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From silence to surprise: How does paraganglioma lurk in your body?
Paraganliomas are rare neuroendocrine tumors that can develop in different parts of the body, including the head, neck, chest, and abdomen. When this tumor occurs in the adrenal gland, it is called a pheochromocytoma. It is estimated that the incidence of this tumor is about 1 in every 300,000 people. Because there are no tests to determine benign versus malignant tumors, long-term follow-up is recommended for all patients with paraganglioma.
Signs and symptoms
Most paragangliomas are asymptomatic, usually presenting as a painless lump or causing symptoms such as high blood pressure, tachycardia, headaches and palpitations.
Although these tumors all contain neurosecretory granules, secretion of hormones such as adrenaline is sufficient to be clinically significant in only 1-3% of cases; in such cases, symptoms are usually similar to those of pheochromocytoma.
Genetics
About 75% of paragangliomas are sporadic, while the remaining 25% are hereditary and more likely to occur multiple times and at an early age. Mutations in genes such as SDHD, SDHA, SDHC, and SDHB have been identified as causing familial head and neck paraganglioma. In particular, mutations in SDHB play a key role in familial adrenal pheochromocytoma and paraganglioma of the abdomen and chest.
Pathological characteristics
Paraganliomas usually appear as round, nodular masses that are often firm or rubbery in texture. These tumors are highly vascular and may be dark red in color.
Under microscopic examination, tumor cells can be easily identified. They are usually polygonal to oval in shape and arranged in characteristic cell spheres (Zellballen) separated by fibrovascular stroma and surrounded by supporting cells.
Origin site
About 85% of paragangliomas occur in the abdomen; 12% occur in the chest, and 3% occur in the head and neck (which are most likely to cause symptoms). These tumors are generally named after the site of origin, although some specific paragangliomas have special names:
- Head and neck paraganglioma (HNPGL): There are different types depending on the exact location, the most common being the carotid paraganglioma, which usually presents as a painless lump in the neck.
- Aortic bifurcation paraganglioma: These tumors develop in the abdomen at the bifurcation of the aorta and are usually harmless but may be a common origin of abdominal paragangliomas.
- Pulmonary paraganglioma: These tumors may appear in the lungs as single or multiple tumors.
Diagnosis
The diagnosis of paraganglioma is usually confirmed by imaging studies, and Gallium-68 DOTATATE PET/CT imaging is often used to confirm the presence of a paraganglioma. In the World Health Organization classification of neuroendocrine tumors, paragangliomas are classified as tumors of the neural cell lineage.
Treatment methods
The main treatments for paraganglioma include surgery, embolization, and radiotherapy. The specific treatment plan depends on many factors, including the patient's symptoms and the size and location of the tumor.
How to prevent paraganglioma from lurking silently in your body?
