Paraganglioma is a rare type of tumor that arises from paraganglia, groups of neuroendocrine cells located along nerve pathways in various parts of the body, including the head, neck, chest, abdomen, and pelvis. These tumors can be either benign or malignant and may or may not produce hormones called catecholamines, which include adrenaline and noradrenaline. The signs of paraganglioma vary widely depending on the tumor’s location, whether it secretes hormones, and its size.
When paragangliomas produce excess catecholamines, they cause symptoms related to the overstimulation of the sympathetic nervous system. The most common signs include:
– **High blood pressure (hypertension):** This can be persistent or episodic, sometimes causing sudden spikes in blood pressure that can be severe and difficult to control.
– **Palpitations:** A sensation of rapid, pounding, or irregular heartbeat often accompanies the hypertension.
– **Excessive sweating (diaphoresis):** Patients may experience profuse sweating unrelated to heat or exercise.
– **Headaches:** These are often severe and throbbing, sometimes described as bursting or pounding.
– **Tremors:** Shaking or trembling, especially in the hands, can occur during episodes.
– **Pallor:** The skin may appear pale due to vasoconstriction caused by catecholamine surges.
– **Anxiety or panic-like attacks:** Sudden overwhelming feelings of fear or impending doom can mimic panic attacks.
– **Breathing difficulties:** Shortness of breath or a feeling of tightness in the chest may be present.
– **Nausea and weight loss:** Some patients report gastrointestinal symptoms and unintended weight loss.
– **Visual disturbances:** Blurred vision or other vision problems can occur during hypertensive crises.
These symptoms often occur in sudden episodes or “paroxysms,” lasting from minutes to hours, and may be triggered by physical activity, stress, changes in posture, or even urination if the tumor is located near the bladder.
In contrast, **non-secreting paragangliomas**, especially those located in the head and neck region, often do not produce catecholamines and therefore do not cause the classic symptoms related to hormone excess. Instead, their signs are usually related to the tumor’s size and location, causing a “mass effect.” This can include:
– **A visible or palpable lump:** Especially in the neck or near the carotid artery.
– **Hearing loss or tinnitus:** If the tumor is near the ear or auditory nerves.
– **Hoarseness or voice changes:** Due to involvement of the vagus nerve.
– **Difficulty swallowing or breathing:** If the tumor compresses the throat or airway.
– **Neurological symptoms:** Such as weakness, numbness, or pain if nerves are affected.
Rarely, paragangliomas can occur in unusual sites like the urinary bladder, where symptoms may include episodes of headache, sweating, palpitations, and fainting triggered by urination, known as “micturition attacks.”
Because paragangliomas are often slow-growing and rare, their symptoms can be mistaken for other conditions such as anxiety disorders, essential hypertension, or other tumors. The presence of the classic triad—palpitations, excessive sweating, and headache—especially when accompanied by high blood pressure, should raise suspicion for a catecholamine-secreting tumor like paraganglioma.
In some cases, patients may have no symptoms at all, and the tumor is discovered incidentally during imaging for unrelated reasons. However, even asymptomatic tumors can pose risks due to potential hormone secretion or local invasion.
Genetic factors also play a role, as many paragangliomas occur as part of hereditary syndromes, often presenting at a younger age and sometimes with multiple tumors. This genetic predisposition can influenc
