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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:

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 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 14-year-old female student presented with cough, hemoptysis and dyspnea for one week was reported.[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]
  • Symptoms Signs and symptoms of pheochromocytomas often include: High blood pressure Heavy sweating Headache Rapid heartbeat (tachycardia) Tremors Paleness in the face (pallor) Shortness of breath (dyspnea) Less common signs or symptoms may include: Anxiety[mayoclinic.org]
Respiratory Distress
  • Untreated, high blood pressure associated with pheochromocytomas can result in a number of critical conditions, including: Heart disease Stroke Kidney failure Acute respiratory distress Damage to the nerves of the eye Cancerous (malignant) tumors Rarely[mayoclinic.org]
Tachypnea
  • Symptoms Patients presenting with pheochromocytoma usually come in with high blood pressure, diaphoresis, pallor, tachycardia, tachypnea, and severe headache.[symptoma.com]
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]
  • In persons with tumours that secrete an appreciable amount of catecholamines, anxiety may be increased, and the patient may experience weight loss and develop hyperglycemia and diabetes mellitus .[britannica.com]
  • […] 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 Increased appetite Weight[uvahealth.com]
Pallor
  • 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 include: Headaches Heart palpitations Sweating attacks Facial flushing or pallor Blood sugar control problems Constipation or abdominal distention Sudden low blood pressure upon standing Advanced treatments for pheochromocytoma Treatments[cancercenter.com]
  • 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]
  • […] criteria for research PET imaging in children ---Children over 10 years old with very high suspicion of sporadic or familial pheochromocytoma/paraganglioma (e.g. the presence of new onset of hypertension or hypertensive episodes, sweating, headaches, pallor[clinicaltrials.gov]
Fever
  • To explore the fever of unknown origin (FUO) in patients with interleukin-6 (IL-6)-producing pheochromocytoma. 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]
  • 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]
  • Presenting signs include pallor, orthostatic hypotension, tremor and syncope as well as abdominal pain, diarrhea other gastrointestinal manifestations, hyperglycemia, low-grade fever and behavioral disturbances.[clinicalpainadvisor.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, etc.) family history of pheochromocytoma/paraganglioma or genetic mutations known to predispose individuals to develop these tumors, or the presence of a tumor on conventional imaging /ultrasounds, CT, MRI.[clinicaltrials.gov]
  • 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]
  • Thus, blacks who have paroxysmal episodes or resistant hypertension should be screened for pheochromocytoma. Diagnosis Pregnancy occurs in an age range where vigilance to detect secondary hypertension should always be emphasized.[hyper.ahajournals.org]
Swelling
  • We report on a 68-year-old survivor of pheochromocytoma multisystem crisis, whose clinical course was triggered inadvertently by a short innocuous course of oral dexamethasone to suppress inflammation and swelling after a left orbital floor fracture repair[ncbi.nlm.nih.gov]
  • One year postoperatively, abdominal computed tomography revealed a local recurrence and retroperitoneal lymph node swelling. The local recurrence was positive for MIBG uptake, whereas the swollen retroperitoneal lymph nodes were negative.[ncbi.nlm.nih.gov]
  • Thyroid echography demonstrated no thyroid swelling (23   43 mm). A whole body computed tomography was performed for systemic evaluation. This revealed exophthalmos and a mass of size 57   64 mm in the anterior pararenal space.[ncbi.nlm.nih.gov]
  • Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs.[drugs.com]
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]
  • 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 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming.[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]
  • These hormones normally regulate the body's response to stress, and when released into the blood, they cause increases in heart rate and blood pressure, headaches, nausea, abdominal pain and palpitations.[montefiore.org]
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]
  • 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 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming.[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]
  • 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]
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]
  • She presented to us again in December 2016 with abdominal pain. Examination revealed hypertension with postural drop.[ncbi.nlm.nih.gov]
  • The patient felt abdominal pain and his abdominal ultrasound showed suspicious right adrenal gland tumor.[ncbi.nlm.nih.gov]
  • These hormones normally regulate the body's response to stress, and when released into the blood, they cause increases in heart rate and blood pressure, headaches, nausea, abdominal pain and palpitations.[montefiore.org]
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]
  • Other symptoms include: Headaches Heart palpitations Sweating attacks Facial flushing or pallor Blood sugar control problems Constipation or abdominal distention Sudden low blood pressure upon standing Advanced treatments for pheochromocytoma Treatments[cancercenter.com]
  • 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]
  • […] 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]
  • […] 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]
Hypertension
  • 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]
  • Hypertension secondary to pheochromocytoma is often paroxysmal, and patients occasionally present with sudden attacks of alternating hypertension and hypotension.[ncbi.nlm.nih.gov]
  • It classically presents with paroxysmal headaches, hypertension, palpitations and sweating related to catecholamine excess.[ncbi.nlm.nih.gov]
  • Four patients had 1 dose reduction due to the following reasons: fatigue (1), abnormal liver tests (2), hypertension and (Takotsubo) cardiomyopathy (1), and headaches (1).[ncbi.nlm.nih.gov]
  • Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma.[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]
  • 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]
  • It classically presents with paroxysmal headaches, hypertension, palpitations and sweating related to catecholamine excess.[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 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]
  • 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]
  • Symptoms Patients presenting with pheochromocytoma usually come in with high blood pressure, diaphoresis, pallor, tachycardia, tachypnea, and severe headache.[symptoma.com]
  • To control tachycardia, continuous intravenously administered propranolol and diltiazem infusions were given. At the same time, small doses of doxazosin and carvedilol were used for both alpha and beta adrenergic blockade.[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]
  • 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, and[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]
  • Patients present clinically with the pharmacologic effects of excess catecholamines, manifested by episodic, postural, paroxysmal and/or labile hypertension, headaches, diaphoresis, palpitations, chest pain, and anxiety.[journals.lww.com]
Orthostatic Hypotension
  • The definition of orthostatic hypotension was different among studies. The sensitivity was 23–50 %.[jdmdonline.biomedcentral.com]
  • 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]
  • Orthostatic hypotension is undesirable preoperatively: fluid boluses should be administered to render the child euvolemic. c. Pulmonary N/A d. Renal-GI: N/A e. Neurologic: N/A f. Endocrine: Adrenal.[clinicalpainadvisor.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]
  • 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]
  • Other symptoms include: Headaches Heart palpitations Sweating attacks Facial flushing or pallor Blood sugar control problems Constipation or abdominal distention Sudden low blood pressure upon standing Advanced treatments for pheochromocytoma Treatments[cancercenter.com]
  • 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]
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]
Polyuria
  • Other presenting findings that occur in children include weight loss, nausea and vomiting, polyuria, visual disturbances and anxiety.[clinicalpainadvisor.com]
Anxiety Disorder
  • 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]
Headache
  • 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]
  • Four patients had 1 dose reduction due to the following reasons: fatigue (1), abnormal liver tests (2), hypertension and (Takotsubo) cardiomyopathy (1), and headaches (1).[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]
  • 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]
  • 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]
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]
  • 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]
  • 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]
  • Pheochromocytomas secrete adrenaline in an uncontrolled fashion and can cause serious health problems including stroke, heart attack, and even death. Some physicians call pheochromocytoma a "pharmacological time bomb" because of its danger.[endocrinediseases.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]
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]
  • Symptoms of pheochromocytomas can include: High blood pressure (including sporadic or episodic high blood pressure) Elevated or pounding heartbeat Heart palpitations Profound sweating Severe headache Tremors Paleness in the face Shortness of breath Flank[my.clevelandclinic.org]
  • The following common presenting signs 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[symptoma.com]
  • […] excessive hormone production and include: Headaches that typically are severe Excessive sweating Accelerated or racing heartbeat, called tachycardia, and palpitations Anxiety and/or nervousness, such as feelings of impending death Nervous shaking or tremors[ucsfhealth.org]
Seizure
  • 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]
  • As Brian explains in this interview, the years of high blood pressure, emergency visits, seizures, sporadic episodes of excessive strength and fatigue were not relieved until he was able to get a proper diagnosis and have the tumor surgically removed.[raredr.com]
  • Signs and symptoms of this: headache, vision changes, neuro changes, seizures, shortness of breath Monitor for chest pain (risk for MI), neuro status (stroke), EKG changes, hyperglycemia Provide a calm and cool environment….no overstimulation ![registerednursern.com]
  • Skin findings observed in Noonan patients include: Lymphedema 1 Prominent pads of fingers and toes (67%) 1 Follicular keratosis of the face and extensor surfaces (14%) 1 Multiple lentigines (3%) 1 Neurologic findings include the following: Hypotonia 1 Seizure[centogene.com]
Confusion
  • Flushing or feeling hot Excessive sweating Lightheadedness, especially when arising from a sitting position Chest or abdominal pain Tingling, burning, numbness in the legs and feet Constipation Weight loss Nervousness, irritability, or anxiety Mental confusion[healthcommunities.com]
  • ., visual disturbances, confusion, hematuria) is present, it is called a hypertensive emergency.[endocrinologyadvisor.com]
  • Pheochromocytoma has been called the “Great Mimic” since its manifestations can resemble so many other conditions, which may confuse clinicians [ 14 ].[jdmdonline.biomedcentral.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.
  7. Elenkova A, Matrozova J, Zacharieva S, Kirilov G, Kalinov K. Adiponectin - A possible factor in the pathogenesis of carbohydrate metabolism disturbances in patients with pheochromocytoma. Cytokine. Jun 2010; 50(3):306-10.
  8. Rivers G, Rosas AL, Quezado Z, Manger WM, Eisenhofer G, Pacak K. Adverse drug reactions in patients with phaeochromocytoma: incidence, prevention and management. Drug Saf. 2007; 30(11):1031-62.
  9. Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection.Eur J Endocrinol. 2012 Feb; 166(2):143-50. Epub 2011 Sep 2.
  10. Wong C, Yu R. Preoperative preparation for pheochromocytoma resection: physician survey and clinical practice. Exp Clin Endocrinol Diabetes. Jul 2010; 118(7):400-4. 

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Last updated: 2018-06-22 07:38