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The Hidden Danger of Paraganglioma: Why Do Some Tumors Become Deadly?
Paraganglioma is a rare neuroendocrine tumor that can develop in different parts of the body, including the head, neck, chest, and abdomen. When this type of tumor is found in the adrenal gland, it is called a pheochromocytoma. The incidence of this type of tumor is estimated to be about 1 in 300,000 people. Since it is currently impossible to detect benign and malignant tumors, long-term follow-up of all patients with paraganglioma is necessary.
Signs and symptoms
Most paragangliomas are asymptomatic, often appearing as a painless lump or causing symptoms such as high blood pressure, palpitations, headaches, and rapid heartbeat. Although all of these tumors contain neurosecretory granules, secretion of hormones (such as catecholamines) is abundant enough to be clinically significant in only 1-3% of cases, and such conditions often mimic the presentation of pheochromocytoma.
Genetics
About 75% of paragangliomas are sporadic, and the remaining 25% are hereditary. These patients tend to have a higher incidence of tumors and earlier onset. It has been determined that mutations in genes affecting fibroblastic abnormalities, such as SDHD, SDHA, SDHC, and SDHB, are associated with the development of familial head and neck paragangliomas. Mutations in SDHB also play an important role in familial adrenal pheochromocytoma and paragangliomas of the abdomen and chest. The occurrence of these tumors may be related to a variety of genetic diseases, such as MEN 2A type and 2B type.
Pathology
On gross examination, paragangliomas appear as well-defined polypoidal masses that are firm to rubbery in texture, highly vascularized, and usually dark red in color. On microscopic examination, tumor cells formed polygonal to oval shapes, showed a characteristic cell ball (Zellballen) morphology, and were surrounded by fibrovascular stroma. The predominant cells in these spheroids will typically be positive for several markers, including specific markers for tryptamine precursors and neuroenzymes, which are critical in the differential diagnosis of tumors.
Origin site
About 85% of paragangliomas occur in the abdomen, only 12% in the chest, and only 3% in the head and neck (the latter is most likely to cause symptoms). Although most tumors are single, a few multiple cases occur in certain genetic syndromes. Paragangliomas are subdivided into different types based on their site of origin, with carotid paragangliomas being the most common. They usually present as a painless neck mass, but larger tumors may compress cranial nerves.
Diagnosis
The diagnosis of paraganglioma is usually confirmed with imaging techniques such as Gallium-68 DOTATATE PET/CT. These tumors originate from apigmented granule cells derived from the embryonic neural crest, making their association with pheochromocytoma even more obvious. Because of the unique properties of these cells, the World Health Organization considers them a category of neuroendocrine tumors.
Treatment
Treatment mainly relies on various methods such as surgery, embolization and radiotherapy. Treatment options will vary based on the patient's specific symptoms and the size and location of the tumor. Regardless, patience and continued medical monitoring are extremely important for this rare tumor.
Although paraganglioma appears as a rare tumor, it lurks health risks that cannot be ignored. Long-term follow-up and early diagnosis are crucial to improving the prognosis.
The insidious properties of paragangliomas have aroused widespread concern in the medical community. Are these tumors likely to worsen over time and become a fatal disease?
