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)
• Anxiety 
• Abdominal discomfort or pain
• Constipation 
• Weight loss

• 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]

  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]

  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. [medlineplus.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]

  Classic clinical features are due to excess sympathetic nervous system stimulation and involve episodic blood pressure crises with paroxysmal headaches, diaphoresis, heart palpitations, and pallor. [amboss.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. [medlineplus.gov]

  Other less common signs and symptoms include: pale skin (pallor), low blood pressure, blurred vision, weight loss, increased thirst and urination, constipation, abdominal pain, elevated blood sugar and white blood cell counts, psychiatric disturbances [endocrinediseases.org]

• 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]

  DO call your health care provider if you have vision changes, severe headache, weakness on one side of the body, chest pains, or increasing palpitations. [carle.org]

  […] hypertensive paroxysms, associated with chest pressure, unilateral flushing, dizziness, weakness, tachycardia, and polyuria. [archinte.ama-assn.org]

  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]

• 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]

  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]

• Fatigue

  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]

  Fatigue, food intolerances, medicine in tolerances make life difficult but every day is a gift and I've come so far. [raredr.com]

  Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs. High-salt diet. [mayoclinic.org]

  Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs. [drugs.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]

  She had also experienced dyspnea and palpitation followed by chest discomfort. Each time, she was admitted to the hospital with high blood pressure and heart rate. But the physical exam between attacks was normal. [jdmdonline.biomedcentral.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]

  Weight gain or loss • Overdoing activities (e.g. exercising, shopping) • Skin breakouts (hives, eczema) • Overreacting to unexpected problems • Loss of sex drive • Picking fights with others • Frequent colds • Pallor or blushing • Profuse sweating • Tachypnea [ncbi.nlm.nih.gov]

• 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]

  Pulmonary manifestations include pulmonary edema and acute respiratory distress syndrome. [intechopen.com]

  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 [drugs.com]

  An unusual cause of respiratory distress and hypotension following removal of a pheochromocytoma. Can J Anaesth 49, 1099–1100 (2002). 14. Streeten, D. H. P. & Anderson, G. H. [nature.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]

• Vomiting

  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]

  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]

• 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]

  Other less common signs and symptoms include: pale skin (pallor), low blood pressure, blurred vision, weight loss, increased thirst and urination, constipation, abdominal pain, elevated blood sugar and white blood cell counts, psychiatric disturbances [endocrinediseases.org]

  Other possible symptoms include: Anxiety attacks Fever Irregular heartbeat Extreme paleness in the face Shortness of breath Tremors or shakiness Nausea Vomiting Clammy skin Constipation Tingling fingers Vision disturbances Chest or stomach pain Weight [cancer.net]

• 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 present two cases of bladder pheochromocytoma, one of them presented with vague abdominal pain and the other with hematuria. Biphasic CT in both the cases showed hypervascular intravesical mass suggestive of bladder pheochromocytoma. [doi.org]

• Polydipsia

  Patients can present other symptoms: papilledema, dyspnea, pallor, general weakness, panic spells, orthostatic hypotension, blurred vision, papilledema, weight loss, polyuria, polydipsia, constipation, globular sedimentation speed, hyperglycemia, leukocytosis [scielo.org.mx]

• Hypertension

  Hypertension. 1995;25266- 271 Google Scholar Crossref [archinte.ama-assn.org]

  Pheochromocytoma is a rare catecholamine-secreting neoplasm that is the cause of hypertension in 0.2% of patients with hypertension. [ncbi.nlm.nih.gov]

  Examination Again, the most common features are in bold: Hypertension but it may be paroxysmal in 50%. Postural hypotension. Tremor. Hypertensive retinopathy. Pallor. Fever. [patient.info]

• 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 set of symptoms include: Headaches Heart palpitations Sweating High blood pressure As the tumor grows, the attacks often increase in frequency, length, and severity. [medlineplus.gov]

• Tachycardia

  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]

• 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]

• Orthostatic Hypotension

  And also patients with orthostatic hypotension sense sinus tachycardia on standing. [medicaljoyworks.com]

  The definition of orthostatic hypotension was different among studies. The sensitivity was 23–50 %. [jdmdonline.biomedcentral.com]

  An adrenergically mediated process is suggested by the combination of paroxysmal hypertension, orthostatic hypotension and tachyarrhythmias. [cancertherapyadvisor.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]

  These agents do not produce hypotension or orthostatic hypotension; therefore, they may be used safely in patients who are normotensive but have occasional episodes of paroxysmal hypertension. [onlinelibrary.wiley.com]

• Flushing

  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]

  Before her initial visit to the emergency department, the patient had severe episodic hypertension accompanied by heaviness in her chest, palpations, sweating, flushing, shortness of breath, and tinnitus. [jaoa.org]

  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]

• Cafe-Au-Lait Spots

  He had four cafe au lait spots on his trunk and several cutaneous and subcutaneous soft tissue lesions over his forehead, neck and extremities. His father had a history of hypertension diagnosed in his 50s. [mayoclinic.org]

  For women presenting with hypertension and cafe-au-lait spots and cutaneous neurofibromas, think pheochromocytoma. [cancertherapyadvisor.com]

  The subject should be examined for cafe au lait spots if neurofibromatosis is suspected. [clinicalpainadvisor.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]

• 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]

  Other less common signs and symptoms include: pale skin (pallor), low blood pressure, blurred vision, weight loss, increased thirst and urination, constipation, abdominal pain, elevated blood sugar and white blood cell counts, psychiatric disturbances [endocrinediseases.org]

  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]

• Hypertensive Retinopathy

  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]

  retinopathy Weight loss Pallor Fever Tremor Neurofibromas Tachyarrhythmias Pulmonary edema Cardiomyopathy Ileus Café au lait spots See Clinical Presentation for more detail. [emedicine.com]

• Anxiety Disorder

  Pheochromocytoma is a rare disease and the prevalence of pheochromocytoma in individuals with anxiety disorder is unknown. [edm.bioscientifica.com]

  Other considerations include: Anxiety disorder. Carcinoid tumour. Alcohol withdrawal. Labile hypertension. Drug abuse. Factitious phaeochromocytoma - has been described. [patient.info]

  Venlafaxine is a drug commonly used in daily clinical practice for the treatment of major depressive episodes, generalized anxiety disorders, and social phobia. [elsevier.es]

  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]

• Aggressive Behavior

  A PASS score of 4 indicates a tumor with potentially aggressive behavior (PASS 4). [intechopen.com]

  However, specific histologic features help to differentiate adrenal pheochromocytomas with a potential for biologically aggressive behavior from those that behave in a benign fashion. [emedicine.com]

• 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]

  Other presenting findings that occur in children include weight loss, nausea and vomiting, polyuria, visual disturbances and anxiety. [clinicalpainadvisor.com]

  Patients can present other symptoms: papilledema, dyspnea, pallor, general weakness, panic spells, orthostatic hypotension, blurred vision, papilledema, weight loss, polyuria, polydipsia, constipation, globular sedimentation speed, hyperglycemia, leukocytosis [scielo.org.mx]

• 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]

• 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]

• 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]

  Patients with pheochromocytoma may experience blood pressures high enough to cause a stroke or heart attack in patients. This study is designed to take patients suspected of having pheochromocytoma and confirm the diagnosis. [clinicaltrials.gov]

  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]

  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]

• 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]

  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]

  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]

• Dizziness

  Hypertensive episodes were accompanied by severe physical symptoms, such as headache, chest pain, dizziness, nausea, palpitations, flushing, and diaphoresis. [archinte.ama-assn.org]

  Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs. High-salt diet. [mayoclinic.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]

  DO call your health care provider if you have ankle swelling, shortness of breath, or weakness or dizziness when standing. DO call your health care provider if symptoms return after surgery. [carle.org]

  The other symptoms (dizziness, chest pain, dyspnea) are not independent. So we cannot simply multiply them to calculate the final LR. By considering the value of signs and symptoms, we decided to move forward in our evaluation. [jdmdonline.biomedcentral.com]

• 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. [medlineplus.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 Treatment Treatment involves removing the tumor with surgery. [account.allinahealth.org]

  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]

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]

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.

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].

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.

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].

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].

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.

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.

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.

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.
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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.