出广泛的软脊膜扩散，在60%的脊髓胶质母细胞瘤中可以看到这一现象。
Radiographic features
影像学特征
The most common location of astrocytomas is the thoracic cord (67%), followed by the cervical cord (49%), and tumour may of course involve both regions. Involvement of the entire spinal cord (holocord presentation) may occur and is more common in children than in adults. Isolated conus medullaris involvement is seen only rarely (3%) and involvement of the filum terminale is rare (whereas this is the typical location of myxopapillary ependymomas).
星形细胞瘤最常见的部位是胸椎（67%），其次是颈髓（49%），也可能同时累及两个区域。可能会发生整个脊髓受累(全皮质表现)，而且在儿童中比在成人中更常见。孤立性延髓圆锥受累罕见(3%)，终丝受累较少(而这是粘液囊性室管膜瘤的典型位置。)。
Astrocytomas are typically long multisegment intramedullary masses that cause diffuse cord expansion. The average length of involvement is 4-7 vertebral body segments .
星形细胞瘤是一种典型的长节段髓内肿块，可引起弥漫性脊髓扩张。平均受累长度为4-7椎体节段。
Plain radiograph/CT
平片/CT
Can be often normal however /as these tumours are slow growing bony remodelling is not infrequently visible, with posterior vertebral body scalloping or thinning of the pedicle or laminae. Scoliosis is also present in a reasonable number of patients, especially in children presenting with holocord involvement . These features are however less common than in spinal ependymomas.
由于这些肿瘤生长缓慢，骨重塑并不少见，椎弓根或椎板后椎体皱缩或变薄往往是正常的。脊柱侧凸也存在于一定数量的患者中，尤其是在全脑皮质受累的儿童。然而，这些特征并不如脊柱室管膜瘤常见。
On CT expansion of the cord is frequently visible, but due to the lower contrast resolution compared to MRI can be subtle. If contrast is administered contrast enhancement is frequently visible.
脊髓的扩张很常见，但由于对比度分辨率比MRI低，在CT上表现可能很细微，对比增强之后则通常可见。
Myelography
脊髓造影
May show non specific multisegmental cord enlargement, and may results in a block to normal flow of contrast past the lesion, although this is more common with ependymoma.
可能显示非特异性多节段脊髓扩张，并可能导致造影剂流经病变区域时受阻，尽管这在室管膜瘤中更常见。
MRI
As astrocytomas arise from cord parenchyma (c.f. ependymomas that arise in the central canal), they typically have an eccentric location within the spinal cord.
由于星形细胞瘤起源于脊髓实质(注意对比出现在中央管的室管膜瘤)，它们通常位于脊髓内偏心部位。
They may be exophytic, and even appear largely extramedullary. They usually have poorly defined margins. Peritumoral oedema is present in ~40%. Intratumoural cysts are present in ~20% and peritumoral cysts are present in ~15% . Unlike ependymomas, haemorrhage is uncommon.
它们可能呈外生性生长，甚至大部分在髓外，通常边界不清。瘤周水肿出现在约40%的病例。瘤内囊肿约20%，瘤周囊肿约15%。与室管膜瘤不同的是，星型细胞瘤出血少见。
Reported signal characteristics include
T1: isointense to hypointense
T2: hyperintense
T1 C+ (Gd)
ovast majority enhance (used to thought that all enhance but this is not the case )
ousually patchy enhancement
预警信号特征包括
T1：等或低信号
T2：高信号
T1 C+ (Gd)
o绝大多数增强（过去认为都会增强，但事实并非如此）。
o通常呈片状强化
Treatment and prognosis
治疗和预后
Astrocytomas are generally faster growing than ependymomas and typically have a worse prognosis.
星形细胞瘤的生长速度通常比室管膜瘤快，而且预后更差。
Surgical excision is usually the treatment of choice, however due to the infiltrative nature of astrocytomas, resection is almost always histologically incomplete.
手术切除是治疗首选，但由于星形细胞瘤的浸润性质，很难从组织学上完全切除。
Cord astrocytomas in children tend to be associated with a good prognosis, as they behave much like grade I cerebellar pilocytic astrocytomas and displace neural tissue rather than infiltrate it.
儿童脊髓星形细胞瘤往往预后良好，因为它们表现得很像Ⅰ级小脑毛状胶质星形细胞瘤，取代神经组织而不是浸润它。
Differential diagnosis
鉴别诊断
The main differential diagnosis is a spinal ependymoma. Certain imaging features may help to differentiate between the two, which are covered in this article:
ependymoma
omore common in adults
oscoliosis and bony remodelling more common
ocentral location in spinal canal
owell-circumscribed
ohaemorrhage is common
may rarely present as a subarachnoid haemorrhage
haemosiderin staining especially at the superior and inferior margins (so-called haemosiderin capping) is common
ofocal, intense homogeneous contrast enhancement
omore frequent and more prominent cysts (intratumoral and polar)
主要鉴别诊断为脊髓室管膜瘤。某些成像特征可能有助于区分这两者，本文将对两者进行区分。
室管膜（细胞）瘤
o在成年人中更常见
o脊柱侧凸和骨重塑更常见
o椎管中央位置
o边界清楚
o出血很常见
可罕见地表现为蛛网膜下腔出血
血黄素染色，特别是在上、下缘(所谓的血黄素盖)是常见的。
o聚焦、强均匀对比增强
o更频繁和更突出的囊肿(瘤内和两极)
分享范子妤的一封信
“小时候觉得做医生好伟大，既可以医好人的病，又可以医好他们的创伤，但是当我做了医生以后我才发现，有时候医生可以做的事实在是太少太少了，有时我会觉得很迷惘，医生的责任是什么？医生每天都要不停抢救病人，但死亡是每个人都逃避不了的结局，医生做的一切到底还有没有意义？
这一刻我都很怀疑自己到底专不专业，做医生每天都要面对无数生死，很多人都以为我们已经习惯了、麻木了，但其实我们真的很怕面对失败，也不知道应该怎么去面对。失败以后要去承受最后的悲痛，承受失去家人的悲痛的是他们的家属，可是看到他们哭的死去活来，其实我们的心里不会比他们好过，究竟医生的使命是什么呢？背起医生的身份，就要肩负起救人的责任，和死神搏斗抢救生命，每打赢一场仗，救回一条命，我都会觉得很开心，会因为自己的医学知识帮到人而骄傲，可是越做的久，我就越怀疑，医学真的只是为了抢救生命？科技不断进步，也拖长的病人的死亡过程，延长了病人的痛苦，很多病人最终还是救不回来，我们做医生的又应该怎样去面对？
两三千年前，医学之父希波克拉底讲过：不是每个病人都能救得活，没有人可以逃避死亡，可是在病人离世之前，医生尽全力让他们舒服安详地渡过最后的日子，就是对病人最大的帮助，对家属最大的安慰。这就是医生的使命。”
以及范子妤因为自己患有脊髓星型细胞瘤，而不接受张一健求婚时，一键的誓词：
“结婚的誓词，如果只是循例念出来是没有意义的，必须实践出来。年轻时漂亮健康，当然爱，但当对方年纪渐大，又老又有皱纹，病痛越来越多，甚至眼看对方老死，难道就不爱了吗？既然将来不会离弃对方，现在也应该一样，如果因为对方有病就不爱他，就不是真正的爱。没人知道生命的长短，即使我们是医生，也不能保证自己长命百岁，所以结婚誓词没说要保证白头偕老，却要保证无论健康疾病都要在一起。范子妤，我再问你一次，无论健康疾病，你愿意和我不离不弃，终生不渝吗？”
（资料来源：radiopaedia；『the hippocratic crush』台词；中文为讲者自译，不当之处敬请指正。）
Bgm-连续剧（粤）
撰稿讲解：福建医科大学 淑铉铉铉
