概述
脊索瘤是一种罕见的骨癌，最初表现为脊柱或颅骨骨骼内细胞异常增殖形成的生长物。通常在脊柱上方的颅骨（颅底）或脊柱底部（骶骨）形成。
脊索瘤始发于出生前帮助形成脊柱的细胞。这些细胞通常会变成脊柱的椎间盘。如果这些细胞在婴儿出生时仍有残留，残留细胞通常会在婴儿出生后不久消失。但其中少数细胞有时会保留下来，并在罕见情况下会发展成癌细胞。
脊索瘤通常生长缓慢。脊索瘤可能很难治疗，因其在非常靠近脊髓和其他重要结构处生长，如动脉、神经或脑。，这种癌症通常会在治疗后复发。
Types
Different types of chordomas include:
- Conventional chordoma, also called classic chordoma and classical chordoma. This is the most common type. Under a microscope, it looks like bubbly cells in a slimy mix, similar to soap bubbles.
- Chondroid chordoma. Up to roughly 15% of chordomas are this type. Under a microscope, chondroid chordomas look similar to classic chordomas. But they also have tissue that looks similar to cartilage tissue found in parts of the body such as the nose.
- Dedifferentiated. Up to just under 10% of chordomas are this type. Under a microscope, this type of chordoma has two parts. One part looks like classic chordoma cells that also have a special protein called brachyury. The other part looks like a different type of cancer cell.
- Poorly differentiated. This rare type of chordoma mostly affects kids and young adults and is usually found in the skull base. It's identified due to a missing gene called SMARCB1 (INI1).
症状
脊索瘤的体征和症状可能包括：
- 头痛。
- 颈部疼痛。
- 视力问题，包括视力模糊、复视或视力丧失。
- 平衡问题。
- 听力减退。
- 面神经麻痹。
- 肌无力。
- 吞咽困难。
- 气短。
Skull base chordoma symptoms
The first signs and symptoms of a clival bone chordoma or a skull base chordoma may include:
- Headache.
- Facial numbness, weakness, drooping or paralysis.
- Vision trouble, including blurred or double vision or, sometimes, vision loss.
Other possible symptoms include:
- Hearing loss.
- Hoarseness.
- Trouble speaking as usual.
- Trouble swallowing.
Middle spine, also called mobile spine, chordoma symptoms
The first signs and symptoms of a chordoma in the part of the spine between the skull base and the sacrum may include:
- Arm or leg numbness or weakness.
- Back pain that may spread to the buttocks and legs.
- Breathing trouble.
- Headache.
- Neck stiffness or pain.
- Neck pain that may spread to the shoulders.
- Swallowing trouble.
- Walking trouble.
Bottom of the spine, also called sacral, chordoma symptoms
The first signs and symptoms of a sacral chordoma may include:
- Constipation.
- Lump at the site of the tumor.
- Numbness or tingling in the buttocks and inner thighs.
- Muscle weakness.
- Pain in the back, buttocks, pelvis or tailbone that may be worse when sitting.
- Sudden urge to urinate that's hard to control.
Because chordomas grow very slowly and have general symptoms caused by many things, it's common not to notice signs and symptoms for years.
何时就医
如有任何症状令您担心，请与医生或其他医疗护理专业人员约诊。
病因
大多数脊索瘤的病因尚不明确。这种癌症通常始发于颅骨或脊柱的骨骼。
骨骼中细胞的 DNA 发生变化时就会出现脊索瘤。细胞的 DNA 含有指示细胞工作的指令。在健康细胞中，DNA 会发出指令让细胞以一定的速度生长和繁殖。这些指令会指示细胞在设定的时间死亡。而在癌细胞中，DNA 的变化给出了不同的指令。这些变化指示癌细胞快速生长和增殖。癌细胞会在健康细胞本应死亡时继续存活。这会导致细胞过多。
癌细胞形成一个肿块，称为肿瘤。肿瘤可能生长，侵入并破坏健康的机体组织。随着时间的推移，癌细胞可能会脱离原发病灶并扩散至身体其他部位。当癌症病灶扩散时，称为转移性癌。
风险因素
可能增加罹患脊索瘤的风险因素包括：
- 年龄。 脊索瘤可能在任何年龄发生。不过这种癌症最常影响 40 到 60 岁的人群。
- 家族史。 有脊索瘤家族史者可能有更高概率患病。
并发症
Complications of chordomas typically are caused by the tumors growing and pressing into blood vessels, nerves and other tissues. Other complications happen if the cancer spreads to other parts of the body.
Chordoma complications of the nervous system may include:
- Cerebrospinal fluid leaks, though this is rare.
- Headaches.
- Hearing loss.
- Problems with balance, coordination and walking.
- Trouble swallowing or speaking.
- Trouble breathing.
- Vision problems.
- Water buildup on the brain, which can increase pressure inside the skull and cause headaches, nausea and vomiting.
- Weakness or numbness in the face, arms or legs.
Sometimes chordoma tumors can spread to other parts of the body. You may hear this called metastatic chordoma. Less than half of chordomas spread to other parts of the body. It's most common in chordomas that are found in areas of the spine below the neck and with the poorly differentiated type of chordoma. Sometimes metastatic chordoma may be called advanced chordoma or end-stage chordoma.
The most common place for a chordoma to spread is to the lungs. But it also can spread to the liver or other bones or soft tissues. Depending on where metastatic chordoma has spread, complications may include:
- Bone breaks.
- Extreme tiredness.
- Lumps.
- Pain.
- Swelling of the belly.
- Trouble breathing.
- Yellow skin or eyes, called jaundice.
- Weight loss.
Oct. 17, 2025