However, the distinction between pheochromocytoma and paraganglioma is an important one because of implications for associated neoplasms, risk for malignancy, and genetic testing. Following intravenous contrast lower photo, the right adrenal mass enhances brightly red arrow. A paraganglioma grows in the sympathetic or parasympathetic nerves. Introduced new biochemical and imaging approaches to this tumor, especially the use of. Fewer than 5 per cent are found in the pediatric age group 4, and little has appeared in the radiologic literature about their manifestations in childhood. Pheochromocytoma knowledge for medical students and physicians. Ae1ae3, ck7 and er are consistent with metastatic breast carcinoma. May 07, 2012 pheochromocytoma and paraganglioma imaging findings. Imaging modalities for pheochromocytoma and paraganglioma. Internal hemorrhage can sometimes be seen within the tumor fig. Best cases from the afip radiographics rsna publications online. Pheochromocytoma and paraganglioma imaging findings. Extraadrenal pheochromocytoma was first reported by alezais and peyron in 1908. These cells synthesize, store, and secrete catecholamines in response to sympathetic stimulation figures 711 and 712.
I think that we are headed directly into an era where these resources are going to become more and more significant to learning radiology and the earlier we understand how to use them, the better. Introduced new biochemical and imaging approaches to this tumor, especially the use of 18ffluorodopamine in localization of pheochromocytoma. A pheochromocytoma is a tumor arising in the adrenal gland medulla. Plain ct abdomen showed a circumscribed lobulated left adrenal mass measuring 9. Find local pheochromocytoma resources for the top u. Usually the tumour is in the medullary cells of the adrenal gland. Pheochromocytoma is a rare type of tumor that arises from certain cells known as chromaffin cells, which produce hormones necessary for the body to function properly. The diagnosis of pheochromocytomas provides a potentially correctable cause of hypertension, and their removal can. On computed tomography ct, the typical appearance of a pheochromocytoma or paraganglioma is a mass with an unenhanced density greater than 10 hounsfield units hu, avid contrast enhancement due to a rich capillary network, and delayed washout fig. A paraganglioma is a pheochromocytoma outside the vicinity of the adrenal gland. Pheochromocytomas are rare but treacherous catecholamineproducing tumors, which if missed or not properly treated, will almost invariably prove fatal. Additional considerations include pheochromocytoma if direct injection into the adrenal. Oxford university press makes no representation, express or implied, that the drug dosages in this book are correct.
The next step is the measurement of plasma fractionated metanephrines and plasma catecholamines. Study participants will undergo blood, urine, and imaging tests, described below, to detect pheochromocytoma. Rare, catecholaminesecreting, neuroendocrine tumor usually found in medulla of the adrenal gland originating in. Aberrant right subclavian artery aortic stenosis arterial placement of central line atrial septal defect atrial. The tumors arise from the chromaffin cells of the adrenal medulla and are associated with increased catecholamine production. The selfassessment component consists of six multiplechoice questions with solutions. Candidates will be screened with a medical history and physical examination, electrocardiogram, and blood and urine tests. Biochemical diagnosis of phaeochromocytoma must be established prior to undertaking imaging studies to avoid unnecessary surgery because 3% to 7% of patients over the age of 50 have nonfunctioning adrenal nodules incidentalomas 5, 6 imaging may take priority over biochemical testing in emergency situations with critically unwell patients 1, 7. The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Aoife kilcoyne would like to acknowledge the support of the mac erlaine research scholarship from the academic radiology research trust, st. The treatment of malignant pheochromocytoma is mainly based on the use of nuclear medical techniques and selected chemotherapeutic approaches.
Hyde, the first case of pheochromocytoma pcc was documented in germany and described an 18yearold woman with hypertensive crisis due to bilateral adrenal tumors. Imaging of pheochromocytoma and incidental adrenal lesions. Between those two, you will learn pretty much everything you want to know about radiology as a nonradiologist or a young radiologist. Diagnosis of a pheochromocytoma columbia university. This research did not receive a specific grant from any funding agency in the public, commercial, or notforprofit sector. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo.
Pheochromocytoma pcc diagnosis and surgery medical. Every weekday, learningradiology posts an unknown case that will help you hone your radiologic skills. Recognizing the basics with student consult online access. May 18, 2017 recurrent malignant pheochromocytoma in a 70yearold woman who underwent right adrenalectomy for pheochromocytoma in 1975 same patient as in the previous 3 images. They develop in both genders and peak in the fourth to fifth decades, although familial tumors occur at a younger age. This imaging characteristic helps distinguish pheochromocytomas from adrenal carcinomas, which are usually isointense or hyperintense to fat. Part of the medical radiology book series medrad abstract patients with catecholamineproducing tumors pheochromocytomas and paragangliomas do not need any special preparation before they receive nonionic iodinebased contrast medium or any gadoliniumbased contrast medium intravenously. Prompt diagnosis is, therefore, essential for effective treatment, usually by surgical resection. The name pheochromocytoma, proposed by pick in 1912, 14 comes from the greek words phaios dusky and chroma color. Recognizing the basics, containing new chapters on ultrasound, interventional radiology and mammography as well as online. She also complains of diarrhea for the past 2 weeks. The leading introductory radiology text for medical students and others who are required to read and interpret common radiologic images, learning radiology, 4th edition, stresses an easytofollow pattern recognition approach that teaches how to differentiate normal and abnormal images. Jun 06, 2011 a paraganglioma is a pheochromocytoma outside the vicinity of the adrenal gland.
An awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, handout notes, interactive material, most commons lists and pictorial differential diagnoses. Aug 30, 2009 central necrosis, calcification and cystic changes may be present in minority of cases. The biggest problem for pheochromocytoma is to suspect it in the first place. However, as many neuroendocrine tumors demonstrate uptake with mibg, it is not as specific for pheochromocytoma outside of the adrenal. Localization of pheochromocytoma by computed tomography. Correct and timely diagnosis of pheochromocytomas is crucial for patients.
Most pheochromocytomas originate in one of the two adrenal glands located above the kidneys in the back of the upper abdomen. The diagnosis of pheochromocytomas provides a potentially correctable cause of hypertension, and their removal can prevent hypertensive crises. Download for offline reading, highlight, bookmark or take notes while you read pheochromocytoma. Pheochromocytomas arise from the chromaffin tissue of the adrenal medulla and paraspinal sympathetic ganglia. Rare, catecholaminesecreting, neuroendocrine tumor usually found in medulla of the adrenal gland originating in the chromaffin cells. Advances in the field of molecular genetics have led to novel diagnostic and. These vascular lesions enhance uniformly after administration of contrast material. She states that the pain is crampy and colicky and moderate in severity. The radiographic approach to the patient with suspected pheochromocytoma is discussed. Pheochromocytoma may occur alone, or as part of a multiple endocrine. Pdf learning radiology download full pdf book download. 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. Mr is also excellent for evaluating intraadrenal pheochromocytoma.
Current diagnostic imaging of pheochromocytomas and implications. Pheochromocytomas are an uncommon tumor of the adrenal gland, with characteristic clinical, and to a lesser degree, imaging features. Radiodiagnosis imaging is amazinginteresting cases. Pheochromocytoma pcc diagnosis and surgery medical library. This process is experimental and the keywords may be updated as the learning algorithm improves. Most chromaffin cells are found in the adrenal gland. 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. Followup computed tomography ct scans obtained in 2000 and a metaiodobenzylguanidine uptake scan not shown demonstrate a mass adjacent to the left renal vein arrow. Patients present clinically with the pharmacologic effects of excess catecholamines, manifested by episodic, postural, paroxysmal andor labile hypertension, headaches, diaphoresis, palpitations, chest pain, and anxiety. Presence of adrenal mass is not conclusive for the diagnosis of pheochromocytoma. The extraadrenal lesions are also referred to as paragangliomas. Ossification of the posterior longitudinal ligament opll osteitis condensans ilii.
Hpi 67 year old female presents with ruq pain following large meals. A case report of a pheochromocytoma presenting with. Radiologic and clinical evaluation of pheochromocytoma in. The imaging modalities for pheochromocytomas can be either. And you could probably read the faq section like a book because you will inevitably have those scenarios pop up.
Pheochromocytoma is a tumor of the chromaffin cells of the adrenal medulla. Pheochromocytomas and paragangliomas are catecholamine producing tumors derived from the sympathetic or parasympathetic nervous system. The educational component consists of four required articles that the participant should read. Clinical presentation and diagnosis of pheochromocytoma. Diagnosis, localization, and treatment ebook written by karel pacak, graeme eisenhofer, jacques lenders. Pheochromocytoma pheochromocytoma is a rare endocrine tumor originating in the adrenal glands, specifically, the medulla of adrenal glands. The educational objectives for this selfassessment module on imaging of pheochromocytoma and incidental adrenal lesions are for the participant to exercise, selfassess, and improve his or her knowledge of the imaging evaluation of pheochromocytoma and incidental adrenal lesions. Radiology museum depicting important events in radiology history like roentgens journey of discovering xrays. A pheochromocytoma is a catecholaminesecreting tumor that typically develops in the adrenal medulla. Pheochromocytoma and paraganglioma endotext ncbi bookshelf.
In 1886, the year that robert louis stevenson published his novel strange case of dr. Precontrast axial ct of abdomen shows a mass in the right adrenal gland white arrow the left adrenal gland is normal yellow arrow. Catecholamines gene mutation identification and classification gene mutations genetic susceptibility research hypertension diagnosis care and treatment genetic aspects radiology radiology, medical. Recognizing the basics, 2nd edition, is an imagefilled, practical, and clinical introduction to this integral part of the diagnostic process.
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. Pheochromocytoma, in which symptoms are not very specific and hypertension is present in only half of patients, remains rare. Pheochromocytoma has been called the 10% tumor because approximately 10% are bilateral figs. Pheochromocytoma, tumour, most often nonmalignant, that causes abnormally high blood pressure hypertension because of hypersecretion of substances known as catecholamines epinephrine, norepinephrine, and dopamine. The manifestations are diverse and the tumor can mimic a variety of conditions, often resulting in either erroneous diagnoses or a delayed. These tumors, as it was in this case, usually present as large masses within the ad. Diagnosis of a pheochromocytoma is confirmed by biochemical evidence in the plasma or urine of catecholamine production by the tumor.
Sympathetic pgls show diffuse distribution, frequently. These tumors in rare instances occur in the brain 1 and neck 2 and, in a few cases, the bladder 3. Pheochromocytoma online surgery course lecturio online. Aug 10, 2018 a pheochromocytoma is a rare, catecholaminesecreting tumor derived from chromaffin cells. Novel approaches for locating occult pheochromocytoma. Recognizing the basics order the 4th edition of the bestselling textbook learning radiology. Recommended books for radiology residents ben white.
Pheochromocytoma is rarely the initial manifestation of men 2, more commonly presenting at an older age than mtc i. Join the radpac and radiology advocacy network to learn more about. The manifestations are diverse and the tumor can mimic a variety of conditions, often resulting in either erroneous diagnoses or a delayed diagnosis. Pheochromocytoma occurs in approximately 50% of men 2 patients, although less commonly in those with lowrisk mutations. Readers must therefore always check the product information and clinical procedures with the most up to date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. Medical intelligence from the new england journal of medicine localization of pheochromocytoma by computed tomography. William herrings clear, conversational writing style employs a touch of humor to explain what you. Pheochromocytomas and paragangliomas pheopgl are chromaffin cell tumors that embryonically derive from the neural crest and functionally are capable of production, storage, metabolism, and secretion of catecholamines.
The diagnosis was confirmed with the histology of the tumor. Most recommended book by residents who have cleared neet recently. About the author professor karel pacak put together the pheochromocytoma research program at the nih, one of the most prestigious and largest programs worldwide. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Patients with pheochromocytoma may be eligible for this study. Approximately 100 cases of pheochromocytoma were reported in children by 1963 57, the first case by marchetti in 1904 8. I123 mibg uptake in an adrenal nodule is strong supporting evidence for a pheochromocytoma.
Synaptophysin, chromogranin and s100 are positive in pheochromocytoma. Diagnosis, localization, and treatment and millions of other books are available for amazon kindle. The adrenal glands are two small glands that sit on top of the kidneys and produce hormones called catecholamines. And you wont waste all your time learning radiology instead of your primary specialty. This selfassessment module on imaging of pheochromocytoma and incidental adrenal lesions has an educational component and a selfassessment component. Current diagnostic imaging of pheochromocytomas and. Pheochromocytoma allison becker medical student jayanth h. The facts you need to know pheochromocytoma is a part of the pheochromocytoma and paraganglioma group of syndromes. On t1, mass has intensity similar to or slightly less than solid organs. Pheochromocytoma presenting as musculoskeletal pain from bone metastases article pdf available in skeletal radiology 167. William herring, md, a skilled radiology teacher, masterfully covers everything you need to know to effectively interpret medical images. Mar 26, 2020 pheochromocytoma, tumour, most often nonmalignant, that causes abnormally high blood pressure hypertension because of hypersecretion of substances known as catecholamines epinephrine, norepinephrine, and dopamine. Pdf pheochromocytoma presenting as musculoskeletal pain.
Listing a study does not mean it has been evaluated by the u. A pheochromocytoma is a rare, catecholaminesecreting tumor derived from chromaffin cells. A quarter century later, the german pathologist ludwig pick noted a color change after adding chromium salts. Jul 16, 2019 elevated levels of catecholamines and metanephrines are highly suggestive of pheochromocytoma with a sensitivity that is close to 100%. While pheos arise from the adrenal medulla, pgls can develop from both sympathetic and parasympathetic paraganglia. Ultrasound showed a lobulated mixed echogenicity mass in the leinorenal region displacing the left kidney inferiorly. The first diagnosis of pheochromocytoma was made in 1886 by frankel, 15 who found bilateral tumors of the adrenal gland at autopsy in an 18yearold girl who had died. Ae1ae3, ck7 and ttf1 are consistent with metastatic lung adenocarcinoma. Following clinical and laboratory workup and general radiographic procedures urography with adrenal laminagraphy, arteriography with careful monitoring is indicated. Everybody wants to learn radiology heres how you actually. Pheochromocytoma nord national organization for rare. Pheochromocytomas are most common in the fourth through sixth decades of life. Cardiac radiology genitourinary radiology learning radiology. Elevated levels of catecholamines and metanephrines are highly suggestive of pheochromocytoma with a sensitivity that is close to 100%.
In 1912, a pathologist named pick coined the term pheochromocytoma after the greek words phaios, meaning dark or dusky, and chroma, meaning colorto describe the chromaffin reaction seen in adrenomedullary tumors. Pheochromocytoma knowledge for medical students and. Pheochromocytoma javad ghahremani medical student at shahid beheshti university 2. Radiology books for entrance exams like neetaiims pgi. This book is essential reading for clinicians and scientists in the fields of endocrinology as well as oncology, surgery and nuclear medicine.
Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. The imaging features, in this clinical scenario, are those of a welldefined large pheochromocytoma. Most often produces epinephrine and norepinephrine mostly which is unregulated by any feedback mechanism. The clinical presentation and image are diagnostic of a pheochromocytoma.