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What are paragangliomas/pheochromocytomas?

Paragangliomas and pheochromocytomas are tumors that develop out of the neuroendocrine tissue responsible for making epinephrine. This hormone, also known as adrenaline, and other related hormones help regulate heart rate and blood pressure in response to stress.Paragangliomas, known as PGLs, can develop anywhere in the body from the pelvis to the skull. Some release hormones that are similar to adrenaline and others do not.

在产生肾上腺素的肾上腺中特别发现嗜铬细胞瘤(PCC)。PCC分泌肾上腺素和去甲肾上腺素激素。PCC和PGL罕见,通常生长缓慢。大多数是良性的,但可能会引起高血压,出汗和头痛等问题。许多与遗传或遗传条件有关。遗传性副神经胶质细胞瘤是一种遗传性疾病,当良性肿瘤在paraganglia中生长时发生。

How we care for PGLs and PCCs

患有PGL和PCC的儿童在治疗达娜·法伯(Dana-Farber)/波士顿儿童癌症和血液疾病中心通过我们的Endocrine-Oncology Program。我们综合的儿科肿瘤科服务(在一个专业计划中)提供了领先的癌症中心和主要儿童医院的综合专业知识。我们组建了一个团队,以对待您的孩子,包括肿瘤学家,内分泌学家,遗传咨询师和外科医生。

Our areas of research for PGLs and PCCs

Children who are treated through our Endocrine-Oncology Program benefit from the work of our basic and clinical researchers, who are striving to understand the scientific causes of endocrine cancers. Their work can result in the introduction of new treatment options. We are a world leader in translational research, bringing laboratory advances to the bedside and into doctors’ offices as quickly as possible.

PGL和PCC的临床试验

Clinical trials,或评估新治疗方法的研究是Dana-Farber/Boston儿童的主要产品。对于许多患有罕见或难以治疗的儿童,临床试验提供了新的选择。

It’s possible that your child will be eligible to participate in one of our clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG). If your child has a progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups, or from one of our independent clinical investigators.

paraganglioma pheochromocytoma |症状和原因

paraganglioma/pheochromocytoma的症状是什么?

paraganglioma/pheochromocytoma(PCC和PGLS)的症状可能因儿童而异,并且取决于肿瘤的位置以及是否产生激素。症状可能模仿其他更常见的疾病。

对于PCC/PGL,最常见的症状是:

  • high blood pressure
  • 快速脉冲
  • 心pal
  • headache
  • dizziness
  • poor weight gain despite good appetite
  • abdominal pain
  • 恶心
  • vomiting
  • pale skin
  • clammy skin
  • sweating
  • growth failure

因为许多症状也可以指向其他疾病,所以生孩子很重要diagnosed立即由合格的医学专家。

是什么导致PCC/PGLS?

Sometimes, tumors emerge with no known cause. Some result from a mix of genetic and environmental factors, while others are linked to inherited conditions.

与PGL和PCC相关的疾病的示例包括遗传性副神经胶质瘤 - 细胞瘤综合征,neurofibromatosis,,,,von Hippel-Lindau disease,多内分泌肿瘤综合征,,,,tuberous sclerosis,,,,Sturge-Weber综合征,,,,and ataxia-telangiectasia.

paraganglioma pheochromocytoma |Diagnosis & Treatments

How are paraganglioma/pheochromocytoma diagnosed?

治疗孩子的第一步是形成准确而完整的诊断。您孩子的医师可能会订购许多不同的测试,包括:

您的医生可能会根据孩子的个人情况与您讨论其他诊断测试。在完成所有必要的测试之后,我们的专家开会以审查并讨论他们对您孩子状况的了解。然后,我们将与您和您的家人会面,讨论结果并概述最佳的治疗选择。

What are the treatment options for paraganglioma/pheochromocytoma?

paraganglioma或嗜铬细胞瘤的治疗将取决于孩子肿瘤的位置和类型。您孩子的医生可能建议手术,包括活检,腹腔镜检查或胸腔镜检查。Sometimes before removing a pheochromocytoma, your child’s physician may prescribe medicine to control high blood pressure.There are also some newer agents that are being investigated to reduce the need for big surgeries for advanced paragangliomas.

paraganglioma pheochromocytoma |程式& Services

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