Materiały źródłowe

• #1 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. […] Your OHC doctor will help you determine the best care plan for you. […] Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed. […] Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed.

• #2 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. […] 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. […] The best treatment for paraganglioma is to remove it surgically.

• #3 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. […] If the paraganglioma is spreading to other parts of the body, its known as metastatic paraganglioma. About 35 percent to 50 percent of paragangliomas may spread to other parts of the body, according to the NCI. […] Your multidisciplinary team will work with you to develop a personalized plan to treat your neuroendocrine tumor in a way that fits your individual needs and goals. […] Depending on the features of the patient’s paraganglioma, the care team may suggest several different treatment options. If the paraganglioma is causing symptoms, they may be controlled with medications. […] If the symptoms could become life-threatening, the care team is likely to suggest surgery to remove the tumor.

• #4 Paragangliomas and Other Skull Base Tumors ENT Treatment in Utah – ENT Center of Utah

  https://entcenterutah.com/adult-care/head-neck-oncology/paragangliomas-skull-tumors/

  People with paragangliomas need care by an experienced, multidisciplinary team. Ask your doctor if he or she regularly treats people with this condition, as most doctors rarely (if ever) encounter paragangliomas and are unfamiliar with the best approaches to diagnosing and treating this rare tumor. Under such conditions, it is important to seek a second opinion from a team that specializes in the care of people with rare neuroendocrine tumors such as paragangliomas. Our Head Neck Surgeons have extensive experience in managing these tumors. […] Treatment options include: Medications. Your doctor will first attempt to control symptoms caused by your paraganglioma, such as high blood pressure, if you have the type of paraganglioma that produces hormones. Drugs that usually help people with this condition include alpha blockers and beta blockers. In people with hormone-producing paragangliomas, it is critical to control blood pressure before surgery or other therapy.

• #5

  https://www.healio.com/news/endocrinology/20211122/multidisciplinary-care-key-in-updated-pheochromocytoma-paraganglioma-guideline

  People with metastatic pheochromocytomas and paragangliomas should be closely monitored and undergo routine cross-sectional imaging while also being evaluated by a multidisciplinary team, according to a guideline published in Pancreas. […] Updated guidelines from a North American Neuroendocrine Tumor Society expert panel emphasize close monitoring of metastatic pheochromocytomas and paragangliomas and multidisciplinary care. […] The researchers wrote there is a lack of evidence for any therapy treating metastatic pheochromocytomas and paragangliomas, and follow-up is limited in retrospective studies. Because of this, treatment decisions should be made by a multidisciplinary team. […] Multidisciplinary care at an expert center is critical for patients with metastatic pheochromocytoma and paragangliomas to receive the most up-to-date recommendations and treatments from providers who are experienced in treating this rare cancer, Fishbein said.

• #6 Pheochromocytoma and Paraganglioma | Holden

  https://cancer.uiowa.edu/cancer-types/pheochromocytoma-and-paraganglioma

  Holden Comprehensive Cancer Center is a leader in pheo para care and in the research of new pheo para treatments. […] A team of experts dedicated to pheo para. Pheo para is a complicated disease that requires a team working in close collaboration. Our care teams include adult and pediatric specialists in abdominal, chest, head and neck surgery, endocrinology, oncology, radiation oncology, nuclear medicine, and genetic counseling. The pheo para team meets regularly to discuss patient care plans, treatment, research, and more. […] Genetic testing and counseling is available for adult and pediatric patients. Our genetic counselors provide diagnosis and work closely with our specialized care teams to help develop screening and care plans for patients who are affected by, or at risk for, hereditary paraganglioma syndrome.

• #7 Paragangliomas – ThinkGenetic Foundation

  https://thinkgenetic.org/diseases/paragangliomas-109959/

  Paragangliomas may also occur simultaneously with pheochromocytomas and may occasionally occur as multiple tumors. […] Cancer centers are the primary treatment center for individuals with a paragangliomas, even if their tumor is not cancerous. However monitoring and management involves doctors from many different specialties including endocrinologists, endocrine surgeons, medical oncologists, radiation oncologists, gastroenterologists, dietitians, and genetic counselors or geneticists. […] If a paraganglioma is identified that is causing a medical issue (difficulty breathing, hearing loss, etc) or producing hormones treatment is required. First line therapy often either surgery, medical therapy to block the effects of adrenaline excess, and/or radiation therapy. […] If this occurs, the paragangliomas will often be removed.

• #8 Pheochromocytoma and Paraganglioma | Holden

  https://cancer.uiowa.edu/cancer-types/pheochromocytoma-and-paraganglioma

  Nurse practitioners. Cassie Doolady, ARNP, DNP, Nurse Practitioner. Melissa Tvedte, ARNP, Nurse Practitioner. Lindsey Grieser, ARNP, Nurse Practitioner. […] Nurse Coordinator. Kimberly D. Miller, RN, BSN, OCN, Nurse Coordinator. Kelley McLaughlin, RN, Nurse Coordinator. Joan Ricks McGillin, RN, Nurse Coordinator. Alicia Rock-Cleppe, RN, BSN, Nurse Coordinator.

• #9 Paraganglioma Clinic | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/screening-prevention/paraganglioma-clinic

  Our team of experts is committed to making sure patients with paragangliomas and/or genetic risk for these tumors receive the best possible care. […] The Paraganglioma Clinic helps patients and their families create personal screening plans. We also help schedule follow-up care for people at high risk for paragangliomas. Our health care team includes specialists in every area of paraganglioma care. Our goal is to provide a cohesive and patient-centered approach. […] We recommend all people with a personal diagnosis of paraganglioma or pheochromocytoma get genetic counseling and testing.

• #10 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Before surgery the patient must be adequately blocked with medication. Going under anesthesia without being blocked is very dangerous. Anesthesia can cause tumors to release massive amounts of catecholamines. […] It is extremely important that practitioners involved in the care of the patient have experience with pheo para surgery and that patients be blocked for the best possible outcome. […] Urine or plasma tests should be repeated 2-8 weeks after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients. Yearly urine or plasma tests for paraganglioma should be performed for life to detect remaining disease, return of the disease, or the development of metastases. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing.

• #11 Paraganglioma | Columbia Surgery

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

  Before an operation to remove a paraganglioma, the patient must be properly prepared with certain medications to block the effects of adrenaline that will be released during the operation. […] Even with the best pre-operative preparation, patients may still have wide blood pressure changes, irregular heart rhythms, and bleeding.

• #12 Paragangliomas and Other Skull Base Tumors ENT Treatment in Utah – ENT Center of Utah

  https://entcenterutah.com/adult-care/head-neck-oncology/paragangliomas-skull-tumors/

  People with paragangliomas need care by an experienced, multidisciplinary team. Ask your doctor if he or she regularly treats people with this condition, as most doctors rarely (if ever) encounter paragangliomas and are unfamiliar with the best approaches to diagnosing and treating this rare tumor. Under such conditions, it is important to seek a second opinion from a team that specializes in the care of people with rare neuroendocrine tumors such as paragangliomas. Our Head Neck Surgeons have extensive experience in managing these tumors. […] Treatment options include: Medications. Your doctor will first attempt to control symptoms caused by your paraganglioma, such as high blood pressure, if you have the type of paraganglioma that produces hormones. Drugs that usually help people with this condition include alpha blockers and beta blockers. In people with hormone-producing paragangliomas, it is critical to control blood pressure before surgery or other therapy.

• #13 Extra-dural sporadic sympathetic paraganglioma: A rare care report – IJN

  https://www.ijnonline.org/html-article/22118

  Paraganglioma are rare tumor derived from either parasympathetic or sympathetic paraganglia. These tumors have wide spectrum of clinical presentation, and variable anatomic distribution, however their location in the neck and skull base is common but a sporadic location is rare. […] Early suspicion is important because theses lesion can have catastrophic bleeding during surgery and can also leads to various emergencies like hypertensive crisis, seizure disorder and cardiac vascular complications. […] These tumors are not having any pathognomonic imaging therefore initially misdiagnosed. […] Clinically these tumors present with very nonspecific symptoms that may be due to pressure effect of tumor or active metabolic nature. Metabolically active lesions cause hypertension, palpitation cardiac arrhythmias, sweating and weight loss and pyrexia of unknown origin. Our patient is presented with uncontrolled hypertension due to sympathetic nature of lesion which was confirmed by 24 hours urine normetanephrine level.

• #14 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. If possible, the treatment of choice for the condition is paraganglioma surgery, either open or laparoscopic. […] When surgery is not an option due to multiple tumor sites, metastatic disease, or the location of the paraganglioma, chemotherapy, radiation or radiopharmaceutical therapy are also treatment options. […] Surgery to remove a paraganglioma is the preferred treatment option. During surgery, the tissue and lymph nodes close to the tumor may be checked and if the tumor has spread, these tissues may also be removed. Drugs may be given before, during, and after surgery to keep blood pressure and heart rate normal. […] After surgery, the blood or urine are checked to ensure metanephrine levels and subsequently, catecholamine levels have returned to normal. Normal metanephrine levels are a sign that all the paraganglioma cells were removed, if the tumor was secreting catecholamines.

• #15 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. […] Your OHC doctor will help you determine the best care plan for you. […] Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed. […] Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed.

• #16 Fluorescence-guided minimally-invasive resection of abdominal paragangliomas using indocyanine green | Scientific Reports

  https://www.nature.com/articles/s41598-024-54718-1

  This retrospective study explores the utility of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) in enhancing the intraoperative identification and guidance for the resection of abdominal paragangliomas. […] The study demonstrates the feasibility and potential clinical value of fluorescence-guided minimally-invasive resections of abdominal paragangliomas using a single intravenous ICG dose. […] Treatment is in baseline indicated for all patients diagnosed with paraganglioma, especially in case of patients with catecholamine excess related symptoms. Curative treatment primarily consists of complete resection and, in case of functional lesions, preoperative alpha-blockade to mitigate potential catecholamine release effects during surgery. […] We believe our findings add the growing body of literature examining the potential benefits of intraoperative ICG fluorescence guidance in minimally-invasive resections. Specifically, ICG-FGS could be a valuable tool, enabling surgeons to receive real-time assistance during the minimally-invasive resection of abdominal paragangliomas.

• #17 Childhood Pheochromocytoma and Paraganglioma Tumors | Dana-Farber Cancer Institute

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

  At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our pediatric cancer specialists are deeply committed to providing your child with the best possible care. Our integrated Endocrine-Oncology Program team includes experienced medical oncologists, surgeons, and researchers who work together to advance the treatment of pheochromocytoma and paraganglioma tumors. […] We typically treat pheochromocytoma and paraganglioma tumors with surgery. We perform surgical procedures to biopsy the tumor and remove the tumor and nearby tissue. We use a minimally invasive approach, such as laparoscopy or thoracoscopy, whenever possible. […] After treatment, we will continue to care for your child through our pediatric cancer survivorship programs. Support services include patient and family education, support groups, and counseling. […] Our team of endocrine tumor specialists includes oncologists, surgeons, endocrinologists, nurses, and support staff.

• #18 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Postoperatively, patients are closely monitored in the intensive care unit for hemodynamic fluctuations along with a careful assessment of electrolyte and endocrine abnormalities. To our best knowledge, most of the published articles focus primarily on preoperative and intraoperative care of PPGL patients, whereas studies detailing postoperative management are only available from individual case reports. It is extremely important for physicians of PPGL patients to provide not only appropriate preoperative evaluation and treatment but also adequate postoperative care. […] In this article, we describe the medical approaches to treat these patients after tumor resection based on our unique, long-standing experience with these patients at the National Institutes of Health. Additionally, we present the notable complications physicians should become aware of, including those emergencies that require immediate attention by a well-trained and experienced endocrinologist working alongside intensivists. Finally, this article provides clinical caveats to practicing clinicians regarding postoperative management of these patients.

• #19 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PPGL, patients may develop perplexing and substantially devastating cardiovascular complications during the perioperative period. These complications include hypertension, hypotension, arrhythmias, myocardial infarction, heart failure, and cerebrovascular accident. Other complications seen in the postoperative period include fever, hypoglycemia, cortisol deficiency, urinary retention, etc. In the interest of safe patient care, such emergencies require precise diagnosis and treatment. Surgeons, anesthesiologists, and intensivists must be aware of the clinical manifestations and complications associated with a sudden increase or decrease in catecholamine levels and should work closely together to be able to provide appropriate management to minimize morbidity and mortality associated with PPGLs.

• #20 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Postoperatively, patients are closely monitored in the intensive care unit for hemodynamic fluctuations along with a careful assessment of electrolyte and endocrine abnormalities. To our best knowledge, most of the published articles focus primarily on preoperative and intraoperative care of PPGL patients, whereas studies detailing postoperative management are only available from individual case reports. It is extremely important for physicians of PPGL patients to provide not only appropriate preoperative evaluation and treatment but also adequate postoperative care. […] In this article, we describe the medical approaches to treat these patients after tumor resection based on our unique, long-standing experience with these patients at the National Institutes of Health. Additionally, we present the notable complications physicians should become aware of, including those emergencies that require immediate attention by a well-trained and experienced endocrinologist working alongside intensivists. Finally, this article provides clinical caveats to practicing clinicians regarding postoperative management of these patients.

• #21 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PPGL, patients may develop perplexing and substantially devastating cardiovascular complications during the perioperative period. These complications include hypertension, hypotension, arrhythmias, myocardial infarction, heart failure, and cerebrovascular accident. Other complications seen in the postoperative period include fever, hypoglycemia, cortisol deficiency, urinary retention, etc. In the interest of safe patient care, such emergencies require precise diagnosis and treatment. Surgeons, anesthesiologists, and intensivists must be aware of the clinical manifestations and complications associated with a sudden increase or decrease in catecholamine levels and should work closely together to be able to provide appropriate management to minimize morbidity and mortality associated with PPGLs.

• #22 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Before surgery the patient must be adequately blocked with medication. Going under anesthesia without being blocked is very dangerous. Anesthesia can cause tumors to release massive amounts of catecholamines. […] It is extremely important that practitioners involved in the care of the patient have experience with pheo para surgery and that patients be blocked for the best possible outcome. […] Urine or plasma tests should be repeated 2-8 weeks after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients. Yearly urine or plasma tests for paraganglioma should be performed for life to detect remaining disease, return of the disease, or the development of metastases. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing.

• #23 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paraganglioma can be treated with MIBG, which is a radiopharmaceutical that is injected into the patients bloodstream. […] Somatostatin is a naturally occurring hormone that acts by binding to somatostatin receptor (SSTR), a receptor that is overexpressed in pheo para. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. […] Ablation is a treatment to remove or destroy a body part or tissue or its function. […] Embolization therapy is a treatment to block the artery leading to the adrenal gland. […] TKI is a targeted therapy treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. […] Participation in clinical trials for those with progressive disease is critical to finding better treatments for pheo para.

• #24 Paraganglioma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/paraganglioma?content_id=CON-20308105

  Surgery can be done to remove a paraganglioma. Even if a paraganglioma can’t be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible. […] Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can’t be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body. […] Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body. […] Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

• #25 Paraganglioma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/paraganglioma?content_id=CON-20308105

  Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn’t an option. Targeted therapy also may be used if the tumor spreads to other parts of the body. […] Clinical trials are studies of new treatments or new ways of using older treatments. If you’re interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying. […] Sometimes, healthcare professionals recommend against starting paraganglioma treatment right away. Instead, they may prefer to closely watch your condition with regular healthcare checkups. This is known as watchful waiting. For example, watchful waiting may be an option if a paraganglioma grows slowly and doesn’t cause symptoms.

• #26 Paraganglioma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/paraganglioma?content_id=CON-20308105

  Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn’t an option. Targeted therapy also may be used if the tumor spreads to other parts of the body. […] Clinical trials are studies of new treatments or new ways of using older treatments. If you’re interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying. […] Sometimes, healthcare professionals recommend against starting paraganglioma treatment right away. Instead, they may prefer to closely watch your condition with regular healthcare checkups. This is known as watchful waiting. For example, watchful waiting may be an option if a paraganglioma grows slowly and doesn’t cause symptoms.

• #27 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Before surgery the patient must be adequately blocked with medication. Going under anesthesia without being blocked is very dangerous. Anesthesia can cause tumors to release massive amounts of catecholamines. […] It is extremely important that practitioners involved in the care of the patient have experience with pheo para surgery and that patients be blocked for the best possible outcome. […] Urine or plasma tests should be repeated 2-8 weeks after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients. Yearly urine or plasma tests for paraganglioma should be performed for life to detect remaining disease, return of the disease, or the development of metastases. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing.

• #28 Pheochromocytoma and Paraganglioma – Missouri Cancer Associates

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

  Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue. […] Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma. […] Patients with pheochromocytoma and paraganglioma that cause signs or symptoms are treated with drug therapy. […] Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. […] Six types of standard treatment are used: Surgery, Radiation therapy, Chemotherapy, Ablation therapy, Embolization therapy, Targeted therapy. […] For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. […] Patients with pheochromocytoma or paraganglioma need lifelong follow-up.

• #29 Pheochromocytoma & Paraganglioma Treatment | NY | Montefiore Einstein Comprehensive Cancer Center

  https://montefioreeinstein.org/cancer/types/endocrine-system/pheochromocytoma-paraganglioma-treatment

  Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma. […] All patients who are diagnosed with pheochromocytoma or paraganglioma should have genetic counseling to find out their risk for having an inherited syndrome and other related cancers. […] Treatment for pheochromocytoma and paraganglioma may cause side effects. […] Follow-up care will be needed. […] For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. […] For patients with inherited pheochromocytoma or paraganglioma, catecholamine levels in the blood and urine will be checked on a regular basis. Other screening tests will be done to check for other tumors that are linked to the inherited syndrome.

• #30 Paraganglioma: Symptoms, Causes and Treatments

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

  If surgery is successful, it should resolve the symptoms. […] If the paraganglioma is not causing symptoms, the care team may recommend a watch-and-wait approach. […] A paraganglioma that has turned cancerous and metastasized is a challenge. Treatment options are limited. […] After treatment for a paraganglioma, the patient will likely need yearly testing to check for elevated hormone levels from a cancer recurrence.

• #31 Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas | Oncology Nursing Society

  https://www.ons.org/publications-research/cjon/25/2/hereditary-neuroendocrine-tumors-providing-comprehensive-care

  Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. […] In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation. […] Individuals with a germline pathogenic variant associated with paragangliomas and pheochromocytomas require extensive monitoring starting at a young age and continuing throughout their life. […] Extensive education and support are provided by nurses so that patients understand the importance of surveillance and reporting of any symptoms suggestive of excessive catecholamine release.

• #32 Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas | Oncology Nursing Society

  https://www.ons.org/publications-research/cjon/25/2/hereditary-neuroendocrine-tumors-providing-comprehensive-care

  Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. […] In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation. […] Individuals with a germline pathogenic variant associated with paragangliomas and pheochromocytomas require extensive monitoring starting at a young age and continuing throughout their life. […] Extensive education and support are provided by nurses so that patients understand the importance of surveillance and reporting of any symptoms suggestive of excessive catecholamine release.

• #33 Paragangliomas – ThinkGenetic Foundation

  https://thinkgenetic.org/diseases/paragangliomas-109959/

  Consultation with a medical geneticist or genetic counselor is recommended before genetic testing so that all of the options and possible test results can be discussed ahead of time. This individual will also help explain the test results and coordinate any follow-up as needed. […] Paragangliomas often occur in the head and neck region because they wrap around the nerves. Because of this paragangliomas often press on the wind pipe. This pressure can make breathing difficult. […] If a person has a personal history of paragangliomas or a family history of paragangliomas, they should consider pursuing genetic counseling and testing. […] Paragangliomas are benign tumors. If they are not causing any problems, then there is no need for treatment. However, if a paraganglioma is identified that is causing a medical issue (difficulty breathing, hearing loss, etc) or producing hormones treatment is required.

• #34 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  It is highly recommended for metastatic patients to receive treatment from an experienced, multi-disciplinary pheo para team. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. […] The importance of an experienced multi-disciplinary team is especially critical in the case of pregnant patients. […] If you or your partner have a genetic form of pheo para, your child will have a 50/50 chance of inheriting the genetic mutation. […] If you have had or currently have pheo para, or if you have the metastatic form of the illness, planning is critical to ensure the best possible outcomes for mother and baby. […] For patients who are postponing surgery to remove the tumor(s) until after delivery, there are many factors to consider when planning for delivery such as the number of previous pregnancies, previous delivery method, whether symptoms are well-controlled with medication, and your personal preference. […] If your medical team suggested waiting until after you give birth to surgically remove your tumor(s), it will most likely be scheduled after you recover from delivery.

• #35 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  It is highly recommended for metastatic patients to receive treatment from an experienced, multi-disciplinary pheo para team. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. […] The importance of an experienced multi-disciplinary team is especially critical in the case of pregnant patients. […] If you or your partner have a genetic form of pheo para, your child will have a 50/50 chance of inheriting the genetic mutation. […] If you have had or currently have pheo para, or if you have the metastatic form of the illness, planning is critical to ensure the best possible outcomes for mother and baby. […] For patients who are postponing surgery to remove the tumor(s) until after delivery, there are many factors to consider when planning for delivery such as the number of previous pregnancies, previous delivery method, whether symptoms are well-controlled with medication, and your personal preference. […] If your medical team suggested waiting until after you give birth to surgically remove your tumor(s), it will most likely be scheduled after you recover from delivery.

• #36 Diagnostic features and therapeutic strategies for malignant paraganglioma in a patient: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i27/9834.htm

  Paragangliomas and extra-adrenal pheochromocytomas are uncommon neuroendocrine tumors with ubiquitous distribution. Malignant paraganglioma is a relatively rare entity. […] Clinical management of paraganglioma is challenging for endocrinologists and oncologists. Prospective studies are required to develop standardized therapeutic strategies for malignant paragangliomas. […] The traditional treatment for malignant paragangliomas is surgical excision of the primary lesion. Surgery followed by adjuvant radiation is used less frequently, and chemotherapy is typically reserved for cases with distant metastasis. […] There is a lack of standardized therapeutic strategies for malignant paragangliomas. […] Management strategies for malignant paragangliomas include a combination of surgical resection, drug therapy to control symptoms of catecholamine overdose (metyrosine), radionuclide therapy (131I-MIBG or somatostatin analogue), chemotherapy (cyclophospamide, vincristine combined with dacarbazine), and external irradiation therapy. Most of these treatments are palliative, and targeted gene therapy based on tumor gene expression needs to be further explored. […] The poor characterization of the histological behavior and natural history of the disease makes clinical management of paraganglioma a great challenge for endocrinologists and oncologists.

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

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

  Treatment of metastatic pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. […] Treatment of recurrent pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer.

• #38 Diagnostic features and therapeutic strategies for malignant paraganglioma in a patient: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i27/9834.htm

  Paragangliomas and extra-adrenal pheochromocytomas are uncommon neuroendocrine tumors with ubiquitous distribution. Malignant paraganglioma is a relatively rare entity. […] Clinical management of paraganglioma is challenging for endocrinologists and oncologists. Prospective studies are required to develop standardized therapeutic strategies for malignant paragangliomas. […] The traditional treatment for malignant paragangliomas is surgical excision of the primary lesion. Surgery followed by adjuvant radiation is used less frequently, and chemotherapy is typically reserved for cases with distant metastasis. […] There is a lack of standardized therapeutic strategies for malignant paragangliomas. […] Management strategies for malignant paragangliomas include a combination of surgical resection, drug therapy to control symptoms of catecholamine overdose (metyrosine), radionuclide therapy (131I-MIBG or somatostatin analogue), chemotherapy (cyclophospamide, vincristine combined with dacarbazine), and external irradiation therapy. Most of these treatments are palliative, and targeted gene therapy based on tumor gene expression needs to be further explored. […] The poor characterization of the histological behavior and natural history of the disease makes clinical management of paraganglioma a great challenge for endocrinologists and oncologists.

• #39 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  It is highly recommended for metastatic patients to receive treatment from an experienced, multi-disciplinary pheo para team. […] There are currently no cures for metastatic paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. […] The importance of an experienced multi-disciplinary team is especially critical in the case of pregnant patients. […] If you or your partner have a genetic form of pheo para, your child will have a 50/50 chance of inheriting the genetic mutation. […] If you have had or currently have pheo para, or if you have the metastatic form of the illness, planning is critical to ensure the best possible outcomes for mother and baby. […] For patients who are postponing surgery to remove the tumor(s) until after delivery, there are many factors to consider when planning for delivery such as the number of previous pregnancies, previous delivery method, whether symptoms are well-controlled with medication, and your personal preference. […] If your medical team suggested waiting until after you give birth to surgically remove your tumor(s), it will most likely be scheduled after you recover from delivery.

• #40

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] Women with PPGL in pregnancy are more commonly delivered by Caesarean section, although vaginal delivery appears to be safe in selected cases. […] Since optimal outcomes are only achieved by early recognition of PPGL in (or ideally before) pregnancy, it is incumbent for clinicians to be aware of this diagnosis in a pregnant woman with hypertension occurring before 20 weeks gestation, and acute and/or refractory hypertension particularly if paroxysmal and accompanied by sweating, palpitations and/or headaches.

• #41

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] Women with PPGL in pregnancy are more commonly delivered by Caesarean section, although vaginal delivery appears to be safe in selected cases. […] Since optimal outcomes are only achieved by early recognition of PPGL in (or ideally before) pregnancy, it is incumbent for clinicians to be aware of this diagnosis in a pregnant woman with hypertension occurring before 20 weeks gestation, and acute and/or refractory hypertension particularly if paroxysmal and accompanied by sweating, palpitations and/or headaches.

• #42

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  All women with a past history of PPGL and/or heritable PPGL syndrome should be carefully assessed for the presence of residual or recurrent disease before considering pregnancy. […] Once diagnosed, pregnant women with PPGL should ideally be managed in expert centres with multidisciplinary access to high-level obstetric and neonatal care, endocrinologists and anaesthesiologists experienced in the management of catecholaminergic crises. […] There are four components to managing PPGL in pregnancy: alpha-blockade, PPGL surgery, obstetric care and genetic testing for hereditary PPGL syndromes. […] Adequate alpha-adrenoceptor blockade for at least two weeks has been associated with improved outcomes. […] Achieving adequate control of hypertension in pregnancy is crucial for maintaining uteroplacental circulation and avoiding IUGR or fetal demise.

• #43

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of PPGL is usually regarded an indication for surgical resection, after appropriate alpha-blockade. […] Timing of such surgery remains controversial for PPGLs diagnosed in pregnancy. […] In recent reviews, more than 2/3 of pregnancies associated with PPGLs were delivered by Caesarean section. […] All women diagnosed with PPGL in pregnancy should be referred for genetic counselling and testing for hereditary PPGL syndromes.

• #44

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  All women with a past history of PPGL and/or heritable PPGL syndrome should be carefully assessed for the presence of residual or recurrent disease before considering pregnancy. […] Once diagnosed, pregnant women with PPGL should ideally be managed in expert centres with multidisciplinary access to high-level obstetric and neonatal care, endocrinologists and anaesthesiologists experienced in the management of catecholaminergic crises. […] There are four components to managing PPGL in pregnancy: alpha-blockade, PPGL surgery, obstetric care and genetic testing for hereditary PPGL syndromes. […] Adequate alpha-adrenoceptor blockade for at least two weeks has been associated with improved outcomes. […] Achieving adequate control of hypertension in pregnancy is crucial for maintaining uteroplacental circulation and avoiding IUGR or fetal demise.

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

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

  Children with PGLs and PCCs are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Endocrine-Oncology Program. Our integrated pediatric oncology service offers — in one specialized program — the combined expertise of a leading cancer center and a premier children’s hospital. We build a team to treat your child consisting of oncologists, endocrinologists, genetic counselors, and surgeons. […] Treatment for paraganglioma or pheochromocytoma will depend on the location and type of your child’s tumor. Your child’s doctor may recommend surgery including a biopsy, laparoscopy, or thoracoscopy. Sometimes before removing a pheochromocytoma, your child’s physician may prescribe medicine to control high blood pressure. There are also some newer agents that are being investigated to reduce the need for big surgeries for advanced paragangliomas.

• #46 Childhood Pheochromocytoma and Paraganglioma Tumors | Dana-Farber Cancer Institute

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

  At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our pediatric cancer specialists are deeply committed to providing your child with the best possible care. Our integrated Endocrine-Oncology Program team includes experienced medical oncologists, surgeons, and researchers who work together to advance the treatment of pheochromocytoma and paraganglioma tumors. […] We typically treat pheochromocytoma and paraganglioma tumors with surgery. We perform surgical procedures to biopsy the tumor and remove the tumor and nearby tissue. We use a minimally invasive approach, such as laparoscopy or thoracoscopy, whenever possible. […] After treatment, we will continue to care for your child through our pediatric cancer survivorship programs. Support services include patient and family education, support groups, and counseling. […] Our team of endocrine tumor specialists includes oncologists, surgeons, endocrinologists, nurses, and support staff.

• #47

  https://www.nicklauschildrens.org/conditions/hereditary-paraganglioma-pheochromocytoma-syndrome

  Children with hereditary paraganglioma-pheochromocytoma syndrome are often under frequent monitoring due to their high risk of developing cancer. […] When cancer occurs, it is often treated with surgery to remove the tumor, as well as chemotherapy and radiation therapy to rid the body of cancer. […] It is important to have annual follow by a comprehensive Neuro-Oncology program in order to have blood and urine tests as well as ultrasound or MRI studies that can detect these tumors at an early stage.

• #48 A Child with Paraspinal Paraganglioma: A Rare Case Presentation

  https://www.wjoes.com/abstractArticleContentBrowse/WJOES/28019/JPJ/fullText

  Aim: To manage a rare case of paraspinal paraganglioma in an 8-year-old female. […] Managing pediatric patients with safe outcomes is a challenge. […] Biochemical evaluation, perioperative care with adequate -blockade, and timely follow-up is necessary for best outcomes in functional paraspinal paraganglioma. […] Preoperative -blockade helps maintain perioperative and postoperative hemodynamic stability by allowing the chronically contracted extravascular space to expand and prevent catastrophic chronic vagal tone suppression. […] Paraspinal paragangliomas lie close to major vascular and neural structures. Hence, proper surgical planning with a multidisciplinary team is mandatory. […] Timely follow-up is necessary to rule out any recurrence or residual disease.

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

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

  Children with PGLs and PCCs are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Endocrine-Oncology Program. Our integrated pediatric oncology service offers — in one specialized program — the combined expertise of a leading cancer center and a premier children’s hospital. We build a team to treat your child consisting of oncologists, endocrinologists, genetic counselors, and surgeons. […] Treatment for paraganglioma or pheochromocytoma will depend on the location and type of your child’s tumor. Your child’s doctor may recommend surgery including a biopsy, laparoscopy, or thoracoscopy. Sometimes before removing a pheochromocytoma, your child’s physician may prescribe medicine to control high blood pressure. There are also some newer agents that are being investigated to reduce the need for big surgeries for advanced paragangliomas.

• #50 Pheochromocytoma & Paraganglioma | Mays Cancer Center

  https://cancer.uthscsa.edu/cancer-care/types-and-treatments/pheochromocytoma-and-paraganglioma

  Mays Cancer Center, home to UT Health San Antonio MD Anderson Cancer Center, delivers leading pheochromocytoma and paraganglioma (pheo para) care. […] We deliver comprehensive services to diagnose, treat and manage pheo para. […] Genetic testing shows whether you or your family members carry mutations for pheochromocytoma or paraganglioma. […] Your personalized care plan depends on many factors: […] If you are at risk but do not have tumors, we provide ongoing monitoring to check for the early signs of pheo para. […] If there are tumors, and you can have surgery, you undergo a procedure to remove them. […] If there are tumors and you are not eligible for surgery, you may receive other treatments, such as chemotherapy or clinical trials. […] If tumors come back after treatment, we explore additional treatment options, which may include clinical trials. […] Our team includes cancer experts from different medical specialties who share a commitment to delivering precise, personalized care.

• #51 Center of Excellence for Care of Pheochromocytoma and Paraganglioma. – News & Views

  https://pendia.peds.uiowa.edu/2021/11/center-of-excellence-for-care-of-pheochromocytoma-and-paraganglioma/

  The University of Iowa Hospitals and Clinics has been designated a Center of Excellence for Care of patients with Pheochromocytoma and Paraganglioma. […] The tumors can usually be successfully treated, but require careful expert care to avoid severe complications. […] Our center of excellence represents a multidisciplinary collaboration between pediatric endocrinology, adult endocrinology, oncologists, surgical specialists, and clinical genetics.

• #52 Treating patients with complex adrenal issues and rare pheochromocytomas and paragangliomas | Ohio State Medical Center

  https://wexnermedical.osu.edu/departments/innovations/endocrinology/complex-adrenal-issues

  Patients with adrenal disorders rare, complicated or straightforward are finding the highest quality multidisciplinary care and treatment within the Comprehensive Adrenal Program at The Ohio State University Wexner Medical Center. […] The mission of the Comprehensive Adrenal Program at the Ohio State Wexner Medical Center is to deliver innovative, evidence-based clinical care to patients and to advance the scientific understanding of benign and malignant adrenal disorders. […] To earn the Center of Excellence distinction, the program demonstrated that it provides patients with multidisciplinary care and actively participates in paraganglioma and pheochromocytoma research. […] Kirschner and his colleagues in the Comprehensive Adrenal Program treat a wide variety of adrenal issues, including: Pheochromocytomas and paragangliomas.

• #53 Treating patients with complex adrenal issues and rare pheochromocytomas and paragangliomas | Ohio State Medical Center

  https://wexnermedical.osu.edu/departments/innovations/endocrinology/complex-adrenal-issues

  To address all its patients care needs, the Comprehensive Adrenal Program offers access to experts within endocrinology, oncology and many other fields and disciplines. […] Our Comprehensive Adrenal Program offers patients access to clinical trials with researchers who are looking at new therapeutics for adrenal cancer. […] The specialists with the Comprehensive Adrenal Program at the Ohio State Wexner Medical Center strive to offer lifechanging care for people who have adrenal cancers and complicated adrenal issues.

• #54 Pheochromocytoma and Paraganglioma | Holden

  https://cancer.uiowa.edu/cancer-types/pheochromocytoma-and-paraganglioma

  Holden Comprehensive Cancer Center is a leader in pheo para care and in the research of new pheo para treatments. […] A team of experts dedicated to pheo para. Pheo para is a complicated disease that requires a team working in close collaboration. Our care teams include adult and pediatric specialists in abdominal, chest, head and neck surgery, endocrinology, oncology, radiation oncology, nuclear medicine, and genetic counseling. The pheo para team meets regularly to discuss patient care plans, treatment, research, and more. […] Genetic testing and counseling is available for adult and pediatric patients. Our genetic counselors provide diagnosis and work closely with our specialized care teams to help develop screening and care plans for patients who are affected by, or at risk for, hereditary paraganglioma syndrome.

• #55 Pheochromocytoma and Paraganglioma | Holden

  https://cancer.uiowa.edu/cancer-types/pheochromocytoma-and-paraganglioma

  Nurse practitioners. Cassie Doolady, ARNP, DNP, Nurse Practitioner. Melissa Tvedte, ARNP, Nurse Practitioner. Lindsey Grieser, ARNP, Nurse Practitioner. […] Nurse Coordinator. Kimberly D. Miller, RN, BSN, OCN, Nurse Coordinator. Kelley McLaughlin, RN, Nurse Coordinator. Joan Ricks McGillin, RN, Nurse Coordinator. Alicia Rock-Cleppe, RN, BSN, Nurse Coordinator.

• #56 Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas | Oncology Nursing Society

  https://www.ons.org/publications-research/cjon/25/2/hereditary-neuroendocrine-tumors-providing-comprehensive-care

  Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. […] In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation. […] Individuals with a germline pathogenic variant associated with paragangliomas and pheochromocytomas require extensive monitoring starting at a young age and continuing throughout their life. […] Extensive education and support are provided by nurses so that patients understand the importance of surveillance and reporting of any symptoms suggestive of excessive catecholamine release.

• #57 Paragangliomas and Other Skull Base Tumors ENT Treatment in Utah – ENT Center of Utah

  https://entcenterutah.com/adult-care/head-neck-oncology/paragangliomas-skull-tumors/

  People with paragangliomas need care by an experienced, multidisciplinary team. Ask your doctor if he or she regularly treats people with this condition, as most doctors rarely (if ever) encounter paragangliomas and are unfamiliar with the best approaches to diagnosing and treating this rare tumor. Under such conditions, it is important to seek a second opinion from a team that specializes in the care of people with rare neuroendocrine tumors such as paragangliomas. Our Head Neck Surgeons have extensive experience in managing these tumors. […] Treatment options include: Medications. Your doctor will first attempt to control symptoms caused by your paraganglioma, such as high blood pressure, if you have the type of paraganglioma that produces hormones. Drugs that usually help people with this condition include alpha blockers and beta blockers. In people with hormone-producing paragangliomas, it is critical to control blood pressure before surgery or other therapy.

• #58 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PPGL, patients may develop perplexing and substantially devastating cardiovascular complications during the perioperative period. These complications include hypertension, hypotension, arrhythmias, myocardial infarction, heart failure, and cerebrovascular accident. Other complications seen in the postoperative period include fever, hypoglycemia, cortisol deficiency, urinary retention, etc. In the interest of safe patient care, such emergencies require precise diagnosis and treatment. Surgeons, anesthesiologists, and intensivists must be aware of the clinical manifestations and complications associated with a sudden increase or decrease in catecholamine levels and should work closely together to be able to provide appropriate management to minimize morbidity and mortality associated with PPGLs.

• #59 Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

  https://www.mdpi.com/2072-6694/11/7/936

  Postoperatively, patients are closely monitored in the intensive care unit for hemodynamic fluctuations along with a careful assessment of electrolyte and endocrine abnormalities. To our best knowledge, most of the published articles focus primarily on preoperative and intraoperative care of PPGL patients, whereas studies detailing postoperative management are only available from individual case reports. It is extremely important for physicians of PPGL patients to provide not only appropriate preoperative evaluation and treatment but also adequate postoperative care. […] In this article, we describe the medical approaches to treat these patients after tumor resection based on our unique, long-standing experience with these patients at the National Institutes of Health. Additionally, we present the notable complications physicians should become aware of, including those emergencies that require immediate attention by a well-trained and experienced endocrinologist working alongside intensivists. Finally, this article provides clinical caveats to practicing clinicians regarding postoperative management of these patients.

• #60 Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas | Oncology Nursing Society

  https://www.ons.org/publications-research/cjon/25/2/hereditary-neuroendocrine-tumors-providing-comprehensive-care

  Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. […] In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation. […] Individuals with a germline pathogenic variant associated with paragangliomas and pheochromocytomas require extensive monitoring starting at a young age and continuing throughout their life. […] Extensive education and support are provided by nurses so that patients understand the importance of surveillance and reporting of any symptoms suggestive of excessive catecholamine release.

• #61 Paraganglioma Clinic | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/screening-prevention/paraganglioma-clinic

  Our team of experts is committed to making sure patients with paragangliomas and/or genetic risk for these tumors receive the best possible care. […] The Paraganglioma Clinic helps patients and their families create personal screening plans. We also help schedule follow-up care for people at high risk for paragangliomas. Our health care team includes specialists in every area of paraganglioma care. Our goal is to provide a cohesive and patient-centered approach. […] We recommend all people with a personal diagnosis of paraganglioma or pheochromocytoma get genetic counseling and testing.

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