Materiały źródłowe

• #1 Hereditary paraganglioma-pheochromocytoma: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/hereditary-paraganglioma-pheochromocytoma/

  Hereditary paraganglioma-pheochromocytoma is an inherited condition characterized by the growth of tumors in structures called paraganglia. […] People with hereditary paraganglioma-pheochromocytoma develop one or more paragangliomas, which may include pheochromocytomas. […] Pheochromocytomas and some other paragangliomas are associated with ganglia of the sympathetic nervous system. […] These excess catecholamines can cause signs and symptoms such as high blood pressure (hypertension), episodes of rapid heartbeat (palpitations), headaches, or sweating. […] Most paragangliomas are associated with ganglia of the parasympathetic nervous system, which controls involuntary body functions such as digestion and saliva formation. […] However, large tumors may cause signs and symptoms such as coughing, hearing loss in one ear, or difficulty swallowing.

• #2 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal paraganglia. While akin to pheochromocytomas, they manifest diverse symptoms contingent on their location. Typically found along the sympathetic chain, they often secrete catecholamines, causing hypertension and tachycardia. Parasympathetic variants, located along cranial nerves, are usually non-secretory. Though mostly benign, malignancy can occur, underscoring the importance of early detection and treatment. […] Associated symptoms are episodic hypertension, tachycardia, headache, and diaphoresis. […] The most common feature of catecholamine excess is hypertension, with episodes that can be continuous or intermittent and often paroxysmal. These episodes commonly present with the classic associated symptoms of headache, palpitations, and profuse sweating, known as „the classic triad.”

• #3 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paraganglioma can occur at any age, but most commonly affects people between the ages of 20 and 50. While very rare, the illness often causes a range of symptoms that when recognized can help with diagnosis. Many of these symptoms can be caused by multiple other conditions as well. Some paragangliomas do not cause any symptoms. […] Signs or symptoms of paragangliomas may include: Hypertension, Sustained hypertension, Paroxysmal hypertension, Orthostatic hypotension, Headache, Palpitations+/-tachycardia, Diaphoresis/sweating, Anxiety, Swelling at tumor site. […] Less common signs or symptoms may include: Pallor, Flushing, Hyperglycemia, Vomiting, Abdominal/back pain, Nausea, Fatigue, Dyspnea, Dizziness, Visual symptoms. […] The signs and symptoms of paraganglioma can be caused by a number of different conditions.

• #4 Paraganglioma – Rare Cancers Australia

  https://www.rarecancers.org.au/knowledgebase/cancer-types/paraganglioma/

  Paragangliomas are most commonly diagnosed in adults between the ages of 30 and 50. […] Sympathetic paragangliomas may present with some of the following symptoms: Hypertension, headaches, unexplainable sweatiness, tachycardia, shakiness, being very pale, anxiety, heart palpitations. […] Not everyone with the symptoms above will have cancer but, see your GP if you are concerned.

• #5 Paraganglioma | Altru Health System

  https://www.altru.org/health-library/conditions/paraganglioma

  Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis. […] Symptoms of a paraganglioma in the head or neck can include: A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus. Trouble swallowing. Hoarse voice. Hearing loss. Blurred vision. Dizziness. […] Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures. […] When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone. […] Symptoms of paragangliomas that make hormones include: High blood pressure. Feelings of having a fast-beating, fluttering or pounding heart. The sudden loss of color in the face. Sweating. Headache. Uncontrolled shaking in the hands or arms. General weakness. […] These symptoms may come and go. […] Some people with paragangliomas don’t have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

• #6 Paraganglioma | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/paraganglioma/

  A paraganglioma is a rare type of tumour that forms along major blood vessels and nerves. […] In many cases, paragangliomas may not cause any symptoms and are found when a test or procedure is done for another reason. Sometimes they can cause signs and symptoms when: […] They grow large enough to press on other organs or spread to other organs. For example, if the tumour has spread to the bones, this may cause bone pain. […] They release hormones called catecholamines into the bloodstream. Adrenaline and noradrenaline are two types of catecholamines. The release of these extra catecholamines into the blood can cause sweating, a racing heartbeat, feelings of anxiety, headache, and high blood pressure. These symptoms can be similar to panic attacks. These signs and symptoms may occur at any time or be brought on by certain events, such as intense physical activity, having an operation or childbirth. […] Paragangliomas are usually slow-growing tumours. The outcome depends on whether they are benign or malignant and how far the tumour has spread. However, it is possible for patients with treatment to live for many years beyond their original diagnosis.

• #7 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Not all paragangliomas release hormones, meaning some are asymptomatic. Those that develop in the head and neck usually don’t release hormones, but if they grow big enough, they can obstruct swallowing, cause coughing or lead to hearing loss. […] Other symptoms of paragangliomas may include: Gastrointestinal issues, including constipation and dry mouth, Chest pain and other cardiac effects of hormone surges, Hoarseness, nerve paralysis or dilated pupils from a mass in the head or neck, Generalized fatigue, Blurry vision, Weight loss, Excessive thirst or hunger, Lightheadedness. […] If the paraganglioma is spreading to other parts of the body, it’s known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] In about 10 percent of cases, paragangliomas have already spread by the time they’re diagnosed, according to Seminars in Oncology. Metastasis is more likely for paragangliomas that are discovered outside of the adrenal glands and for those that are larger than 5 centimeters (cm).

• #8 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Paragangliomas typically start out as benign (not cancerous) tumors, but they may cause symptoms that may become life-threatening. Benign paragangliomas may also become cancerous later and spread to other parts of the body (a process called metastasis). […] Paragangliomas release hormones that activate the body’s fight-or-flight response. Called catecholamines, these hormones are usually released in response to stress. A paraganglioma can release these hormones at will, sometimes in large doses. […] These hormones may lead to symptoms that include: High blood pressure, Flushing, Palpitations, Sweating, Headache, Shaking. […] These symptoms may feel like a panic or anxiety attack. […] These attacks, if they become severe, can cause a catecholamine crisis, which may result in a heart attack, stroke or severe high blood pressure.

• #9 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  Signs and symptoms of paraganglioma happen when the tumor releases too much adrenaline or noradrenaline into your blood. However, some paraganglioma tumors dont make extra adrenaline or noradrenaline and dont cause symptoms (are asymptomatic). Common symptoms of paraganglioma include episodes of: […] High blood pressure (hypertension). […] Headaches. […] Excessive sweating for no known reason. […] A pounding, fast or irregular heartbeat. […] Feeling shaky. […] Some people who have a paraganglioma may experience symptoms infrequently or in bursts. […] Paragangliomas usually grow very slowly. But this could vary from case to case. […] If paragangliomas are left untreated, whether benign or malignant, they can potentially cause serious, life-threatening complications due to the excess amounts of adrenaline and noradrenaline they can secrete. Complications can include:

• #10 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paraganglioma can occur at any age, but most commonly affects people between the ages of 20 and 50. While very rare, the illness often causes a range of symptoms that when recognized can help with diagnosis. Many of these symptoms can be caused by multiple other conditions as well. Some paragangliomas do not cause any symptoms. […] Signs or symptoms of paragangliomas may include: Hypertension, Sustained hypertension, Paroxysmal hypertension, Orthostatic hypotension, Headache, Palpitations+/-tachycardia, Diaphoresis/sweating, Anxiety, Swelling at tumor site. […] Less common signs or symptoms may include: Pallor, Flushing, Hyperglycemia, Vomiting, Abdominal/back pain, Nausea, Fatigue, Dyspnea, Dizziness, Visual symptoms. […] The signs and symptoms of paraganglioma can be caused by a number of different conditions.

• #11 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. […] Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events. […] The most common sign is high blood pressure that may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death. […] Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumors are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason. […] Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have: High blood pressure. Headache. Heavy sweating for no known reason. A strong, fast, or irregular heartbeat. Being shaky. Being extremely pale.

• #12 Paraganglioma – Information – Oncology Hematology Care – OHC

  https://ohcare.com/condition/paraganglioma/

  Paragangliomas are rare tumors that form near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. They form outside the adrenal gland. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of these extra catecholamines into the blood may cause signs or symptoms of disease. […] Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. […] Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have any of the following: High blood pressure, Headache, Heavy sweating for no known reason, A strong, fast, or irregular heartbeat, Being shaky, Being extremely pale. […] The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death.

• #13 Pheochromocytoma and Paraganglioma Syndromes | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/pheochromocytoma-paraganglioma-syndromes

  Symptoms of pheochromocytoma and paraganglioma syndromes include: […] High blood pressure/hypertension (most common) […] Headache […] Heavy sweating for no reason (hyperhidrosis) […] Strong, fast, or irregular heartbeat […] Shakiness […] Extreme paleness (pallor). […] Sometimes pheochromocytoma and paraganglioma syndromes release extra neurohormones, adrenaline (epinephrine) and noradrenaline (norepinephrine), into the blood and cause signs or symptoms of disease.

• #14 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1–5

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4532956/

  The paraganglioma (PGL) syndromes types 15 are autosomal dominant disorders characterized by familial predisposition to PGLs, phaeochromocytomas (PCs), renal cell cancers, gastrointestinal stromal tumours and, rarely, pituitary adenomas. […] The clinical manifestations of these syndromes are protean: patients may present with features of catecholamine excess (including the classic triad of headache, sweating and palpitations), or with symptoms from local tumour mass, or increasingly as an incidental finding on imaging performed for some other purpose. […] Clinical features of catecholamine excess include hypertension, headache, sweating, palpitations, and often morbid apprehension or anxiety. These symptoms often come in paroxysms that can last for minutes or hours, with variable frequency.

• #15 Paraganglioma | Altru Health System

  https://www.altru.org/health-library/conditions/paraganglioma

  Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis. […] Symptoms of a paraganglioma in the head or neck can include: A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus. Trouble swallowing. Hoarse voice. Hearing loss. Blurred vision. Dizziness. […] Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures. […] When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone. […] Symptoms of paragangliomas that make hormones include: High blood pressure. Feelings of having a fast-beating, fluttering or pounding heart. The sudden loss of color in the face. Sweating. Headache. Uncontrolled shaking in the hands or arms. General weakness. […] These symptoms may come and go. […] Some people with paragangliomas don’t have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

• #16 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. […] Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events. […] The most common sign is high blood pressure that may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death. […] Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumors are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason. […] Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have: High blood pressure. Headache. Heavy sweating for no known reason. A strong, fast, or irregular heartbeat. Being shaky. Being extremely pale.

• #17 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Paragangliomas typically start out as benign (not cancerous) tumors, but they may cause symptoms that may become life-threatening. Benign paragangliomas may also become cancerous later and spread to other parts of the body (a process called metastasis). […] Paragangliomas release hormones that activate the body’s fight-or-flight response. Called catecholamines, these hormones are usually released in response to stress. A paraganglioma can release these hormones at will, sometimes in large doses. […] These hormones may lead to symptoms that include: High blood pressure, Flushing, Palpitations, Sweating, Headache, Shaking. […] These symptoms may feel like a panic or anxiety attack. […] These attacks, if they become severe, can cause a catecholamine crisis, which may result in a heart attack, stroke or severe high blood pressure.

• #18 Paraganglioma | Altru Health System

  https://www.altru.org/health-library/conditions/paraganglioma

  Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis. […] Symptoms of a paraganglioma in the head or neck can include: A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus. Trouble swallowing. Hoarse voice. Hearing loss. Blurred vision. Dizziness. […] Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures. […] When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone. […] Symptoms of paragangliomas that make hormones include: High blood pressure. Feelings of having a fast-beating, fluttering or pounding heart. The sudden loss of color in the face. Sweating. Headache. Uncontrolled shaking in the hands or arms. General weakness. […] These symptoms may come and go. […] Some people with paragangliomas don’t have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

• #19 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  Signs and symptoms of paraganglioma happen when the tumor releases too much adrenaline or noradrenaline into your blood. However, some paraganglioma tumors dont make extra adrenaline or noradrenaline and dont cause symptoms (are asymptomatic). Common symptoms of paraganglioma include episodes of: […] High blood pressure (hypertension). […] Headaches. […] Excessive sweating for no known reason. […] A pounding, fast or irregular heartbeat. […] Feeling shaky. […] Some people who have a paraganglioma may experience symptoms infrequently or in bursts. […] Paragangliomas usually grow very slowly. But this could vary from case to case. […] If paragangliomas are left untreated, whether benign or malignant, they can potentially cause serious, life-threatening complications due to the excess amounts of adrenaline and noradrenaline they can secrete. Complications can include:

• #20 Phaeochromocytoma

  https://www.nhs.uk/conditions/phaeochromocytoma/

  The symptoms of a phaeochromocytoma tend to be unpredictable, often occurring in sudden attacks lasting from a few minutes to an hour. […] The attacks may last longer, and become more frequent and severe, as the tumour grows. […] The symptoms and signs of a phaeochromocytoma can include: headaches, heavy sweating, heart palpitations, high blood pressure, a pale face, feeling or being sick, feeling anxious or panicky, shakiness (tremor). […] Some people with a phaeochromocytoma do not have symptoms, and their condition is either never diagnosed or only discovered during tests for another problem, such as high blood pressure. Others have symptoms for many years before a phaeochromocytoma is diagnosed. […] Without treatment, phaeochromocytomas can cause severe problems and significantly affect your quality of life.

• #21 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Signs and symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens: Hard physical activity. A physical injury or having a lot of emotional stress. Childbirth. Going under anesthesia. Surgery, including procedures to remove the tumor. Eating foods high in tyramine (such as red wine, chocolate, and cheese). […] Pheochromocytoma and paraganglioma can recur (come back) after they have been treated.

• #22 Pheochromocytoma and Paraganglioma – Missouri Cancer Associates

  https://missouricancer.com/cancer_type/pheochromocytoma-and-paraganglioma/

  Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. […] Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events. […] The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death. […] Signs and symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens: Hard physical activity, A physical injury or having a lot of emotional stress, Childbirth, Going under anesthesia, Surgery, including procedures to remove the tumor, Eating foods high in tyramine (such as red wine, chocolate, and cheese).

• #23 Paraganglioma | Altru Health System

  https://www.altru.org/health-library/conditions/paraganglioma

  Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis. […] Symptoms of a paraganglioma in the head or neck can include: A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus. Trouble swallowing. Hoarse voice. Hearing loss. Blurred vision. Dizziness. […] Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures. […] When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone. […] Symptoms of paragangliomas that make hormones include: High blood pressure. Feelings of having a fast-beating, fluttering or pounding heart. The sudden loss of color in the face. Sweating. Headache. Uncontrolled shaking in the hands or arms. General weakness. […] These symptoms may come and go. […] Some people with paragangliomas don’t have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

• #24 Paragangliomas – Treatment, Types & Symptoms | Mount Sinai – New York

  https://www.mountsinai.org/locations/cerebrovascular-center/conditions/tumors/paragangliomas

  Paragangliomas, also called glomus tumors, occur in a variety of places in the head and neck. They are rarely if ever cancerous. Almost half of all paragangliomas appear in the bone on the side of the head/skull (temporal bone). […] Caroid paragangliomas appear with an expanding and pulsing mass. When this paraganglioma appears as a simple, isolated lesion, you may experience relatively few symptoms. These tumors usually appears in the neck, but can extend into the space between the head and the neck. Some of most common symptoms are pain, hoarseness, fainting (syncope), difficulty swallowing (dysphagia), eye problems (Horner syndrome), and paralysis of the tongue. […] Cervical paragangliomas are slow-growing tumors that can cause palsy and spread into the skull base. It can be difficult to distinguish between tumors that affect the vagus and those that involve the carotid artery.

• #25 Paraganglioma | Altru Health System

  https://www.altru.org/health-library/conditions/paraganglioma

  Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis. […] Symptoms of a paraganglioma in the head or neck can include: A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus. Trouble swallowing. Hoarse voice. Hearing loss. Blurred vision. Dizziness. […] Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures. […] When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone. […] Symptoms of paragangliomas that make hormones include: High blood pressure. Feelings of having a fast-beating, fluttering or pounding heart. The sudden loss of color in the face. Sweating. Headache. Uncontrolled shaking in the hands or arms. General weakness. […] These symptoms may come and go. […] Some people with paragangliomas don’t have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

• #26 Paragangliomas – Treatment, Types & Symptoms | Mount Sinai – New York

  https://www.mountsinai.org/locations/cerebrovascular-center/conditions/tumors/paragangliomas

  Jugular paragangliomas, which are usually large, often appear with paralysis of the fourth, fifth, and 11th cranial nerves. The symptoms vary. If they paralyze the fourth nerve paralysis, it can produce double vision; if it disturbs the fifth nerve paralysis, you can have some numbness of the face; and if the tumor affects the 11th cranial nerve, you may find it hard to move your shoulder. When the tumor extends beyond the jugular foramen, it may involve the 12th cranial nerve, which can produce paralysis and/or weakness in your tongue. […] Secretory paragangliomas can occur in up to one in eight patients and may involve the release of catecholamines. The symptoms may include headaches, hypertension or high blood pressure, palpitations, excessive sweating, and anxiety. Treatment may be difficult because it will jar the paraganglioma to release some hormones.

• #27 Paragangliomas – Treatment, Types & Symptoms | Mount Sinai – New York

  https://www.mountsinai.org/locations/cerebrovascular-center/conditions/tumors/paragangliomas

  Temporal paragangliomas can have one or more of the following symptoms: hearing loss, rhythmic thumping in the ear, paralysis of the face, discharge from the ear, pain, vertigo, hemorrhage from the ear (otorrhagia), lower cranial nerve paralysis, nausea and vomiting, vision problems, headaches, and changes in behavior. […] Vagal paragangliomas can cause paralysis to the lower cranial nerves (including the 10th cranial nerve, which can cause paralysis of the diaphragm), 12th cranial nerve paralysis (with paralysis of the tongue), problems in swallowing, and problems with raising your shoulder.

• #28 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Patients experiencing intermittent episodes of hypertension along with other classic symptoms, particularly when triggered by factors such as postural change, anxiety, medications, exercise, or maneuvers that increase intra-abdominal pressure, highly suggest a catecholamine paraganglioma. […] The constellation of hypertension, hematuria, and symptoms of catecholamine excess with micturition or sexual activity are classic signs of a bladder paraganglioma. […] Symptoms are dependent on the tumor location. The most common presentations of all paragangliomas are mass effect and catecholamine excess. […] Other possible clinical signs include upper GI bleeding, back or chest pain, cough, dyspnea, exercise-induced nausea, and vomiting. […] Examination usually reveals signs of sympathetic overload or mass effect, including but not limited to vocal cord paralysis causing hoarseness, cranial nerve paralysis, restlessness, tachycardia, diaphoresis, uncontrolled episodic hypertension, perspiration, cold and clammy skin, dilated eyes, dry mouth, and sometimes piloerection.

• #29 Progress in the diagnosis and treatment of paraganglioma – Wang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/32832/html

  The clinical manifestations of PGL are generally related to the location of the tumor and the concentration of catecholamines secreted by it, but the general symptoms include a painless mass that grows slowly, and in functional PGL, excessive catecholamine secretion causes additional symptoms, such as paroxysmal hypertension, hyperglycemia, palpitations, dizziness, headaches, excessive sweating and fatigue, polyuria, polydipsia, nausea, fever, and flushing as rare symptoms. […] Different disease sites can lead to different clinical manifestations, which can provide clues for the discovery of extra-adrenal occupancy. Retroperitoneal PGL often causes abdominal and lower back pain and may be associated with constipation, and the mass can often be felt during examination. […] PGL has a variety of clinical manifestations and a higher malignancy rate. It is essential to improve our understanding of PGL to ensure timely diagnosis and treatment.

• #30 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paraganglioma can occur at any age, but most commonly affects people between the ages of 20 and 50. While very rare, the illness often causes a range of symptoms that when recognized can help with diagnosis. Many of these symptoms can be caused by multiple other conditions as well. Some paragangliomas do not cause any symptoms. […] Signs or symptoms of paragangliomas may include: Hypertension, Sustained hypertension, Paroxysmal hypertension, Orthostatic hypotension, Headache, Palpitations+/-tachycardia, Diaphoresis/sweating, Anxiety, Swelling at tumor site. […] Less common signs or symptoms may include: Pallor, Flushing, Hyperglycemia, Vomiting, Abdominal/back pain, Nausea, Fatigue, Dyspnea, Dizziness, Visual symptoms. […] The signs and symptoms of paraganglioma can be caused by a number of different conditions.

• #31 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Not all paragangliomas release hormones, meaning some are asymptomatic. Those that develop in the head and neck usually don’t release hormones, but if they grow big enough, they can obstruct swallowing, cause coughing or lead to hearing loss. […] Other symptoms of paragangliomas may include: Gastrointestinal issues, including constipation and dry mouth, Chest pain and other cardiac effects of hormone surges, Hoarseness, nerve paralysis or dilated pupils from a mass in the head or neck, Generalized fatigue, Blurry vision, Weight loss, Excessive thirst or hunger, Lightheadedness. […] If the paraganglioma is spreading to other parts of the body, it’s known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] In about 10 percent of cases, paragangliomas have already spread by the time they’re diagnosed, according to Seminars in Oncology. Metastasis is more likely for paragangliomas that are discovered outside of the adrenal glands and for those that are larger than 5 centimeters (cm).

• #32 What Is Paraganglioma?

  https://www.icliniq.com/articles/cancer/paraganglioma

  Paraganglioma is a rare and treatable condition. It is a neuroendocrine tumor formed in the neck and head’s blood vessels and nerves. These tumors are benign. In sporadic cases, it is malignant. Symptoms caused by this condition are high blood pressure and headache. […] The signs and symptoms of this condition occur when excess adrenaline or noradrenaline is released into the blood. However, it does happen on occasion even when too much noradrenaline or adrenaline does not result in any symptoms. Some common signs and symptoms of this condition are: Hypertension. Headaches. Excessive sweating. Fast or irregular heartbeat. Feeling shaky. […] Very Rare Symptoms Are: Being much paler. Nausea and vomiting. Diarrhea. Constipation. Hyperglycemia, high glucose level. A sudden decrease in blood pressure on standing is called orthostatic hypotension. Weight loss.

• #33 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  Signs and symptoms of paraganglioma happen when the tumor releases too much adrenaline or noradrenaline into your blood. However, some paraganglioma tumors dont make extra adrenaline or noradrenaline and dont cause symptoms (are asymptomatic). Common symptoms of paraganglioma include episodes of: […] High blood pressure (hypertension). […] Headaches. […] Excessive sweating for no known reason. […] A pounding, fast or irregular heartbeat. […] Feeling shaky. […] Some people who have a paraganglioma may experience symptoms infrequently or in bursts. […] Paragangliomas usually grow very slowly. But this could vary from case to case. […] If paragangliomas are left untreated, whether benign or malignant, they can potentially cause serious, life-threatening complications due to the excess amounts of adrenaline and noradrenaline they can secrete. Complications can include:

• #34 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas typically represent benign hypervascular tumors that grow slowly. […] Most paragangliomas are benign. The incidence of malignant paragangliomas is around 90 to 95 cases per 400 million person-years. […] Approximately 20% of extra-adrenal paragangliomas are malignant, while 10% of pheochromocytomas are malignant. Nearly all paragangliomas in the head and neck are benign. […] Without subsequent radiation therapy, continued growth is probable following a subtotal resection. […] A significant portion, 65%, of individuals initially presenting with metastatic disease will experience recurrent metastatic disease. […] Those with metastatic disease exhibit a five-year survival rate ranging between 34% and 60%, with recurrence typically occurring around 5.5 years. […] Notably, 31% of individuals initially diagnosed with suspected benign disease may encounter recurrence, manifesting even 50 years after the initial diagnosis.

• #35 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas typically represent benign hypervascular tumors that grow slowly. […] Most paragangliomas are benign. The incidence of malignant paragangliomas is around 90 to 95 cases per 400 million person-years. […] Approximately 20% of extra-adrenal paragangliomas are malignant, while 10% of pheochromocytomas are malignant. Nearly all paragangliomas in the head and neck are benign. […] Without subsequent radiation therapy, continued growth is probable following a subtotal resection. […] A significant portion, 65%, of individuals initially presenting with metastatic disease will experience recurrent metastatic disease. […] Those with metastatic disease exhibit a five-year survival rate ranging between 34% and 60%, with recurrence typically occurring around 5.5 years. […] Notably, 31% of individuals initially diagnosed with suspected benign disease may encounter recurrence, manifesting even 50 years after the initial diagnosis.

• #36 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  If any of the listed signs or symptoms come and go suddenly, you should see a doctor. Its important to get a prompt diagnosis. […] Although high blood pressure is a primary sign of a paraganglioma, most people with high blood pressure don’t have a paraganglioma, and not all patients with a paraganglioma have hypertension. […] All paraganglioma have the potential to become metastatic, or spread to other parts of the body. This happens in approximately 15-25% of cases. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. Some patients live for decades with metastatic paraganglioma. The prognosis is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5-6 cm are more likely to metastasize), levels of methoxytyramine (a metabolite of the neurotransmitter dopamine which can be measured in the blood), and genetic status. […] Metastatic para often spreads to the bone and sometimes to the lymph nodes, lungs and liver.

• #37 Paraganglioma – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/paraganglioma

  Some people with paraganglioma have symptoms, but others don’t. Symptoms can include: High blood pressure Fast heartbeat Sweating Headache Shaking or tremors […] Approximately 35-50% of paragangliomas may spread to other parts of the body. […] Patients with a small paraganglioma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with paraganglioma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.

• #38 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Not all paragangliomas release hormones, meaning some are asymptomatic. Those that develop in the head and neck usually don’t release hormones, but if they grow big enough, they can obstruct swallowing, cause coughing or lead to hearing loss. […] Other symptoms of paragangliomas may include: Gastrointestinal issues, including constipation and dry mouth, Chest pain and other cardiac effects of hormone surges, Hoarseness, nerve paralysis or dilated pupils from a mass in the head or neck, Generalized fatigue, Blurry vision, Weight loss, Excessive thirst or hunger, Lightheadedness. […] If the paraganglioma is spreading to other parts of the body, it’s known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] In about 10 percent of cases, paragangliomas have already spread by the time they’re diagnosed, according to Seminars in Oncology. Metastasis is more likely for paragangliomas that are discovered outside of the adrenal glands and for those that are larger than 5 centimeters (cm).

• #39 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  If any of the listed signs or symptoms come and go suddenly, you should see a doctor. Its important to get a prompt diagnosis. […] Although high blood pressure is a primary sign of a paraganglioma, most people with high blood pressure don’t have a paraganglioma, and not all patients with a paraganglioma have hypertension. […] All paraganglioma have the potential to become metastatic, or spread to other parts of the body. This happens in approximately 15-25% of cases. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. Some patients live for decades with metastatic paraganglioma. The prognosis is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5-6 cm are more likely to metastasize), levels of methoxytyramine (a metabolite of the neurotransmitter dopamine which can be measured in the blood), and genetic status. […] Metastatic para often spreads to the bone and sometimes to the lymph nodes, lungs and liver.

• #40 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Not all paragangliomas release hormones, meaning some are asymptomatic. Those that develop in the head and neck usually don’t release hormones, but if they grow big enough, they can obstruct swallowing, cause coughing or lead to hearing loss. […] Other symptoms of paragangliomas may include: Gastrointestinal issues, including constipation and dry mouth, Chest pain and other cardiac effects of hormone surges, Hoarseness, nerve paralysis or dilated pupils from a mass in the head or neck, Generalized fatigue, Blurry vision, Weight loss, Excessive thirst or hunger, Lightheadedness. […] If the paraganglioma is spreading to other parts of the body, it’s known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] In about 10 percent of cases, paragangliomas have already spread by the time they’re diagnosed, according to Seminars in Oncology. Metastasis is more likely for paragangliomas that are discovered outside of the adrenal glands and for those that are larger than 5 centimeters (cm).

• #41 Pheo Para in Kids – Pheo Para Alliance

  https://pheopara.org/education/pheoparakids

  A pheo or para is defined as metastatic is if it has spread to a distant site, where pheos and paras are typically not found, such as the bone, liver or lungs. Currently, there is no cure for metastatic pheo or para. However, existing treatment options may reduce the tumor burden and prolong survival. Many patients live for decades with metastatic tumors. […] The prognosis of metastatic pheo para is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5 cm are more likely to metastasize, except in the case of SDHB, where tumors larger than 3 cm are more likely to metastasize) and genetic status. Paras that are originally in the head and neck are less likely to metastasize than paras that develop in other areas. […] Some genetic mutations, such as SDHB, are more likely to develop metastatic para.

• #42 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  If any of the listed signs or symptoms come and go suddenly, you should see a doctor. Its important to get a prompt diagnosis. […] Although high blood pressure is a primary sign of a paraganglioma, most people with high blood pressure don’t have a paraganglioma, and not all patients with a paraganglioma have hypertension. […] All paraganglioma have the potential to become metastatic, or spread to other parts of the body. This happens in approximately 15-25% of cases. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. Some patients live for decades with metastatic paraganglioma. The prognosis is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5-6 cm are more likely to metastasize), levels of methoxytyramine (a metabolite of the neurotransmitter dopamine which can be measured in the blood), and genetic status. […] Metastatic para often spreads to the bone and sometimes to the lymph nodes, lungs and liver.

• #43 Long-term outcomes and prognostic factors of metastatic or recurrent pheochromocytoma and paraganglioma: a 20-year review in a single institution | Scientific Reports

  https://www.nature.com/articles/s41598-024-75354-9

  Pheochromocytoma and paraganglioma (PPGL) represent a group of rare neuroendocrine tumors known for their potential to metastasize. […] Median duration from initial PPGL diagnosis to the onset of metastasis or recurrence stood at 5.8 years. […] Predominant sites for metastasis included the bone, lung, lymph nodes, and peritoneum. […] A salient finding was that surgical interventions targeting metastatic lesions significantly improved prognosis. […] Further analysis revealed that a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) exceeding 7 closely associated with unfavorable outcomes. […] The risk of metastasis is 10-20% in pheochromocytoma and up to 50% in paraganglioma. […] PPGL is characterized by local invasion or distant metastases, which often makes it challenging to treat and results in poor prognosis.

• #44 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  Not all paragangliomas release hormones, meaning some are asymptomatic. Those that develop in the head and neck usually don’t release hormones, but if they grow big enough, they can obstruct swallowing, cause coughing or lead to hearing loss. […] Other symptoms of paragangliomas may include: Gastrointestinal issues, including constipation and dry mouth, Chest pain and other cardiac effects of hormone surges, Hoarseness, nerve paralysis or dilated pupils from a mass in the head or neck, Generalized fatigue, Blurry vision, Weight loss, Excessive thirst or hunger, Lightheadedness. […] If the paraganglioma is spreading to other parts of the body, it’s known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] In about 10 percent of cases, paragangliomas have already spread by the time they’re diagnosed, according to Seminars in Oncology. Metastasis is more likely for paragangliomas that are discovered outside of the adrenal glands and for those that are larger than 5 centimeters (cm).

• #45 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas typically represent benign hypervascular tumors that grow slowly. […] Most paragangliomas are benign. The incidence of malignant paragangliomas is around 90 to 95 cases per 400 million person-years. […] Approximately 20% of extra-adrenal paragangliomas are malignant, while 10% of pheochromocytomas are malignant. Nearly all paragangliomas in the head and neck are benign. […] Without subsequent radiation therapy, continued growth is probable following a subtotal resection. […] A significant portion, 65%, of individuals initially presenting with metastatic disease will experience recurrent metastatic disease. […] Those with metastatic disease exhibit a five-year survival rate ranging between 34% and 60%, with recurrence typically occurring around 5.5 years. […] Notably, 31% of individuals initially diagnosed with suspected benign disease may encounter recurrence, manifesting even 50 years after the initial diagnosis.

• #46 Long-term outcomes and prognostic factors of metastatic or recurrent pheochromocytoma and paraganglioma: a 20-year review in a single institution | Scientific Reports

  https://www.nature.com/articles/s41598-024-75354-9

  Pheochromocytoma and paraganglioma (PPGL) represent a group of rare neuroendocrine tumors known for their potential to metastasize. […] Median duration from initial PPGL diagnosis to the onset of metastasis or recurrence stood at 5.8 years. […] Predominant sites for metastasis included the bone, lung, lymph nodes, and peritoneum. […] A salient finding was that surgical interventions targeting metastatic lesions significantly improved prognosis. […] Further analysis revealed that a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) exceeding 7 closely associated with unfavorable outcomes. […] The risk of metastasis is 10-20% in pheochromocytoma and up to 50% in paraganglioma. […] PPGL is characterized by local invasion or distant metastases, which often makes it challenging to treat and results in poor prognosis.

• #47 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Signs and symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens: Hard physical activity. A physical injury or having a lot of emotional stress. Childbirth. Going under anesthesia. Surgery, including procedures to remove the tumor. Eating foods high in tyramine (such as red wine, chocolate, and cheese). […] Pheochromocytoma and paraganglioma can recur (come back) after they have been treated.

• #48 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  Signs and symptoms of paraganglioma happen when the tumor releases too much adrenaline or noradrenaline into your blood. However, some paraganglioma tumors dont make extra adrenaline or noradrenaline and dont cause symptoms (are asymptomatic). Common symptoms of paraganglioma include episodes of: […] High blood pressure (hypertension). […] Headaches. […] Excessive sweating for no known reason. […] A pounding, fast or irregular heartbeat. […] Feeling shaky. […] Some people who have a paraganglioma may experience symptoms infrequently or in bursts. […] Paragangliomas usually grow very slowly. But this could vary from case to case. […] If paragangliomas are left untreated, whether benign or malignant, they can potentially cause serious, life-threatening complications due to the excess amounts of adrenaline and noradrenaline they can secrete. Complications can include:

• #49 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  Heart muscle disease (cardiomyopathy). […] Inflammation of your heart muscle (myocarditis). […] Uncontrolled bleeding in your brain (cerebral hemorrhaging). […] Accumulation of fluid in your lungs (pulmonary edema). […] Heart attack (myocardial infarction). […] Stroke. […] Coma. […] Death.

• #50 What Is Paraganglioma?

  https://www.icliniq.com/articles/cancer/paraganglioma

  The prognosis of this condition depends on: The location and size of the tumor. If the condition is malignant and has spread to other regions. If the tumor can be eliminated surgically. […] If paraganglioma is left untreated, whether benign or malignant, it can result in severe life-threatening conditions due to increased adrenaline or noradrenaline in the blood. Some common complications are: Cardiomyopathy – A heart muscle disease. Myocarditis – Heart muscle inflammation. Cerebral Hemorrhage – Uncontrolled bleeding in the head. Pulmonary Edema – A collection of fluid in the lungs. Heart attack. Coma. […] The signs and symptoms of this condition are caused due to excessive adrenaline and noradrenaline in the blood. These tumors can be benign or malignant, depending on the cause; they are being treated through medications or other methods.

• #51 Paraganglioma | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/paraganglioma

  Paragangliomas are rare tumors that make too much adrenaline. […] Paraganglioma can occur at any age and there are no known risk factors except for certain genetic syndromes. Up to 25% of paragangliomas are inherited. […] The classic symptoms of paraganglioma include: high blood pressure, rapid heart rate (palpitations), headache, flushing, and sweating. In addition, patients may feel like they are having an anxiety or panic attack (difficulty breathing, weakness, a feeling that something „bad” is happening). Other less common symptoms may include pale skin, blurred vision, weight loss, constipation, abdominal pain, high blood sugar levels, low blood pressure, and psychiatric disturbances. […] High blood pressure (i.e. hypertension) is the most common symptom related to paraganglioma, but in 10% of patients, the blood pressure may be normal. […] Patients in pheo/paraganglioma crisis have had a massive release of adrenaline that causes major health problems including stroke, heart attack, heart failure, multiple organ failure, coma, and even death.

• #52 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1–5

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4532956/

  Rarely, patients may present with catecholaminergic crisis accompanied by acute cardiomyopathy and severe hypertension (but sometimes with shock), and/or multiorgan failure, lactic acidosis, encephalopathy, fever and hyperglycaemia. […] Clinical features of local tumour mass are relevant for HNPGLs and also for metastatic PPGLs. […] Incidental discovery of PPGLs on imaging is becoming an increasingly important mode of their diagnosis; for instance, the proportion of PCs detected incidentally is now 25-30% of all cases. […] The penetrance of tumour development in subjects carrying SDHx mutations is highly variable, in particular for PGL3 highlighted by the occasional finding that a child or young adult is the index case in a family where the parent (and sometimes grandparent) carrying the pathogenic allele has not developed tumours and iii) tumour development does not clearly follow a predetermined pattern PPGLs, renal cancer, GISTs and/or pituitary tumours occur in a seemingly random fashion in affected subjects. […] PGL1 has very high lifetime penetrance, and 75% of carriers will manifest disease by age 40. […] PGL4 is more likely to be associated with TAPGL and/or PC, is more likely to present with symptoms from local tumour mass, and malignant disease occurs in around a third of patients.

• #53 What Is Paraganglioma?

  https://www.icliniq.com/articles/cancer/paraganglioma

  The prognosis of this condition depends on: The location and size of the tumor. If the condition is malignant and has spread to other regions. If the tumor can be eliminated surgically. […] If paraganglioma is left untreated, whether benign or malignant, it can result in severe life-threatening conditions due to increased adrenaline or noradrenaline in the blood. Some common complications are: Cardiomyopathy – A heart muscle disease. Myocarditis – Heart muscle inflammation. Cerebral Hemorrhage – Uncontrolled bleeding in the head. Pulmonary Edema – A collection of fluid in the lungs. Heart attack. Coma. […] The signs and symptoms of this condition are caused due to excessive adrenaline and noradrenaline in the blood. These tumors can be benign or malignant, depending on the cause; they are being treated through medications or other methods.

• #54 Paraganglioma – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/paraganglioma

  Some people with paraganglioma have symptoms, but others don’t. Symptoms can include: High blood pressure Fast heartbeat Sweating Headache Shaking or tremors […] Approximately 35-50% of paragangliomas may spread to other parts of the body. […] Patients with a small paraganglioma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with paraganglioma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.

• #55 Long-term outcomes and prognostic factors of metastatic or recurrent pheochromocytoma and paraganglioma: a 20-year review in a single institution | Scientific Reports

  https://www.nature.com/articles/s41598-024-75354-9

  The 5-, 10-, and 15-year survival rates after metastasis or recurrent diagnosis were 73.3%, 62.9%, and 31.4%, respectively, which is consistent with previous studies. […] Our study revealed significantly higher PASS in the rapid progression group. […] In addition, patients with PASS7 for the primary tumor had a significantly worse prognosis. […] Surgical intervention was significantly associated with a greater probability of OS and PASS7 for the primary tumor was a poor prognostic factor.

• #56 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  If any of the listed signs or symptoms come and go suddenly, you should see a doctor. Its important to get a prompt diagnosis. […] Although high blood pressure is a primary sign of a paraganglioma, most people with high blood pressure don’t have a paraganglioma, and not all patients with a paraganglioma have hypertension. […] All paraganglioma have the potential to become metastatic, or spread to other parts of the body. This happens in approximately 15-25% of cases. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. Some patients live for decades with metastatic paraganglioma. The prognosis is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5-6 cm are more likely to metastasize), levels of methoxytyramine (a metabolite of the neurotransmitter dopamine which can be measured in the blood), and genetic status. […] Metastatic para often spreads to the bone and sometimes to the lymph nodes, lungs and liver.

• #57 Pheo Para in Kids – Pheo Para Alliance

  https://pheopara.org/education/pheoparakids

  A pheo or para is defined as metastatic is if it has spread to a distant site, where pheos and paras are typically not found, such as the bone, liver or lungs. Currently, there is no cure for metastatic pheo or para. However, existing treatment options may reduce the tumor burden and prolong survival. Many patients live for decades with metastatic tumors. […] The prognosis of metastatic pheo para is highly variable and is dependent upon the size of the primary tumor (tumors larger than 5 cm are more likely to metastasize, except in the case of SDHB, where tumors larger than 3 cm are more likely to metastasize) and genetic status. Paras that are originally in the head and neck are less likely to metastasize than paras that develop in other areas. […] Some genetic mutations, such as SDHB, are more likely to develop metastatic para.

• #58 Pheo Para in Kids – Pheo Para Alliance

  https://pheopara.org/education/pheoparakids

  Among all ages, signs and symptoms of pheo para are highly variable and dependent on tumor location, tumor size and whether it is secreting hormones or not. Some patients do not experience any symptoms. […] Among children specifically, 70-90% present with hypertension, which is usually sustained (i.e., always having high blood pressure, as opposed only sometimes high). Signs and symptoms in children may include sweating, visual problems, weight loss, and nausea/vomiting. Other symptoms may include headache, neurologic symptoms and tachycardia (rapid heartbeat). Due to high catecholamines, children may also experience decreased school performance, behavioral problems and/or be diagnosed with ADD/ADHD. […] It is highly recommended that patients with metastatic disease receive treatment from an experienced, multi-disciplinary pheo para team. Also, if your child has a genetic mutation, this may help to determine an appropriate course of treatment.

• #59 Paraganglioma and Pheochromocytoma | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/paraganglioma-and-pheochromocytoma

  Paragangliomas and pheochromocytomas are tumors that develop out of the neuroendocrine tissue responsible for making epinephrine. This hormone, also known as adrenaline, and other related hormones help regulate heart rate and blood pressure in response to stress. Paragangliomas, known as PGLs, can develop anywhere in the body from the pelvis to the skull. Some release hormones that are similar to adrenaline and others do not. […] The symptoms of paraganglioma/pheochromocytoma (PCCs and PGLs) may vary from child to child and depend on where the tumor is located and whether it produces hormones. Symptoms might mimic other, more common ailments. […] For PCCs/PGLs, the most common symptoms are: High blood pressure, Rapid pulse, Heart palpitations, Headache, Dizziness, Poor weight gain despite good appetite, Abdominal pain, Nausea, Vomiting, Pale skin, Clammy skin, Sweating, Growth failure.

• #60 Hereditary pheochromocytoma-paraganglioma

  https://www.genturis.eu/l=eng/thematic-disease-groups/other-rare-genturis/hereditary-pheochromocytoma-paraganglioma.html

  Pheochromocytomas and paragangliomas may produce excessive amounts of hormones, called catecholamines, causing a variety of symptoms such as palpitations, headaches, excessive sweating and nausea. These symptoms might be the first manifestation of the tumours. […] Individuals with hereditary pheochromocytoma-paraganglioma tend to develop tumours at a younger age (as early as during childhood), and are more likely to develop multifocal, multiple and/or recurrent disease. […] A lifelong regular screening is advised for individuals with hereditary pheochromocytoma-paraganglioma. This typically includes clinical exams and questionnaires, biochemical examinations (metanephrines) and imaging techniques (including magnetic resonance tomography). The initiation of screening is typically recommended during childhood and the periods of evaluations differ for children and adults.

• #61 Hereditary Paraganglioma-Pheochromocytoma Syndrome | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hereditary-paraganglioma-pheochromocytoma-syndrome

  Paragangliomas (PGLs) and pheochromocytomas (PCCs) are rare tumors that occur sporadically or as part of a hereditary cancer predisposing syndrome. Compared to people with sporadic tumors, individuals with hereditary PGL/PCC tend to develop tumors at a younger age and are more likely to have multifocal disease (more than one tumor, all of which have arisen from one original tumor and are often located near each other), bilateral disease (tumors in paired organs, such as both adrenal glands) and/or recurrent disease. […] The diagnosis of a hereditary paraganglioma-pheochromoctyoma (PGL/PCC) syndrome should be considered in all individuals with PGL and/or PCCs, particularly those where the tumors are: Multiple (more than one separate tumor or tumor type), including bilateral tumors (tumors found in each of a pair of organs), Multifocal (more than one tumor, all of which have arisen from one original tumor and are often located near each other), Recurrent, Early in onset (i.e. development of a tumor earlier than age 40), Linked to a family history of such tumors.

• #62 Paraganglioma | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/paraganglioma

  Paragangliomas are rare tumors that make too much adrenaline. […] Paraganglioma can occur at any age and there are no known risk factors except for certain genetic syndromes. Up to 25% of paragangliomas are inherited. […] The classic symptoms of paraganglioma include: high blood pressure, rapid heart rate (palpitations), headache, flushing, and sweating. In addition, patients may feel like they are having an anxiety or panic attack (difficulty breathing, weakness, a feeling that something „bad” is happening). Other less common symptoms may include pale skin, blurred vision, weight loss, constipation, abdominal pain, high blood sugar levels, low blood pressure, and psychiatric disturbances. […] High blood pressure (i.e. hypertension) is the most common symptom related to paraganglioma, but in 10% of patients, the blood pressure may be normal. […] Patients in pheo/paraganglioma crisis have had a massive release of adrenaline that causes major health problems including stroke, heart attack, heart failure, multiple organ failure, coma, and even death.

• #63 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1–5

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4532956/

  Rarely, patients may present with catecholaminergic crisis accompanied by acute cardiomyopathy and severe hypertension (but sometimes with shock), and/or multiorgan failure, lactic acidosis, encephalopathy, fever and hyperglycaemia. […] Clinical features of local tumour mass are relevant for HNPGLs and also for metastatic PPGLs. […] Incidental discovery of PPGLs on imaging is becoming an increasingly important mode of their diagnosis; for instance, the proportion of PCs detected incidentally is now 25-30% of all cases. […] The penetrance of tumour development in subjects carrying SDHx mutations is highly variable, in particular for PGL3 highlighted by the occasional finding that a child or young adult is the index case in a family where the parent (and sometimes grandparent) carrying the pathogenic allele has not developed tumours and iii) tumour development does not clearly follow a predetermined pattern PPGLs, renal cancer, GISTs and/or pituitary tumours occur in a seemingly random fashion in affected subjects. […] PGL1 has very high lifetime penetrance, and 75% of carriers will manifest disease by age 40. […] PGL4 is more likely to be associated with TAPGL and/or PC, is more likely to present with symptoms from local tumour mass, and malignant disease occurs in around a third of patients.

• #64 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1–5

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4532956/

  Rarely, patients may present with catecholaminergic crisis accompanied by acute cardiomyopathy and severe hypertension (but sometimes with shock), and/or multiorgan failure, lactic acidosis, encephalopathy, fever and hyperglycaemia. […] Clinical features of local tumour mass are relevant for HNPGLs and also for metastatic PPGLs. […] Incidental discovery of PPGLs on imaging is becoming an increasingly important mode of their diagnosis; for instance, the proportion of PCs detected incidentally is now 25-30% of all cases. […] The penetrance of tumour development in subjects carrying SDHx mutations is highly variable, in particular for PGL3 highlighted by the occasional finding that a child or young adult is the index case in a family where the parent (and sometimes grandparent) carrying the pathogenic allele has not developed tumours and iii) tumour development does not clearly follow a predetermined pattern PPGLs, renal cancer, GISTs and/or pituitary tumours occur in a seemingly random fashion in affected subjects. […] PGL1 has very high lifetime penetrance, and 75% of carriers will manifest disease by age 40. […] PGL4 is more likely to be associated with TAPGL and/or PC, is more likely to present with symptoms from local tumour mass, and malignant disease occurs in around a third of patients.

• #65 Cardiac Paraganglioma in a Young Patient Presents with Angina-like Symptoms: A Case Report and Literature Review

  https://www.mdpi.com/1648-9144/60/9/1495

  Paragangliomas are rare extra-adrenal neuroendocrine tumors originating from chromaffin tissue that present a diagnostic and therapeutic challenge due to their diverse clinical manifestations and low incidence. […] This study presents the case of a 22-year-old patient with cardiac paraganglioma who initially presented with angina-like symptoms, highlighting the importance of considering this rare condition in young individuals with nonspecific complaints. […] Episodic headaches, palpitations, and sweating are the characteristic features of catecholamine-secreting tumors. The intermittent nature of catecholamine release in paraganglioma can lead to a delayed diagnosis. […] Although tumor resection is reported as the most essential treatment for cardiac paraganglioma, the 5-year overall survival is only 78.8% for those with benign tumors and even lower for others with malignant tumors.

• #66 Cardiac Paraganglioma in a Young Patient Presents with Angina-like Symptoms: A Case Report and Literature Review

  https://www.mdpi.com/1648-9144/60/9/1495

  Paragangliomas are rare extra-adrenal neuroendocrine tumors originating from chromaffin tissue that present a diagnostic and therapeutic challenge due to their diverse clinical manifestations and low incidence. […] This study presents the case of a 22-year-old patient with cardiac paraganglioma who initially presented with angina-like symptoms, highlighting the importance of considering this rare condition in young individuals with nonspecific complaints. […] Episodic headaches, palpitations, and sweating are the characteristic features of catecholamine-secreting tumors. The intermittent nature of catecholamine release in paraganglioma can lead to a delayed diagnosis. […] Although tumor resection is reported as the most essential treatment for cardiac paraganglioma, the 5-year overall survival is only 78.8% for those with benign tumors and even lower for others with malignant tumors.

• #67 Top Differences between Pheochromocytoma and Paragangliomas

  https://www.adrenal.com/blog/top-6-differences-between-pheochromocytoma-and-paraganglioma

  Pheochromocytomas tend to have classic episodic symptoms (spells) of adrenaline excess than paragangliomas. […] Given that the types and degree of hormone overproduction of adrenaline-type hormones are different between paraganglioma and pheochromocytoma, it is no surprise that the symptoms can be distinct. As mentioned above paraganglioma patients tend to have sustained high blood pressure whereas pheochromocytoma patients have more episodic blood pressure elevation. Spells are more common in pheochromocytoma. […] This list summarized a typical paroxysm (spell) from a pheochromocytoma or paraganglioma: Headache, sweating, and palpitations; Very high BP (frequently with tachycardia); Chest or abdominal pain; Pallor/flush; Apprehension (sense of impending doom); Duration (5 min to an hour or longer); Spells may be spontaneous or precipitated by change in body position, anxiety, medications (e.g., metoclopramide, anesthetic agents), and maneuvers that increase intraabdominal pressure (e.g. going to the bathroom).

• #68 Top Differences between Pheochromocytoma and Paragangliomas

  https://www.adrenal.com/blog/top-6-differences-between-pheochromocytoma-and-paraganglioma

  Other typical signs and symptoms include: Headaches (severe); Excess sweating (generalized); Racing heart (tachycardia and palpitations); Anxiety and nervousness; Nervous shaking (tremors); Pain in the lower chest or upper abdomen; Nausea (with or without vomiting); Weight loss; Heat intolerance; Recent onset of hypertension; Severe or malignant hypertension; Marked blood pressure lability; Carbohydrate intolerance or overt new onset diabetes mellitus; Adrenal mass on imaging; Orthostatic hypotension in untreated state; Family history of pheochromocytoma; Unanticipated prominent changes in BP (up or down) in response to drugs or diagnostic manipulations. […] Both paraganglioma and pheochromocytoma can be cancerous (have a metastatic or malignant potential). However, as a group, paragangliomas are more commonly aggressive and can metastasize. The vast majority of these aggressive tumors harbor a genetic alteration in one of the SDHx genes.

• #69

  https://link.springer.com/article/10.1007/s12672-021-00404-x

  The clinical signs and symptoms of PPGLs are highly variable and mainly relate to excessive tumoral production of catecholamines. Recurrent paroxysms of hypertension, headache, palpitations, diaphoresis and pallor represent the main clinical signs and symptoms. Anxiety, tremor, nausea, vomiting, and weight loss are also relevant to consider. […] The most common symptom in patients with mPPGL was diaphoresis (48.8%), followed by weakness (46.5%), headaches (34.9%) and palpitations (32.6%). Palpitations were the most common symptom in patients with non-mPPGL (49%), followed by diaphoresis (45.6%), weakness (41.3%) and headaches (39.3%). There were no significant differences in clinical signs and symptoms between patients with non-mPPGLs and mPPGLs. In particular, the classic triad of palpitations, headache and diaphoresis did not differ.

• #70

  https://link.springer.com/article/10.1007/s12672-021-00404-x

  In this study we found no differences in signs and symptoms associated with catecholamine excess between patients with and without mPPGLs. This applied not only to singular signs and symptoms but also to the cumulative cSS score. A high burden of signs and symptoms was associated with high urinary excretion of norepinephrine and normetanephrine in patients with mPPGLs, whereas in patients without metastases the association was stronger for urinary epinephrine and metanephrine. […] Despite clear differences in the catecholamine secretion patterns between patients with and without metastatic PPGLs, this study demonstrates that presenting signs and symptoms do not appear to differ between the two groups. Therefore, signs and symptoms cannot be used for identification of patients with PPGLs, who either have metastases at presentation or who are prone to future metastatic development.

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