Paraganglioma

Have you been lately suffering from hearing pulsations in the ear? You need not panic about it although you should seek immediate treatment as you might be suffering from a peripheral nervous system disorder, known as Paraganglioma. Read and know all about the causes, symptoms, diagnosis and treatment of this disorder.

Paraganglioma Definition

These are non-cancerous tumors associated with Pheochromocytoma. Such rare growths originate in the exterior nerves of the spinal cord and brain. Although Pheochromocytoma and Paraganglioma are inter-linked, the former is far more common than the latter and is distinguished by the existence of large amounts of hormones known as catecholamines.

The growth is also known by the name “Glomus Jugulare Tumor”.

Paraganglioma Synonyms

Paraganglioma tumor is the universal term for a number of disorders, namely:

Picture of Paraganglioma

Picture 1 – Paraganglioma

  • Chemodectoma
  • Fibroangioma
  • Sympathetic nevi
  • Glomus tumor
  • Perithelioma

Paraganglioma ICD9 Code

The ICD9 code for this condition is 194.6.

Paraganglioma Incidence

This disease can occur at any age but usually arises in the later stage of life, when an affected individual is aged between 60 and 70 years. It occurs in approximately 1 out of every million people worldwide.

Paraganglioma Types

This abnormal growth generally originates in the abdominal area. In a few cases, it develops in the head, chest and neck region. On the basis of their site of growth, these tumors can be differentiated into various type like:

Symptomatic

Most growths of this type are symptomatic and commonly arise in the head region. These tumors are commonly found to arise on the neck of such patients.

Hereditary

These develop at a later age and comprise of several compounds.

Glomus jugulare

It generally occurs in the middle ear and causes Tinnitus along with a number of physical problems. Such tumors generally affect the ear drum of patients.

Pulmonary

It is generally present in the lung area.

Carotid

A painless mass on the neck represents carotid body Paraganglioma syndrome. However, big tumors can lead to cranial nerve palsies, typically of the hypoglossal and vagus nerve.

Vagal

It is indicated by indysphagia, along with hoarseness, and is manifested by the presence of a painless mass in the head and neck area.

Metastatic

It is an incurable form of Paraganglioma, giving rise to a tumor that can be partially removed by surgery. The main objective of any suggestive treatment for such disorders is to increase the life expectancy of the patient.

Extra Adrenal

Although these tumors can arise in patients at any age, individuals are generally affected by this syndrome during the fourth or fifth decade of their life span.

Gangliocytic

These are rare neoplasms occuring in the periampulary area. This disorder is benign in nature and can extend to nearby lymphatics.

Familial

These tumors develop at multiple sites at a younger age. Almost 10-50% of PGLs occurring among patients are Familial in nature.

Paraganglioma Causes

The real cause of this ailment is still unknown. However, 85% of the malignant cases of the disease affecting the inhabitants of the US are generally sporadic in nature.

Paraganglioma Symptoms

The symptoms of such benign slow growing tumors arise due to elevated secretion of norepinephrine or noradrenaline leading to very high blood pressure levels. The disorder is exhibited through a number of physical problems, including:

  • Pounding in the chest
  • Dizziness
  • Headches
  • Hearing problems
  • Rapid heartbeat
  • Hearing pulsations in the ear
  • Weakness of facial nerve palsy
  • Dysphagia ( swallowing difficulty)
  • Anxiety
  • Pain
  • Sweaty skin

Paraganglioma Diagnosis

The diagnosis of this disorder is analogous to pheochromocytomas. There are few recommended procedures which help to discover the existence of such tumors formed in the center of the adrenal gland, which is situated just above the kidney. The diagnosis is usually assumed by the existence of a painless adrenal mass, with the help of certain medical procedures which includes:

  • CT scan
  • MRI scan
  • Cerebral Angiography
  • Plasma-free fractionated metanephrines

Paraganglioma Treatment

This rare neuroendocrine neoplasm occurs at various parts of the body and is generally non-cancerous in nature. Avoiding treatment can be life-threatening for the patient. The treatment for this ailment depends upon its severity. Medical cure involves:

Surgery

Surgery is considered to be the ideal treatment for patients of this condition. Expert hand and neck surgeons (neurotologist) or neurosurgeons conduct the complicated surgery. However, embolization should be conducted prior to surgery to prevent excessive bleeding from the tumor during the treatment.

Radiation

In some cases, a small part of the tumor is left behind after surgery. Radiation therapy is highly recommended to remove such remaining fractions following operation.

Stereotactic Radiosurgery

This technique helps treat patients affected by glomus tumors.

Paraganglioma Prognosis

The condition generally has a good outcome. Patients who have undergone surgery or radiation as a part of their treatment experience a better life as compared to those who have encountered other forms of treatment. A large number of patients suffering from this order are completely cured via surgery.

Paraganglioma Complications

Complications are common among patients suffering from PGL. Health issues arise as a result of nerve damage which is quite common during surgery. This nerve damage can cause:

Image of Paraganglioma

Picture 1 – Paraganglioma Image

  • Voice change
  • Hearing loss
  • Swallowing problems

Paraganglioma is a life-threatening disorder. If proper medical treatment is not provided, the condition can give rise to a number of severe complications and eventually cause death of sufferers. Hence, if you notice a lump in your neck or experience any problem in your facial muscles, call your health care provider immediately.


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