纳尔逊的综合症

Nelson's syndrome is a rare disorder that occurs in some patients with 库兴氏病 patients as a result of removing both adrenal glands. In Nelson's syndrome, the pituitary tumor continues to grow and release the hormone ACTH.

This invasive tumor enlarges, often causing visual loss, pituitary failure and headaches. One key characteristic of Nelson's disease is dark skin pigmentation, resulting from the skin pigment cells responding to the release of ACTH.

纳尔逊的综合症: Expert Care in Southern California

的 皇冠hga025大学洛杉矶分校垂体肿瘤项目 offers comprehensive management of Nelson's syndrome. 我们的医生 有多年的诊断经验吗, treating and managing pituitary conditions and treat a high volume of patients. 我们先进的诊断设备, team approach and minimally invasive surgical procedures make our program one of the best in the United States.

Use these links to explore more about Nelson's syndrome:

• 生理学
• 症状
• 诊断。
• 治疗
• 皇冠hga025

纳尔逊综合症:生理学

Nelson's syndrome can develop as a result of a specific treatment (bilateral adrenalecomy) for the pituitary disease called 库兴氏病. 的 harmful effects of 库兴氏病 are due to the excessive amount of the hormone cortisol produced by the adrenal glands.

治疗库欣氏病, your doctor may recommend removing the adrenal glands, 在一个叫做双侧肾上腺切除术的过程中. 的 procedure will stop cortisol production and provide relief. However, the procedure does not treat the actual tumor. 垂体瘤可发生快速生长.

In about 15-25 percent of patients who had a bilateral adrenalectomy, 纳尔逊综合症在一到四年内发病.

纳尔逊综合症:症状

的 most obvious symptom of Nelson's syndrome is the darkening of the skin color (hyperpigmentation).

Macroadenomas

大腺瘤是一种大的垂体肿瘤. 大的肿瘤会压迫周围的组织, primarily the normal pituitary gland and optic (visual) pathways, 引起的症状. 的 symptoms that result from the compression are independent of the effects of excess growth hormone secretion.
这可能会导致视力问题:

• 视力丧失. This occurs when macroadenomas grow upward into the brain cavity, compressing the optic chiasm.
• A loss of the outer peripheral vision, called a bitemporal hemianopsia 
  • 
    When severe, a patient can only see what is directly in front of them.
  • Many patients do not become aware of their visual loss until it is quite severe.
• 其他视力问题包括:
  • 视力下降(视力模糊), especially if the macroadenoma grows forward and compresses an optic nerve.
  • 颜色不像平常那样明亮

垂体功能衰竭或垂体功能减退

Increased compression of the normal gland can cause hormone insufficiency, called hypopituitarism. 症状取决于所涉及的激素.

纳尔逊综合症:诊断

Most patients with Nelson's syndrome have undergone a bilateral adrenalectomy for the treatment of 库兴氏病

诊断测试包括:

• 激素测试. 通常情况下，血液ACTH水平非常高. 了解更多关于 激素测试 在皇冠hga025大学洛杉矶分校脑垂体肿瘤项目工作.
• 核磁共振成像. Magnetic resonance imaging (MRI) scan of the pituitary gland can detect the presence of an adenoma, 垂体瘤.

纳尔逊综合症:治疗方案

Treating Nelson's syndrome effectively requires an experienced team of experts. 专家 at the UCLA's 垂体 Tumor Program have years of experience managing the complex coordination and care for treatment of Nelsons' syndrome.

治疗方案包括:

• 手术
• 放射治疗

纳尔逊综合症的手术

Surgical removal of the pituitary adenoma is the ideal treatment; however, it is not always possible. Surgical removal requires advanced surgical approaches, including delicate procedures involving the cavernous sinus.

If surgery is required, typically the best procedure is through a nasal approach. Our neurosurgeons who specialize in pituitary tumor surgery are experts in the minimally invasive expanded 内窥镜鼻内技术. This procedure removes the tumor while minimizing complications, hospital time and discomfort. This advanced technique requires specialized training and equipment.

Very large tumors that extend into the brain cavity may require opening the skull (craniotomy) to access the tumor. 我们的外科医生也是 微创“锁眼”开颅术利用隐藏在眉毛中的小切口.

纳尔逊综合症的放射治疗

放射治疗 can be effective in controlling the growth of the tumor. However, if you received radiation therapy in the past, additional radiation may not be safe.

Our 垂体 Tumor Program offers the latest in radiation therapy, including 立体定向放射治疗. This approach delivers a highly focused dose of radiation to the tumor while leaving the surrounding brain structures unharmed (with the exception of the normal pituitary gland).

One consequence of radiation treatment is that it can cause delayed pituitary failure. This typically occurs several years after treatment, and continued long-term follow-up with an endocrinologist is important. 你可能需要激素替代疗法.

纳尔逊综合症的药物治疗

Medical therapies for the treatment of Nelson's syndrome are currently limited, but include:

• Somatostatin-analogs(介绍). 这些药物通常用于治疗 肢端肥大症. A small number of Nelson's syndrome patients may respond.
• 卡麦角林. 这种药通常用于治疗 泌乳素瘤; you may require a very high dose.
• Temozolomide. This is a type of chemotherapy used to treat primary brain tumors called glioblastoma.

If you require medication to treat Nelson's syndrome, our endocrinologists will monitor you closely.

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To schedule an appointment with one of our physicians at the 垂体 Tumor Program, please call (310) 825 5111.