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Pheochromocytoma

PCC

Pheochromocytoma is an adrenal tumor that excessively secretes catecholamines. The secretion of catecholamines from the chromaffin cells of the adrenals causes paroxysmal or persistent hypertension among patients. The catecholamines secreted from the adrenal tumor includes epinephrine, norepinephrine, and dopamine.


Presentation

The following common presenting signs and symptoms are seen in patients diagnosed with pheochromocytoma:

Weight Loss
  • We describe a case of one of the largest documented pheochromocytomas resected in the United States, an 18-cm tumor in a patient who presented with exertional dyspnea, abdominal pain, constipation, weight loss, and intermittent hypertension.[ncbi.nlm.nih.gov]
  • A 9 years old boy presented to our hospital complaining of night sweat and weight loss (5kg) for 3 months followed by cough, nausea, vomiting and malaise for two weeks. During that time he was found to have continuous hypertension.[ncbi.nlm.nih.gov]
  • A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2.[ncbi.nlm.nih.gov]
  • This cancer type is often discovered after the onset of symptoms, notably pain or a feeling of fullness, resulting in weight loss.[cancercenter.com]
  • Other symptoms that may occur include: Abdominal or chest pain Irritability, nervousness Pallor Weight loss Nausea and vomiting Shortness of breath Seizures Problems sleeping Treatment involves removing the tumor with surgery.[nlm.nih.gov]
Pallor
  • BACKGROUND: Pheochromocytoma is an endocrine tumor that causes hypertension, facial pallor, and headache. Pheochromocytoma patients rarely present with acute heart failure or cardiogenic shock.[ncbi.nlm.nih.gov]
  • Symptoms Patients presenting with pheochromocytoma usually come in with high blood pressure, diaphoresis, pallor, tachycardia, tachypnea, and severe headache.[symptoma.com]
  • Other symptoms that may occur include: Abdominal or chest pain Irritability, nervousness Pallor Weight loss Nausea and vomiting Shortness of breath Seizures Problems sleeping Treatment involves removing the tumor with surgery.[nlm.nih.gov]
  • It may be constant, mimicking the common forms of hypertension, or episodic and associated with headache, excessive perspiration, heart palpitation, pallor, tremour, and anxiety.[britannica.com]
Weakness
  • A 26-year-old pregnant woman with a history of paroxysmal hypertension during the second trimester presented with lower extremity weakness, numbness, urinary incontinence, and back pain.[ncbi.nlm.nih.gov]
  • Other conditions that may accompany these classic symptoms are as follows: anxiety, nausea, tremors, weakness, abdominal pain, and weight loss. Some people, however, never develop symptoms of a pheochromocytoma.[medicinenet.com]
  • Involuntary trembling Pain in the lower chest or upper abdomen Warmth, flushing Increased appetite Weight loss Insomnia High blood pressure, which can be constant or sporadic Tingling, burning or numbness in the legs and feet Shortness of breath Muscle weakness[uvahealth.com]
  • […] hypertensive paroxysms, associated with chest pressure, unilateral flushing, dizziness, weakness, tachycardia, and polyuria.[archinte.ama-assn.org]
  • […] of Zuckerkandl which is close to the origin of the inferior mesenteric artery, bladder wall, heart, mediastinum, and carotid and glomus jugulare bodies Clinical Findings Headaches, palpitations and diaphoresis with hypertension, frequently episodic Weakness[learningradiology.com]
Fever
  • OBJECTIVE: To explore the fever of unknown origin (FUO) in patients with interleukin-6 (IL-6)-producing pheochromocytoma. METHODS: Patients with pheochromocytoma were enrolled from June 2014 to April 2017.[ncbi.nlm.nih.gov]
  • He was subsequently investigated for fever and found to have a 3cm right sided adrenal mass consistent with a pheochromocytoma. After confirmation and appropriate blockade laparoscopic adrenalectomy was performed.[ncbi.nlm.nih.gov]
  • He later developed high fever with core temperature reaching a peak of 42.2 C, rapid breathing, and died after unsuccessful attempts to stabilize him.[ncbi.nlm.nih.gov]
  • Excessive amounts of these hormones may cause: High blood pressure (hypertension) Rapid pulse Palpitations Anxiety attacks Fever Headaches Nausea and vomiting Clammy skin Diagnosis and treatment Specialists at the UPMC Neuroendocrine Cancer Treatment[upmc.com]
  • Clinical presentation includes headaches, palpitations, chest and abdominal pain, hypertension, fever, and tremor. Microscopically, a characteristic nesting (zellballen) growth pattern is usually seen.[fpnotebook.com]
Resistant Hypertension
  • Download Our Free Treatment Guide Resistant Hypertension What is a pheochromocytoma? A pheochromocytoma is a rare, usually noncancerous (benign) tumor that develops in cells in the center of an adrenal gland (glands that sit above the kidney).[my.clevelandclinic.org]
  • Currently Browsing: Nephrology Resistant Hypertension Program The Resistant Hypertension Program at Massachusetts General Hospital specializes in the evaluation, diagnosis and management of patients with resistant or difficult to treat hypertension.[massgeneral.org]
  • hypertension from obstructive sleep apnea Treatment In this sequence of events α-antagonist phenoxybenzamine to prevent unopposed α-action if β receptors are blocked β-blocker tumor resection Prognosis, Prevention, and Complications Prognosis very good[medbullets.com]
Dyspnea
  • We describe a case of one of the largest documented pheochromocytomas resected in the United States, an 18-cm tumor in a patient who presented with exertional dyspnea, abdominal pain, constipation, weight loss, and intermittent hypertension.[ncbi.nlm.nih.gov]
  • A 14-year-old female student presented with cough, hemoptysis and dyspnea for one week was reported.[ncbi.nlm.nih.gov]
  • Abstract A 24-year-old woman at 38 weeks of gestation with no past medical history was transferred to our hospital because of acute onset of severe dyspnea.[ncbi.nlm.nih.gov]
  • A 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming.[ncbi.nlm.nih.gov]
  • […] functional chromaffinoma, usually benign, derived from adrenal medullary tissue cells and characterized by the secretion of catecholamines, resulting in hypertension, which may be paroxysmal and associated with attacks of palpitation, headache, nausea, dyspnea[medical-dictionary.thefreedictionary.com]
Tachypnea
  • Symptoms Patients presenting with pheochromocytoma usually come in with high blood pressure, diaphoresis, pallor, tachycardia, tachypnea, and severe headache.[symptoma.com]
  • Common symptoms and signs are Tachycardia Diaphoresis Postural hypotension Tachypnea Cold and clammy skin Severe headache Angina Palpitations Nausea and vomiting Epigastric pain Visual disturbances Dyspnea Paresthesias Constipation A sense of impending[msdmanuals.com]
Respiratory Distress
  • Her hospital course was complicated by multi-organ injury, including acute respiratory distress syndrome requiring venous-venous extracorporeal membrane oxygenation, non-ST elevation myocardial infarction, cardiogenic shock, acute liver failure, and oliguric[ncbi.nlm.nih.gov]
Nausea
  • Here, we present the case of an adult male with an unusual manifestation of pheochromocytoma in the form of acute pain abdomen with nausea and abdominal guarding, mimicking acute peritonitis. He had fluctuating blood pressure recordings.[ncbi.nlm.nih.gov]
  • We describe the case of a 26-year-old woman with a medical history significant for headaches who presented initially to her family physician with nausea, vomiting, headache and hypertension. She was started on lisinopril 10 mg daily.[ncbi.nlm.nih.gov]
  • A 9 years old boy presented to our hospital complaining of night sweat and weight loss (5kg) for 3 months followed by cough, nausea, vomiting and malaise for two weeks. During that time he was found to have continuous hypertension.[ncbi.nlm.nih.gov]
  • A previously healthy 36-year-old woman presented to the ED with headache and nausea.[ncbi.nlm.nih.gov]
  • Excessive amounts of these hormones may cause: High blood pressure (hypertension) Rapid pulse Palpitations Anxiety attacks Fever Headaches Nausea and vomiting Clammy skin Diagnosis and treatment Specialists at the UPMC Neuroendocrine Cancer Treatment[upmc.com]
Vomiting
  • The patient complained of vomiting and breathlessness. Computed tomography showed a shadow-like region with a similar appearance to interstitial pneumonia.[ncbi.nlm.nih.gov]
  • We describe the case of a 26-year-old woman with a medical history significant for headaches who presented initially to her family physician with nausea, vomiting, headache and hypertension. She was started on lisinopril 10 mg daily.[ncbi.nlm.nih.gov]
  • A 9 years old boy presented to our hospital complaining of night sweat and weight loss (5kg) for 3 months followed by cough, nausea, vomiting and malaise for two weeks. During that time he was found to have continuous hypertension.[ncbi.nlm.nih.gov]
  • Excessive amounts of these hormones may cause: High blood pressure (hypertension) Rapid pulse Palpitations Anxiety attacks Fever Headaches Nausea and vomiting Clammy skin Diagnosis and treatment Specialists at the UPMC Neuroendocrine Cancer Treatment[upmc.com]
  • A 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming.[ncbi.nlm.nih.gov]
Abdominal Pain
  • The patient presented with abdominal pain and had a history of hypertension. CASE REPORT A 64-year-old man with hypertension presented with a clinical history of intermittent abdominal pain for one year.[ncbi.nlm.nih.gov]
  • We describe a case of one of the largest documented pheochromocytomas resected in the United States, an 18-cm tumor in a patient who presented with exertional dyspnea, abdominal pain, constipation, weight loss, and intermittent hypertension.[ncbi.nlm.nih.gov]
  • A 45-year-old man presented with the sudden onset of left lateral abdominal pain, headache, chest discomfort, high blood pressure, and adrenal hemorrhaging on enhanced abdominal computed tomography.[ncbi.nlm.nih.gov]
  • Classic symptoms related to PH are hypertension, abdominal pain, diaphoresis, and headache; but it can be easily misdiagnosed as gestational hypertension or preeclampsia.[ncbi.nlm.nih.gov]
  • She presented to us again in December 2016 with abdominal pain. Examination revealed hypertension with postural drop.[ncbi.nlm.nih.gov]
Constipation
  • We describe a case of one of the largest documented pheochromocytomas resected in the United States, an 18-cm tumor in a patient who presented with exertional dyspnea, abdominal pain, constipation, weight loss, and intermittent hypertension.[ncbi.nlm.nih.gov]
  • In people who have them, symptoms may include: Severe headaches Excessive sweating Fast heart rate Sensation of a panic attack Blurred vision Nausea, vomiting, constipation Pounding heart beat Chest pain Involuntary trembling Pain in the lower chest or[uvahealth.com]
  • […] who have them, pheochromocytoma may cause: High blood pressure, which can be constant or sporadic Severe headaches Excessive sweating Warmth, flushing Fast heart rate and pounding heart beat Sensation of a panic attack Blurred vision Nausea, vomiting, constipation[winchesterhospital.org]
  • […] and symptoms are seen in patients diagnosed with pheochromocytoma: Elevated blood pressure Severe headache Tremors Tachypnea Pallor of the face Tachycardia and distinct heart sounds Diaphoresis (excessive sweating) Anxiety Abdominal discomfort or pain Constipation[symptoma.com]
  • But most people have at least one of the following as well: Constipation Dizziness when standing Nausea Pale skin Racing heartbeat ( heart palpitations ) Severe headache Stomach, side, or back pain Unusual sweating These symptoms can come on suddenly,[webmd.com]
Hypertension
  • Pheochromocytoma is a rare catecholamine-secreting neoplasm that is the cause of hypertension in 0.2% of patients with hypertension.[ncbi.nlm.nih.gov]
  • Hypertension. 1995;25266- 271 Google Scholar Crossref[archinte.ama-assn.org]
  • We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes.[ncbi.nlm.nih.gov]
  • KEYWORDS: Hypertension; neurofibromatosis type 1; obstetric labor complications; pheochromocytoma; pregnancy[ncbi.nlm.nih.gov]
  • Hypertension secondary to pheochromocytoma is often paroxysmal, and patients occasionally present with sudden attacks of alternating hypertension and hypotension.[ncbi.nlm.nih.gov]
Palpitations
  • Common symptoms include sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations. This article describes a patient with an extra-adrenal pheochromocytoma who presented with symptoms associated with voiding.[ncbi.nlm.nih.gov]
  • The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis.[ncbi.nlm.nih.gov]
  • Pheochromocytoma, a neuroendocrine tumor of the adrenal medulla, arising from chromaffin cells, usually presents with the clinical triad of paroxysmal headache, profuse sweating and palpitations, associated with labile hypertension.[ncbi.nlm.nih.gov]
  • Paroxysmal episodes of serious hypertension, headache, palpitation, and diaphoresis are the typical manifestations (Bravo, 2004).[ncbi.nlm.nih.gov]
  • The classic triad of symptoms is headache, palpitations, and diaphoresis, but clinical presentation varies greatly. Pheochromocytoma can also mimic acute coronary syndrome and heart failure.[ncbi.nlm.nih.gov]
Tachycardia
  • We describe the case of a 67-year-old man presenting with ventricular tachycardia (VT) and systolic heart failure secondary to a left adrenal phaeochromocytoma. After treatment with amiodarone, the patient's VT resolved.[ncbi.nlm.nih.gov]
  • The typical symptoms are episodic in nature and include tachycardia, sweating and headache. These tumours can present as transient, reversible cardiomyopathy similar to takotsubo cardiomyopathy (TCM).[ncbi.nlm.nih.gov]
  • The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors.[ncbi.nlm.nih.gov]
  • She developed acute hypertensive emergency (acute agitation, worsening headache, chest pain and wide complex tachycardia) when her blood pressure (BP) increased to 223/102mmHg (initial BP, 134/86mmHg) after receiving intravenous MCP.[ncbi.nlm.nih.gov]
  • Five days later, the patient had ventricular tachycardia, and severe hypertension, remarkable blood pressure fluctuation between 224/76 and 70/50 mm Hg.[ncbi.nlm.nih.gov]
Chest Pain
  • Chest pain with elevated serum troponin is a common clinical presentation and is normally managed as suspected myocardial infarction or acute coronary syndrome (ACS).[ncbi.nlm.nih.gov]
  • She developed acute hypertensive emergency (acute agitation, worsening headache, chest pain and wide complex tachycardia) when her blood pressure (BP) increased to 223/102mmHg (initial BP, 134/86mmHg) after receiving intravenous MCP.[ncbi.nlm.nih.gov]
  • Abstract Pheochromocytomas are neuroendocrine tumors, and its cardiac involvement may include transient myocardial dysfunction, acute coronary syndrome (ACS), and even ventricular arrhythmias.A patient was referred for evaluation of stuttering chest pain[ncbi.nlm.nih.gov]
  • After a preprocedural pain block for elective right shoulder arthroscopy, a 53-year-old hypertensive white man developed chest pain. In the operating room, he had increased blood pressure.[ncbi.nlm.nih.gov]
  • A 69-year-old Japanese man developed acute-onset episodic headaches, palpitations, and chest pains.[ncbi.nlm.nih.gov]
Orthostatic Hypotension
  • The heart rate increases in an attempt to increase the blood pressure as a “reflex” response, and these medications can causes orthostatic hypotension.[registerednursern.com]
  • A non-selective alpha blocker such as phenoxybenzamine (Dibenzyline) may be prescribed to normalize blood pressure, but this type of drug can cause orthostatic hypotension and reflex tachycardia and may increase postsurgical complications.[americannursetoday.com]
  • hypotension, a fall in systolic blood pressure greater than 20 mmHg or a fall in diastolic blood pressure greater than 10 mmHg on making the patient stand Palpitations Anxiety often resembling that of a panic attack Diaphoresis Headaches Pallor Weight[psychology.wikia.com]
Blurred Vision
  • In people who have them, symptoms may include: Severe headaches Excessive sweating Fast heart rate Sensation of a panic attack Blurred vision Nausea, vomiting, constipation Pounding heart beat Chest pain Involuntary trembling Pain in the lower chest or[uvahealth.com]
  • In people who have them, pheochromocytoma may cause: High blood pressure, which can be constant or sporadic Severe headaches Excessive sweating Warmth, flushing Fast heart rate and pounding heart beat Sensation of a panic attack Blurred vision Nausea,[winchesterhospital.org]
  • 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.[columbiasurgery.org]
Flushing
  • A 32-year-old 22-week pregnant hypertensive woman with sporadic episodes of headaches, sweating, and facial flushing was diagnosed with pheochromocytoma through biochemical and imaging tests.[ncbi.nlm.nih.gov]
  • CASE REPORT: We presented a 49-year-old female patient with a history of daily paroxysmal hypertension accompanied with flushing of the face and upper chest, palpitations and excessive sweating prior to micturition.[ncbi.nlm.nih.gov]
  • Pheochromocytomas are usually benign (not cancer) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.[fpnotebook.com]
  • […] symptoms may include: Severe headaches Excessive sweating Fast heart rate Sensation of a panic attack Blurred vision Nausea, vomiting, constipation Pounding heart beat Chest pain Involuntary trembling Pain in the lower chest or upper abdomen Warmth, flushing[uvahealth.com]
  • Symptoms: Severe headache Palpitations Rapid heart rate Sweating Flushing Chest pain Abdominal pain Nervousness Irritability Increased appetite Loss of weight Additional symptoms that may be associated with this disease: Sleeping difficulty Hand tremor[uclahealth.org]
Increased Sweating
  • Between the attacks, people with pheochromocytoma can experience the following: increased sweating cold hands and feet weight loss constipation Diagnosis If a pheochromocytoma is suspected, urine and/or a blood tests are usually recommended.[encyclopedia.com]
  • Between the attacks, people with pheochromocytoma can experience the following: increased sweating cold hands and feet weight loss constipation Diagnosis If a pheochromocytoma is suspected, urine and/or a blood test are usually recommended.[medical-dictionary.thefreedictionary.com]
Hyperhidrosis
  • […] level changes in the circulation, more occipital than temporal • Hypermetabolism due to chronic stimulation of sympathetic receptors in adipocytes, hepatocytes and various tissues • Hyperglycemia due to inhibition of insulin secretion by the pancreas • Hyperhidrosis[wikilectures.eu]
Anxiety Disorder
  • Other considerations include: Anxiety disorder . Carcinoid tumour . Alcohol withdrawal . Labile hypertension. Drug abuse . Factitious phaeochromocytoma - has been described.[patient.info]
  • Other considerations include: Anxiety disorder. Carcinoid tumour. Alcohol withdrawal. Labile hypertension. Drug abuse. Factitious phaeochromocytoma - has been described.[patient.info]
  • Differential diagnosis Edit The differential diagnosis of pheochromocytoma includes: Anxiety disorders Paragangliomas Essential hypertension Hyperthyroidism Insulinoma Paroxysmal supraventricular tachycardia Renovascular hypertension Treatment Edit Surgical[psychology.wikia.com]
  • A psychiatric consultant diagnosed the patient as having an "anxiety disorder, not otherwise specified."[archinte.ama-assn.org]
Urinary Incontinence
  • A 26-year-old pregnant woman with a history of paroxysmal hypertension during the second trimester presented with lower extremity weakness, numbness, urinary incontinence, and back pain.[ncbi.nlm.nih.gov]
Polyuria
  • […] investigation had revealed no cause or objective findings; a 3-year history of labile hypertension; and a 6-month history of daily symptomatic hypertensive paroxysms, associated with chest pressure, unilateral flushing, dizziness, weakness, tachycardia, and polyuria[archinte.ama-assn.org]
Headache
  • We describe the case of a 26-year-old woman with a medical history significant for headaches who presented initially to her family physician with nausea, vomiting, headache and hypertension. She was started on lisinopril 10 mg daily.[ncbi.nlm.nih.gov]
  • A previously healthy 36-year-old woman presented to the ED with headache and nausea.[ncbi.nlm.nih.gov]
  • She also had a history of pulsatile bi-temporal headache for 2 years prior to her referral to us. She underwent complete abdominal and pelvic ultrasound imaging for her flank pain.[ncbi.nlm.nih.gov]
  • Common symptoms include sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations. This article describes a patient with an extra-adrenal pheochromocytoma who presented with symptoms associated with voiding.[ncbi.nlm.nih.gov]
  • The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis.[ncbi.nlm.nih.gov]
Stroke
  • Too much of either one can lead to serious conditions, such as high blood pressure and stroke. Most pheochromocytomas start in the adrenal medulla, in the adrenal glands.[mskcc.org]
  • Pheochromocytomas should never be tested through a biopsy, because this can release large amounts of hormone, which can lead to a severe increase in blood pressure, causing a stroke.[montefiore.org]
  • If it is not treated, it can lead to a heart attack, stroke, and cause the arteries to work poorly. What Happens Under Normal Conditions? The adrenal glands are found above each kidney.[urologyhealth.org]
  • High blood pressure that is left untreated can cause heart disease, stroke and other major health problems. Tumors that develop outside the adrenal glands are called paragangliomas.[ucsfhealth.org]
  • Other problems: seizures, stroke and intracerebral hemorrhage also may occur because of uncontrolled hypertension. Race Pheochromocytomas are diagnosed in people of all races, but are less common in African Americans.[science.nichd.nih.gov]
Tremor
  • The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors.[ncbi.nlm.nih.gov]
  • Clinical presentation includes headaches, palpitations, chest and abdominal pain, hypertension, fever, and tremor. Microscopically, a characteristic nesting (zellballen) growth pattern is usually seen.[fpnotebook.com]
  • Other conditions that may accompany these classic symptoms are as follows: anxiety, nausea, tremors, weakness, abdominal pain, and weight loss. Some people, however, never develop symptoms of a pheochromocytoma.[medicinenet.com]
  • Tremor . Hypertensive retinopathy. Pallor. Fever. Acute hypertension with a tumour that releases predominantly noradrenaline (norepinephrine) may cause reflex bradycardia. Neurofibromas may be felt and café au lait patches may be seen.[patient.info]
  • Tremor. Hypertensive retinopathy. Pallor. Fever. Acute hypertension with a tumour that releases predominantly noradrenaline (norepinephrine) may cause reflex bradycardia. Neurofibromas may be felt and café au lait patches may be seen.[patient.info]
Seizure
  • Other symptoms that may occur include: Abdominal or chest pain Irritability, nervousness Pallor Weight loss Nausea and vomiting Shortness of breath Seizures Problems sleeping Treatment involves removing the tumor with surgery.[nlm.nih.gov]
  • Other problems: seizures, stroke and intracerebral hemorrhage also may occur because of uncontrolled hypertension. Race Pheochromocytomas are diagnosed in people of all races, but are less common in African Americans.[science.nichd.nih.gov]
  • Other symptoms that may occur include: Abdominal or chest pain Irritability, nervousness Pallor Weight loss Nausea and vomiting Shortness of breath Seizures Problems sleeping Exams and Tests The health care provider will perform a physical exam.[ufhealth.org]
  • 35 or after age 60 You have signs such as: Bad headaches Excessive sweating Racing heartbeat Paleness Anxiety and worry Nervous shaking Pain in the lower chest or upper belly area Upset stomach Weight loss Feeling overheated Fatigue Vision problems Seizures[urologyhealth.org]
Dizziness
  • Hypertensive episodes were accompanied by severe physical symptoms, such as headache, chest pain, dizziness, nausea, palpitations, flushing, and diaphoresis.[archinte.ama-assn.org]
  • Other symptoms may include: Rapid pulse Heart palpitations Headache Dizziness In children, poor weight gain despite good appetite Nausea Vomiting Abdominal pain or distension Pale skin Clammy skin Sweating The symptoms of pheochromocytoma may resemble[stanfordchildrens.org]
  • But most people have at least one of the following as well: Constipation Dizziness when standing Nausea Pale skin Racing heartbeat ( heart palpitations ) Severe headache Stomach, side, or back pain Unusual sweating These symptoms can come on suddenly,[webmd.com]
  • The most common symptoms seen are diaphoresis, palpitations, blood pressure fluctuations, and dizziness. Spikes in blood pressure can occur and can be life threatening if not treated appropriately.[nadf.us]
  • Visual disturbances, dilated pupils, lower extremity paresthesia, nausea and vomiting, dizziness, tremors, and tachycardia are also seen during paroxysm.[medical-dictionary.thefreedictionary.com]

Workup

The following diagnostic modalities and test are conducted on a patient suspected to have pheochromocytoma:

  • Epinephrine and norepinephrine assays: Blood samples or a 24 hour urine sample may be collected to determine and measure the serum catecholamine concentration.
  • Suppression testing: This medical test makes use of clonidine or phentolamine to suppress the effects of systemic epinephrine and norepinephrine in pheochromocytoma. 
  • Computed tomography (CT scan): This imaging tests that will elucidate the adrenal tumor in the abdomen. It may show size, location, and the number of adrenal tumors. 
  • Magnetic resonance imaging (MRI): This imaging modality is used demonstrate a more detailed view of the adrenal tumor above the kidneys intraabdominally.
  • M-Iodobenzylguanidine (MIBG) imaging: This is a radio-imaging technology that detects tumors that absorbs the radioactive iodine and dye injected in the circulatory system.
  • Positron emission tomography (PET): PET is used to detect the uptake of radioactive substances in active abdominal tumors.
Increased Basal Metabolic Rate
  • basal metabolic rate (burns more calories) increases thermogenesis (elevates body temperature) how you respond to stress (example: see a bear…jump and feel fear/anxiety).[registerednursern.com]

Treatment

The primary approach in the treatment of pheochromocytoma is by means of surgical removal which can be performed using a minimally invasive abdominal laparotomy or laparoscopic adrenalectomy. This procedure is most beneficial for patients having adrenal tumor which is no bigger than 8 centimeters in length. The patient’s hypertensive crisis prior to surgery is stabilized using alpha-blockers and beta-blockers to inhibit the systemic effects of epinephrine and norepinephrine [10]. Stress doses of steroids may be given if bilateral adrenalectomy is being contemplated preoperatively. Malignant pheochromocytomas are treated using radionuclide therapy, chemotherapy, and targeted cancer therapies.

Prognosis

Patients suffering from malignant pheochromocytoma have a 5-year survival rate of less than 50% in most cases. However, non-malignant pheochromocytoma will have more than 95% chance of survival within the next 5 years from diagnosis. The adrenal tumor has a 10% chance of malignant degeneration. They prompt surgical removal of the tumor (unilateral or bilateral adrenalectomy) usually results to a complete cure.

Uncontrolled pheochromocytoma may complicate to myocardial infarction, myocarditis, dilated cardiomyopathy, intracerebral hemorrhage, and pulmonary edema. Pheochromocytoma tumor occurs rarely in pregnancy with an incidence rate of 2 cases per 100,000 pregnancies. The coexistence of the adrenal tumor with pregnancy carries a grim prognosis for both mother and child [9].

Etiology

The occurrence of pheochromocytoma is generally sporadic, although 30% of the cases are linked to genetic mutations. There are at least 10 genes that are already identified that may phenotypically express as paraganglioma (extrarenal pheochromocytoma) or intraabdominal pheochromocytoma [3]. Patients diagnosed with multiple endocrine neoplasia (MEN) usually present with pheochromocytoma, a gross incidence rate of 19% of patients positively diagnosed with pheochromocytoma are associated with a concomitant MEN syndrome or Von Hippel-Landau (VHL) disease.

Epidemiology

Globally, pheochromocytoma is relatively rare and are often times underdiagnosed. Pheochromocytoma tumors occurs in just 5 to 20 patients per 10,000 diagnosed hypertensive patients. A prospective study conducted in Mayo Clinic reveals that at least 50% of the diagnosed cases of pheochromocytoma were made during autopsy [4]. In the clinics, pheochromocytoma is usually an incidental finding during imaging studies in 10% of cases [5].

Sex distribution
Age distribution

Pathophysiology

The main pathophysiology of pheochromocytoma is brought about by the oversecretion of the catecholamine hormones like norepinephrine and epinephrine from the chromaffin cells of the adrenal medulla [6]. The secreted catecholamine hormones consequentially stimulate the alpha adrenergic receptors which results in hypertension, increased cardiac contractility, increased in heart rate, intestinal relaxation, breakdown of glycogen, and gluconeogenesis [7].

The secretion of the catecholamine from the tumor remains unregulated and it is not influenced by any nerve or neural stimulation. Life-threatening hypertensive crisis in pheochromocytoma may be precipitated by anesthesia induction, intake of opiates, use of metoclopramide, tricyclic antidepressant maintenance, childbirth, and intake of beta-blockers [8].

Prevention

Pheochromocytoma has a rare occurrence and is usually difficult to diagnose; thus, proactive prevention may prove to be most difficult. Genetic counseling among afflicted patients may reduce the recurrence of the disease among the offspring. The prompt diagnosis and treatment of the adrenal tumor prevents life-threatening complications.

Summary

Pheochromocytoma is a rare, often benign tumor that usually develops in the adrenal medulla. The adrenal tumor releases catecholamine hormones that induce cardiac arrhythmias and high blood pressure [1]. Pheochromocytoma can cause other serious complications in different organ systems especially in the cardiovascular system [2]. The surgical removal of the pheochromocytoma tumor of the adrenal gland or adrenalectomy consequently reverts all symptoms to normal.

Patient Information

Definition

Pheochromocytoma is a rare, often benign tumor that usually develops in the adrenal medulla producing excessive amounts of catecholamines like epinephrine and norepinephrine.

Cause

The etiology of pheochromocytoma is usually sporadic but is sometimes linked to genetic mutations and heredity. The tumor may occur along with MEN and VHL disease.

Symptoms

Patients presenting with pheochromocytoma usually come in with high blood pressure, diaphoresis, pallor, tachycardia, tachypnea, and severe headache.

Diagnosis

The diagnosis of pheochromocytoma relies on the demonstration of the catecholamines and derivatives in the patient’s blood and urine. Imaging studies like CT scan, MRI, MIBG, and PET are implored to elucidate the adrenal tumor.

Treatment and follow-up

The primary goal in the treatment of pheochromocytoma is the surgical removal of the tumor through surgical laparotomy or laparoscopic adrenalectomy. Stress doses of corticosteroids are given preoperatively when bilateral adrenalectomy is planned. Antihypertensive medications are given to stabilize the blood pressure preoperatively. Chemotherapy and radionuclide therapy are used to treat malignant adrenal cell tumors.

References

Article

  1. Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens. Nov 2011; 29(11):2049-60.
  2. Waguespack SG, Rich T, Grubbs E, Ying AK, Perrier ND, Ayala-Ramirez M, et al. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. May 2010; 95(5):2023-37.
  3. Därr R, Lenders JWM, Hofbauer LC, Naumann B, Bornstein SR, Eisenhofer G. Pheochromocytoma: Update on Disease Management. Ther Adv in Endo and Metab. 2012; 3(1):11-26.
  4. Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc. Dec 1983; 58(12):802-4.
  5. Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. Feb 8 2007; 356(6):601-10.
  6. Eisenhofer G, Pacak K, Huynh TT, Qin N, Bratslavsky G, Linehan WM, et al. Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma. Endocr Relat Cancer. Feb 2011; 18(1):97-111.
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Last updated: 2018-06-22 07:38