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The Mystery of Tumors: Do You Know What a Paraganglioma Is?
Paraganliomas are rare neuroendocrine tumors that can develop in different parts of the body, including the head, neck, chest, and abdomen. When this type of tumor occurs in the adrenal glands, it is called a pheochromocytoma. The incidence of these tumors is very rare, estimated to be approximately 1 in 300,000 people. Because there are currently no tests that can definitively distinguish benign from malignant tumors, long-term follow-up is recommended for all patients with paraganglioma.
Symptoms and signs
Most paragangliomas are usually asymptomatic and present as a painless mass. As the tumor grows larger, it may cause symptoms such as high blood pressure, a fast heartbeat, headaches, and palpitations. Although all paragangliomas contain neurosecretory granules, only 1–3% of cases secrete enough hormones such as epinephrine to cause clinically relevant symptoms; this often mimics the presentation of pheochromocytomas.
About 75% of paragangliomas are sporadic; the remaining 25% are hereditary and are more likely to be multiple and to present at a younger age.
Genetic factors
The genetic characteristics of paraganglioma cannot be ignored. Studies have shown that certain gene mutations such as SDHD, SDHA, SDHC and SDHB are associated with familial and multiple paragangliomas. Mutations in these genes are thought to be important in the development of head and neck paragangliomas. These tumors may also occur in multiple endocrine neoplasia (MEN) types 2A and 2B.
Pathological features
On microscopic examination, paraganglioma cells are usually polygonal to oval in shape and are arranged in characteristic cell clusters called Zellballen. These cell clusters are surrounded by a fibrovascular stroma and have supporting cells. By immunohistochemical techniques, tumor cells show specific markers, such as staining for granin, synaptophysin and neurotransmitters.
Paraganlioma cells are often misdiagnosed on light microscopy as other related neuroendocrine tumors, such as carcinoid tumors, neuroendocrine carcinomas, and medullary carcinoma of the thyroid gland.
Origin site
About 85% of paragangliomas occur in the abdomen, with only 12% occurring in the chest and 3% in the head and neck area. Although most cases are single, a few multiple cases occur in specific genetic syndromes. Depending on where they originate, paragangliomas may be named different types, such as cervical paragangliomas and cranial nerve paragangliomas.
Diagnostic Procedures
The diagnosis of paraganglioma usually relies on imaging tests, such as Gallium-68 DOTATATE PET/CT scan, to confirm the presence of the tumor. These tumors arise from nerve cells involved in the sympathetic nervous system and are specifically classified in the World Health Organization's classification of neuroendocrine tumors.
Treatment methods
The treatment methods for paraganglioma mainly include surgery, embolization and radiotherapy. The specific treatment method depends on many factors, including the patient's symptoms and the size and location of the tumor.
Although the diagnosis and treatment of paraganglioma are challenging, the prognosis for patients is expected to improve with the advancement of medical technology.
The discussion of paraganglioma not only reveals the complexity of this rare tumor, but also makes us think about what new hope the future development of tumor research may bring to patients?
