Genetic counseling is part of the treatment plan for patients with. Standard imaging protocols this document includes typical imaging protocols for diagnostic imaging. However, the distinction between pheochromocytoma and paraganglioma is an important one because of implications for associated neoplasms, risk for malignancy, and genetic testing. Pheochromocytoma imaging ct scan mri mibg scan ct most costeffective, mri more sensitive, and mibg more specific mibg indications risk for multiple tumors, extraadrenal tumors, metastases young pts and those with syndromes persistentrecurrent malignant disease overall sensitivity 87%, specificity 100%. Images of pheochromocytoma in adrenal glands mcdermott. Imaging of pheochromocytoma and paraganglioma springerlink. A wide spectrum of imaging findings on all modalities has been described for. Ppt pheochromocytoma powerpoint presentation free to. The tumor is often slow growing and noncancerous benign.
Pheochromocytoma, catecholamine metabolites, surgical treatment. Diagnosis and management of pheochromocytoma kidney. These scans may be done before andor after surgical removal of pheochromocytoma. The clinical goal of our program is to establish more effective diagnostic and localization techniques to ensure that no tumor goes undiagnosed as well as to establish new treatment options for this disease. Malignant pheochromocytoma imaging with 124imibg petmr. Definition pheochromocytomas, sometimes simply referred to as pheos, are rare tumors that develop in the inner region medulla of the adrenal gland. Siemens healthcare, erlangen, germany in a malignant pheochromocytoma. For any given ct examination, the protocol will vary according the individual requirement of the imaging site. Precontrast axial ct of abdomen shows a mass in the right adrenal gland white arrow the left adrenal gland is normal yellow arrow. This procedure is also called nuclear magnetic resonance imaging nmri. Along with imaging results, laboratory testing confirmed.
Pheochromocytomas and paragangliomas are catecholamine producing tumors derived from the sympathetic or parasympathetic nervous system. Up to 30% malignant tischler et al, 2014, endocrine pathology for malignant disease, 46% progressionfree survival at 1 year hescot, 20 few markers of metastatic risk adrenal medulla. During the last decade there have been revolutionary breakthroughs in understanding the biology of pheochromocytomas and extraadrenal paragangliomas. Abstract pdf 1167 views full text 6741 views license get permission.
Pheochromocytoma is also capable of causing, at presentation, pseudoobstruction of bowels, diabetic ketoacidosis, or multisystem crisis involving lactic acidosis. It is not always possible to visualize normal adrenal glands especially on the left side with an ultrasound 11,12. A case report of a pheochromocytoma presenting with. The authors received no corporate funding or remuneration. Pheochromocytoma and paragangliomas are neuroendocrine tumors that present with symptoms of hypertension, tachycardia, sweating and anxiety among others. By presenting the key aspects of breast mri in a straightforward manner and with clear images, this practical book benefits all practitioners seeking to increase their working knowledge and. Pheochromocytoma is a rare neuroendocrine tumor, occurring in less than 0. If a mass was found by ct, then mibg scintigraphy for a mass 2 cm or mri for a mass pheochromocytomas. These symptoms may be in part due to the cosecretion of other hormones associated with norepinephrine, such as corticotropinreleasing hormone, adrenocorticotropin, and interleukin six. Choledocholithiasis and cholelithiasis with intrahepatic and extra hepatic biliary ductal dilatation but without sonographic evidence of acute cholecystitis. These tumours can be found anywhere from the neck to the pelvis in locations of sympathetic ganglions. Pheochromocytomas are an uncommon tumor of the adrenal gland, with characteristic clinical, and to a lesser degree, imaging features. Pheochromocytoma free download as powerpoint presentation. The mri protocol should include highresolution, multiplanar, t1weighted.
Imaging of pheochromocytoma and paraganglioma ncbi. Ct scan of the abdomen revealed a large heterogeneous left adrenal mass. More than 90% of pheochromocytomas arise in the adrenal gland, and 98% are. But it can invade nearby parts of the body, become cancerous malignant and spread. Imaging of pheochromocytomas and paragangliomas nature. Varying imaging protocols show a highly necrotic mass displacing the right kidney. Evaluation of adrenal tumors by magnetic resonance imaging with. Hepatobiliary imaging using a novel hepatocyteselective ct con trast agent. Discoveries of new susceptibility genes and genotypephenotype correlations have led to the realization that appropriate patient care requires a. A pheochromocytoma is a rare, catecholaminesecreting tumor derived from chromaffin cells. The diagnosis of pheochromocytomas provides a potentially correctable cause of hypertension, and their removal can. These hormones help keep your heart rate and blood pressure.
Because of their varied clinical, imaging, and pathologic appearances, accurate diagnosis can be challenging. Usefulness of imibg scintigraphy in the evaluation of. Pheochromocytoma is a rare, catecholamineproducing tumor with preferential localization in the adrenal gland presenting with severe, often therapyresistant hypertension, sweating, pallor, anxiety attacks, and headache. Clinical presentation and diagnosis of pheochromocytoma.
The radiographic approach to the patient with suspected pheochromocytoma is discussed. Their physiological thickness does not exceed 12 mm, while the sensitivity of this method is over 90% for diagnosing tumours 2 cm in size,14. This casereport is of a middleaged woman with a nonspecific clinical presentation suggesting acute coronary syndrome or subacute myocarditis. For some of the imaging studies, it is the requirement of the radiology department that within seven days of any imaging studies scan you will be required to have your blood drawn. Anaesthetic implications aliya ahmed department of anaesthesia, aga khan university hospital, karachi.
As a surgery patient, you have access to a multidisciplinary team of physicians, nurses and support staff, led by an experienced pancreatic and biliary physician. The higher sensitivity in pheochromocytoma patients, compared with paraganglioma patients, was also seen in the subgroup with con. Imaging plays an important role in the diagnosis and staging of disease. The addition of mri to mibgpet is promising as it improves tumor delineation because of the high soft tissue contrast in mri that. Small adrenal pheochromocytoma on the right side arrows. A case report of a pheochromocytoma presenting with neurological manifestations nazia shamsuddin, md, whitney jackson, md case report a 51 yearold caucasian female with a past medical history of hypertension, coronary artery disease and cerebrovascular accident presented to the emergency room with acute onset dysarthria and rightsided. Incidental right adrenal mass which is incompletely characterized by ultrasound. Protocol for the examination of specimens from patients. Review article perioperative management of pheochromocytoma. The goal is to provide optimal care to patients with complex pancreatic and biliary diseases. In approximately 60 percent of patients, the tumor is discovered incidentally during computed tomography ct or magnetic resonance imaging mri of the abdomen for unrelated symptoms. Standard imaging tests noninvestigational imaging tests include computed tomography ct, magnetic resonance imaging mri, sonography, and 123imibg scintigraphy and fdg positron emission tomography petct. She was submitted to computed tomography ct and magnetic resonance imaging mri, as shown in figures 1a and 1b, respectively.
Abstract pheochromocytoma is a catecholamine producing tumour that can cause severe hypertension and other systemic disturbances. The extraadrenal lesions are also referred to as paragangliomas. Each layer of these glands makes different hormones. Adrenal adenomas are the most common adrenal incidentaloma in patients without cancer accounting for 80% of lesions, with myelolipoma accounting for 6% and pheochromocytoma for 3%,14. This case reports on the worldwide first 124 imibgpet magnetic resonance imaging mri performed with an integrated petmr biograph mmr. Current diagnostic imaging of pheochromocytomas and implications. However, if the disease remains undiagnosed, the excessive catecholamine secretion might. The adrenal medulla plays an instrumental role in synthesizing and secreting catecholamines hormones such as epinephrine adrenaline and norepinephrine. Mr imaging reveals a large well defined mass in the right adrenal gland. Paragangliomas are tumours that arise within the sympathetic nervous system originating from the neural crest. Pheochromocytoma javad ghahremani medical student at shahid beheshti university 2. Following clinical and laboratory workup and general radiographic procedures urography with adrenal laminagraphy, arteriography with careful monitoring is indicated. Diagnostic tests pheochromocytoma and paraganglioma.
In approximately 37% of patients undergoing computed tomography ct an incidental adrenal lesion is identified. To ascertain the magnetic resonance mr imaging characteristics of pheochromocytomas and paragangliomas and to compare mr with computed tomography ct and iodine1 metaiodobenzylguanidine i1 mibg, 19 patients 18 with pheochromocytomas, one with a paraganglioma were studied. Unsuspected pheochromocytoma incidentally found on chest ct. Although in the majority of paragangliomas the diagnosis is based on measuring catecholamines and metabolites in plasma or urine, imaging plays an important preoperative role. The mainstay of definitive therapy is surgical resection. Employing these guidelines helps providers enhance quality of care and contribute to the most efficacious use of radiology. Pdf the accurate diagnosis of adult pheochromocytoma and. Pheochromocytoma epinephrine adrenal gland free 30. A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed. Ultrasonography us adrenal tumors are most commonly found during abdominal ultrasound us.
A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Adrenergic myocarditis in pheochromocytoma journal of. A small percentage can be in the mediastinum, and in the urinary bladder, with the textbook clinical presentation of the latter. This is an overview of adrenal imaging and an approach to evaluating. Imaging tests are used to assess the location of a pheochromocytoma or paraganglioma. Pheochromocytoma has been called the 10% tumor because approximately 10% are bilateral figs.
The middle part of the adrenal glands makes epinephrine and norepinephrine. T1weighted slightly hypointense to the remainder of the adrenal gland, if there is necrosis andor hemorrhage then the signal will be more heterogeneous. The clinical presentation of pheochromocytoma is variable and many biochemical and imaging methods have been suggested to improve the diagnostic accuracy of what has been termed the great masquerader. Noninvasive monitoring of a murine model of metastatic. A previous whole body planar i123 mibg scan with attenuationcorrected. The term pheochromocytoma in greek, phios means dusky, chroma means color, and cytoma means tumor refers to the color the tumor cells acquire when stained with chromium salts.
Head, neck, chest, and abdominal mri may be helpful in the diagnosis of pheochromocytoma. Pheochromocytomas are most common in the fourth through sixth decades of life. Following intravenous contrast lower photo, the right adrenal mass enhances brightly red arrow. If the lesion is indeterminate on the dedicated adrenal protocol ct, one may. The other ap proach involves positron emission tomographic pet scan. Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Among those with known prior disease, the sensitivity of 123imibg planar imaging was lower for those with paraganglioma 67% vs.
The task force included a chair selected by the endocrine society clinical guidelines subcommittee cgs, seven experts in the field, and a methodologist. Diagnosis of a pheochromocytoma columbia university. Superior to the right kidney in the area of the adrenal gland there is a wellcircumscribed, heterogenous mass which measures 4. Ma blake receives royalties from the book adrenal imaging springer verlag, 2009.