Ma blake receives royalties from the book adrenal imaging springer verlag, 2009. However, more recent studies have shown a growing role of mibg scintigraphy and ct in the evaluation of pheochromocytomas. Pheochromocytoma and paraganglioma imaging findings. Varying imaging protocols show a highly necrotic mass displacing the right kidney. 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.
Mri is preferred for detection of pheochromocytoma in children and in pregnant or lactating women. It requires no contrast dye injection and no radiation exposure. During ct scanning, a computer and xrays are used to create a film showing crosssectional images of. Incidental right adrenal mass which is incompletely characterized by ultrasound.
Head, neck, chest, and abdominal mri may be helpful in the diagnosis of pheochromocytoma. Some studies showed that sensitivity of ct for extraadrenal, residual, recurrent, or metastatic tumors can be as low as 57% and inferior to mri 83, 86, 98102. We have a unique blend of academic and clinical medicine. Diagnostic tests pheochromocytoma and paraganglioma. This case reports on the worldwide first 124 imibgpet magnetic resonance imaging mri performed with an integrated petmr biograph mmr. T1weighted slightly hypointense to the remainder of the adrenal gland, if there is necrosis andor hemorrhage then the signal will be more heterogeneous. Paraganglioma and pheochromocytoma are rare tumors. The higher sensitivity in pheochromocytoma patients, compared with paraganglioma patients, was also seen in the subgroup with con.
Pheochromocytomas are relatively rare tumors of the adrenal medulla. If a tumor is found, the patient will be offered surgery. Pheochromocytoma nord national organization for rare. Pheochromocytoma and paraganglioma treatment is usually surgery, however, preoperative medical preparation is critical. Pheochromocytomas and paragangliomas are catecholamine producing tumors derived from the sympathetic or parasympathetic nervous system. 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. Paragangliomas are tumours that arise within the sympathetic nervous system originating from the neural crest. Magnetic resonance imaging is an important tool for the detection and characterization of adrenal tumors.
The various imaging appearances on ultrasound, ct, mri, and functional imaging can be complementary and have features that are useful for differentiating pheochromocytoma from other lesions of the adrenal. Pheochromocytoma imaging with an unremarkable biochemical. 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. Building 10, room 140 10 center drive bethesda, md 20892.
Ct scan of the abdomen revealed a large heterogeneous left adrenal mass. The middle part of the adrenal glands makes epinephrine and norepinephrine. Find local pheochromocytoma resources for the top u. Malignant pheochromocytoma imaging with 124imibg petmr. A previous whole body planar i123 mibg scan with attenuationcorrected. If the lesion is indeterminate on the dedicated adrenal protocol ct, one may. Our protocol for imaging of the abdomen in patients with suspected pheochromocytoma includes the following sequences. Imaging of pheochromocytoma and paraganglioma springerlink. Ct is preferred to mri for detection of lung metastatic lesions.
Mri is the most sensitive modality for identification of pheochromocytomas and is particularly useful in cases of extraadrenal location. Mri has a reported sensitivity of up to 100% in detecting adrenal pheochromocytomas, does not require contrast, and does not expose the patient to ionizing radiation. The diagnosis of pheochromocytomas provides a potentially correctable cause of hypertension, and their removal can. Siemens healthcare, erlangen, germany in a malignant pheochromocytoma. Pheochromocytoma is a rare tumor that usually starts in the cells of one of your adrenal glands. Mri of adrenal and extraadrenal pheochromocytoma request pdf. Abdominal computed tomography ct scan demonstrating left suprarenal mass of softtissue attenuation representing a paraganglioma. Pheochromocytoma javad ghahremani medical student at shahid beheshti university 2. Ct scan was used to determine thoracic and abdominopelvic. It is the imaging modality of choice in pregnancy and for extraadrenal and cardiac pheochromocytoma. Small adrenal pheochromocytoma on the right side arrows. Patients with pheochromocytoma may be eligible for this study. Although they are usually benign, pheochromocytomas often cause the adrenal gland to make too many hormones. Pheochromocytoma of the adrenal gland scaled score pass.
Although pheochromocytoma has classically been associated with 3 syndromesvon hippellindau vhl syndrome, multiple. Standard imaging tests noninvestigational imaging tests include computed tomography ct, magnetic resonance imaging mri, sonography, and 123imibg scintigraphy and fdg positron emission tomography petct. Eleven consecutive patients with an extraadrenal pheochromocytoma in abdominal cavity who underwent preoperative 0. Axial, t2weighted magnetic resonance imaging mri scan showing large left suprarenal mass of high signal intensity on a t2weighted image. Images of pheochromocytoma in adrenal glands mcdermott.
The tumor is malignant in 10% of cases but may be cured completely by surgical removal. The addition of mri to mibgpet is promising as it improves tumor delineation because of the high soft tissue contrast in mri that. Sometimes pheochromocytoma is part of another condition called multiple. In addition, a delay in contrast washout of magnetic resonance image mri of the pituitary is the investigation of choice, but it may not show an abnormality in up to 40% of cases because of small tumors below the sensitivity of detection. Like ct scan, the specificity has been reported to be as low as 50%. Magnetic resonance imaging mri the most important sequence of the adrenal mri protocol is chemical shift imaging performed with inphase and outofphase sequences.
Internal hemorrhage can sometimes be seen within the tumor fig. Mri of pheochromocytoma typically demonstrates a t2 hyperintense mass, although the. Read more about pheochromocytoma chestabdomenpelivs screening. The tumors arise from the chromaffin cells of the adrenal medulla and are associated with increased catecholamine production.
Get detailed information in this clinician summary. We are one of the largest centers in the world to properly diagnose and treat patients with pheochromocytoma and paraganglioma. The most frequent symptom of the pheochromocytoma is hypertension, and 90100% of cases exhibit it. The cancer typically develops in the central part of the adrenal glands called the adrenal medulla, which is responsible for producing important hormones such epinephrine, adrenalin. Distinct from composite pheochromocytoma, which are tumors composed of pheochromocytoma plus ganglioneuroma, ganglioneuroblastoma, neuroblastoma or peripheral nerve sheath tumors. Current diagnostic imaging of pheochromocytomas and implications. Epidemiology, clinical presentation, diagnosis, and histology. Superior to the right kidney in the area of the adrenal gland there is a wellcircumscribed, heterogenous mass which measures 4. Highdensity t 2weighted images of the mri can also distinguish pheochromocytoma from other adenomas and some malignant. These symptoms may be in part due to the cosecretion of other hormones associated with norepinephrine, such as corticotropinreleasing hormone, adrenocorticotropin, and interleukin six. The relative usefulness of mri and i1metaiodobenzylguanidine mibg scintigraphy is controversial. Pheochromocytoma is also capable of causing, at presentation, pseudoobstruction of bowels, diabetic ketoacidosis, or multisystem crisis involving lactic acidosis. Adrenal mass 14 cm and lipid containing lesion on initial imaging see above consider evaluating endocrine labs above especially to rule out pheochromocytoma repeat unenhanced ct abdomen in 12 months to confirm no change.
Ppt pheochromocytoma powerpoint presentation free to. Ten percent of pheochromocytoma cases are diagnosed incidentally during computed tomography ct or magnetic resonance imaging mri screenings for other reasons. Usefulness of imibg scintigraphy in the evaluation of. Pheochromocytoma is an important, often clinically occult neoplasm with devastating consequences if overlooked. Precontrast axial ct of abdomen shows a mass in the right adrenal gland white arrow the left adrenal gland is normal yellow arrow. Candidates will be screened with a medical history and physical examination, electrocardiogram, and blood and urine tests. 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. Mr imaging reveals a large well defined mass in the right adrenal gland. A small group of 57% of the functional ones 30% may exist as pheochromocytoma. Pheochromocytomas are an uncommon tumor of the adrenal gland, with characteristic clinical, and to a lesser degree, imaging features. Pheochromocytoma is a rare neuroendocrine tumor, occurring in less than 0. Imaging techniques such as computed tomography ct scan and magnetic resonance imaging mri are often performed to determine the specific location and size of a pheochromocytoma.
Each layer of these glands makes different hormones. 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. Pheochromocytoma, in which symptoms are not very specific and hypertension is present in only half of patients, remains rare. Palliative care for metastatic disease may include chemotherapy, radiation therapy, targeted therapy, and other modalities. Epidemiology catecholaminesecreting tumors are rare neoplasms, probably occurring in less than 0. 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. Pheochromocytoma is a rare tumor that usually develops from one of the bodys two adrenal glands, located above each kidney in the back of the upper abdomen. A pheochromocytoma see the image below is a rare, catecholaminesecreting tumor that may precipitate lifethreatening hypertension. Radiologists must be aware of the various forms that pheochromocytomas can assume at imaging. Eligibility criteria pheochromocytoma and paraganglioma. This can lead to high blood pressure and cause symptoms such as.
Diagnosis of a pheochromocytoma columbia university. The imaging appearance of pheochromocytoma is variable on all imaging. These scans may be done before andor after surgical removal of pheochromocytoma. Pheochromocytoma surgical treatment ncbi bookshelf. Imaging tests are used to assess the location of a pheochromocytoma or paraganglioma. This patients adrenal mri scan was highly suggestive of a pheochromocytoma with a high. These tumours can be found anywhere from the neck to the pelvis in locations of sympathetic ganglions. Our nih research protocol focuses on the genetics, diagnosis, localization, and surgical and interventional radiological treatment of pheochromocytoma and. Mri has a sensitivity of 95% and a specificity of 100%.
The mri of extraadrenal pheochromocytoma in the abdominal. Among those with known prior disease, the sensitivity of 123imibg planar imaging was lower for those with paraganglioma 67% vs. Absolute and relative percentage washout values were 69. Xrays, ct scans, mri and other scans for pheochromocytoma. Incidental pheochromocytoma presenting with sublaboratory. Mri is also superior to ct scanning for detecting extraadrenal pheochromocytomas. They suggested that a protocol incorporating the measurement of 24hour urine metanephrines and catecholamines in patients with borderline elevations of fractionated plasma metanephrines was the least costly approach and, at the same time, had a reasonable level of sensitivity for diagnosing a. See pheochromocytoma in genetic disorders and treatment of pheochromocytoma in adults and paragangliomas. Loss of signal intensity of the adrenal mass on outofphase images, compared to the inphase pulse sequence, is diagnostic of the presence of intracellular lipid.
Imaging of pheochromocytoma and paraganglioma ncbi. An adrenalprotocol, contrast enhancement ct scan is best. Thus a ct scan of the adrenal with and without contrast should always be the first scan ordered, and in more than 90% of cases the only scan a patient will need. Following intravenous contrast lower photo, the right adrenal mass enhances brightly red arrow. Choledocholithiasis and cholelithiasis with intrahepatic and extra hepatic biliary ductal dilatation but without sonographic evidence of acute cholecystitis. Study participants will undergo blood, urine, and imaging tests, described below, to detect pheochromocytoma. Employing these guidelines helps providers enhance quality of care and contribute to the most efficacious use of radiology. For skull base and neck paraganglioma, the sensitivity of mri is between 90 and 95%. What is the role of mri for the detection of pheochromocytoma.
The purpose of this study was to summarize the mr appearances of extraadrenal pheochromocytoma in the abdominal cavity and evaluate the capabilities of mri in diagnosis of the tumor. Mri scans are almost never necessary and should never be the first scan that a doctor orders to look at adrenal masses. Evaluate endocrine labs above especially to rule out. These hormones help keep your heart rate and blood pressure.
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