Pulmonary paraganglioma is extremely rare benign lung tumor. Paraganglioma are tumors originating from parasympathetic nerve ganglions. Usually paraganglions are present in head, neck, mediastinum of lung, bladder etc. Pulmonary paraganglioma occur in the lung or in the midiastinum (space between two lungs). The tumor can be primary which has its origin from the lung itself. Secondary pulmonary paraganglioma have its origin somewhere else in the body and they metastasize in lungs.
Paraganglioma are neuroendocrine tumors. They develop as a single tumor or may be a portion of endocrine tumor. Scientists are not able to find the exact cause of pulmonary paraganglioma.This tumor remains undetected as it does not produce any symptoms in majority of cases. However, symptoms when present are cough, pain in chest and fever. Large tumors can cause obstruction in the bronchi.
The usual treatment is to resect the tumor surgically. Since the tumor is benign after its removal prognosis is favorable.
Pulmonary Paraganglioma Symptoms
Pulmonary paraganglioma usually are solitary tumors. They are benign and asymptomatic in majority of cases. They may originate in the lung itself or may be secondary from its origin somewhere else in the body. Paraganglioma in lungs can be in the lung itself or in the space between two lungs known as the mediastinum.
Rarely the tumor compresses or invades the air passage causing breathing problems. They grow very slowly. Although asymptomatic, some large tumor can compress the bronchi and produce cough, chest pain and blood in sputum. Since the tumor are neuroendociral in nature, it may produce too much of norepinephrine hormone in the body giving rise to high blood pressure.
Pulmonary paraganglioma usually occurs in adults. It is mainly found in middle aged men. Most females suffering from this tumor are known to suffer from associated multiple endocrinal neoplasia, a type of tumor pertaining to the endocrines such as the thyroid gland or adrenal gland. This tumor in women develops in early age.
Diagnosis Of Pulmonary Paraganglioma
There are many tests that will help the physician to diagnose pulmonary paraganglioma.
- CT scan, MRI, and X-ray of chest.
- Blood tests to detect elevation of epinephrine hormone.
- Lung function test.
- Cytology examination of sputum.
- Biopsy of the lung tissue.
Tissue biopsy is the definitive test that will detect the mass in lung is pulmonary paraganglioma. Tissue biopsy helps to differentiate the disease from many other kinds of disease such as lung cancer, tuberculoma of lung, metastatic tumor in lung, lung carcinoid tumor etc.
Management And Treatment Of Paraganglioma
Complete removal of the pulmonary paraganglioma is the first option if the tumor is single. In case if the tumor is secondary from its origin somewhere else in the body, than some physicians also recommend radiation therapy. External beam radiotherapy is sensitive in killing the mass and some doctors prefer it as the first choice of treatment. Many consider it when the local disease has progressed or the tumor starts enlarging in size.
There is no unanimous decision yet for chemotherapy as the treatment choice for pulmonary paraganglioma. After the surgery or radiation patient needs to follow regular check up with his doctor. The prognosis of pulmonary paraganglioma is good after removing the tumor.