Bessell-Browne R, O'Malley ME. 16 (1): 22. Accessibility Overall 98% of tumors are in the abdomen, and 90% are confined to the adrenal glands 6. May identify extra-adrenal lesions, which account for 10% of pheochromocytomas. Urinary estimation of catecholamines and their metabolites is a better biochemical investigation than plasma estimation for the diagnosis of these lesions. Patients with extra-adrenal pheochromocytoma commonly present with the classic triad of headache, palpitations and sweating. When a tumor composed of the same cells as a pheochromocytoma develops outside the adrenal gland, it is referred to as a paraganglioma. Women and men are affected with similar frequency. Pheochromocytoma is a rare tumor, accounting for <0.1% of the hypertensive population. CT and MRI of the abdomen and pelvis, a teaching file. 150 (1): 27-32. In 5-10% of cases, a pheochromocytoma is a manifestation of an underlying condition including 1-4,6: It is a rare but classical cause of uncontrolled secondary hypertension, with a minority having superimposed paroxysmal hypertensive crises 11. Please enable it to take advantage of the complete set of features! Radiology. Pheochromocytomas are a type of paraganglioma. We retrospectively reviewed CT and MRI findings in 29 patients with 39 extra-adrenal paragangliomas. Pheochromocytomas are vascular and may appear variable with solid, cystic, calcified, and necrotic components. Advantages. Lippincott Williams & Wilkins. 18F-Dopa PET is thought to be highly sensitive according to initial results 3. Prevalence of extra-adrenal pheochromocytoma Pheochromocytomas Total No. Our review was undertaken to describe CT and MRI features of unusual extra-adrenal paragangliomas (pheochromocytomas). 1991 Jan;11(1):37-57. doi: 10.1148/radiographics.11.1.1671719. Robbins and Cotran pathologic basis of disease. Extra-adrenal tumors that may predominantly secrete epinephrine are rare in the organ of Zuckerkandl. Santhanam P et.al. 2018 Jan 4;18(1):1. doi: 10.1186/s12883-017-0998-8. Clinical Findings. Radiology. B, Coronal 10-minute contrast-enhanced CT image. N Engl J Med 312:721, 1985 (letter) 8. Rather, the distinction is made on demonstrating evidence of direct tumor invasion into adjacent organs/structures or the presence of metastases 4. 6. Radiology 156:789-792, 1985 9. CT imaging of the abdomen and 123I-labeled metaiodobenzylguanidine scintigraphy revealed a right pheochromocytoma and left adrenal incidentaloma. Gurinder Nandra, Oliver Duxbury, Pawan Patel, Jaymin H. Patel, Nirav Patel, Ioannis Vlahos. Langenbecks Arch Surg. There were 17 carotid body tumours, 1 mediastinal, 1 intra-cardiac, 15 retroperitoneal extra-adrenal paragangliomas, 2 bladder, 1 pelvic sidewall and 2 intra-spinal paragangliomas within the lumbo-sacral spine. 7A and 7B). Sometimes, pheochromocytomas arise from chromaffin cells that are located outside of the adrenal gland. MRI is the most sensitive modality for identification of pheochromocytomas and is particularly useful in cases of extra-adrenal location. Endocr Relat Cancer. Elsayes KM, Mukundan G, Narra VR et-al. At detection, 25 tumours were larger than 4 cm, of which 20 were heterogeneous lesions on CT and MRI with variable contrast enhancement. Extra- Adrenal (%) Dahl-Iversen40 18 3(17) Hume41 21 3(14) Crout and Sjoerdsma7 18 3(17) Flint42 17 4(24) de Graeff and Horak43 44 5(11) Sander et al44 29 4(14) Mahoney et al45 21 6 (29) ReMine et al46 138 14 (10) Deoreo et al47 46 7(15) Ohman et al48 20 6 (30) Desmonts et al49 97 11 CD Melicow23 100 17 … North Am. Northcutt BG, Raman SP, Long C et-al. Unfortunately, these agents are not very specific for pheochromocytomas and have a limited spatial resolution, usually requiring the tumor to be >1 cm in diameter. Springer Verlag. Extra-adrenal tumors are more likely to be malignant and metastasize 4. Epub 2005 Nov 30. van Gils AP, Falke TH, van Erkel AR, Arndt JW, Sandler MP, van der Mey AG, Hoogma RP. EAPs probably represent at least 15% of adult and 30% of childhood pheochromocytomas, as opposed to the traditional teaching that 10% of all pheochromocytomas are at extra-adrenal sites. Epub 2013 Jun 6 doi: 10.1089/end.2012.0745. 24-hour urine metanephrines and normetanephrine were negative. 14. We also propose catecholamine-secreting pheochromocytoma as a possible cause of BAT activation in our case. 8600 Rockville Pike (2007) ISBN:1405149507. Extra-adrenal pheochromocytomas (EAPs) are rarer still, accounting for 10% of all pheochromocytomas. Laparoscopic resection of adrenal and extra-adrenal pheochromocytoma. This also applies to those patients who are asymptomatic and have an incidental finding of an adrenal mass on CT scanning. 16. They can be found along the sympathetic chain as well as in the urinary bladder and organ of Zuckerkandl. Radiographics. Reiser MF. An inherited neoplasia syndrome was suspected and molecular genetic analyses were performed. reportedly occurring in 0.05–0.2% of hypertensive individuals. Epub 2017 Dec 28. Role of (18) F-FDOPA PET/CT Imaging in Endocrinology. Radiological Surveillance Screening in Asymptomatic Succinate Dehydrogenase Mutation Carriers. In this review, for the purpose of familiarity, we will refer to … Pheochromocytomas are functional catecholamine-secreting tumors of the paraganglionic chromaffin cells found in the adrenal medulla and the extra-adrenal paraganglia cells. Lack EE. These tumors exhibit variability in symptoms and geographic distribution; therefore, biochemical markers and imaging are required to confirm the diagnosis [ 1, 2 ]. 2014;77 (3): 229-38. Fruit St. Boston, (Departments MA 02114.) 2006 Jan;263(1):23-31. doi: 10.1007/s00405-004-0885-y. Pheochromocytoma (PHEO or PCC) is a rare tumor of the adrenal medulla composed of chromaffin cells, also known as pheochromocytes. 68 Ga-DOTATATE and 18 F-FDG PET/CT in Paraganglioma and Pheochromocytoma: utility, patterns and heterogeneity. 2004;24 Suppl 1 (suppl 1): S73-86. Humana Pr Inc. (2009) ISBN:193411586X. Because of their varied clinical presentations, imaging, and pathologic appearances, accurate diagnosis can be challenging; however, appropriate and timely diagnosis and treatment is essential in preventing severe complications such as myocardial infarctions, strokes, and … 2011;40 (2): 295-311, vii. Check for errors and try again. For each tumour, site, size, MRI characteristics, CT appearances and enhancement after gadolinium and iohexol were recorded. General I Urol Hospital, At UMORS of the adrenal system medulla and paraganglion (pheochromocytomas) the extraadrenal are tions. The 14 smaller paragangliomas were smooth in contour and demonstrated avid, homogeneous contrast enhancement. Precise localization is important for safe excision, and CT, 131 iodine scintigraphy and magnetic resonance imaging are useful modalities. Unfortunately, the kidney also has somatostatin receptors, as do areas of inflammation, mammary glands, liver, spleen, bowel, gallbladder, thyroid gland and salivary glands. Imaging is obtained 4 hours (+/- 24/48 hours) after an intravenous infusion. The majority of cases are sporadic. HOOD 1993-03-01 00:00:00 Extra-Adrenal 1992; i47:i-iO. Locations of extra-adrenal pheochromocytomas include the organ of Zuckerkandl which is close to the origin of the inferior mesenteric artery, bladder wall, heart, mediastinum, and carotid and glomus jugulare bodies. 17. CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, and pelvis, … Falke TH, van Gils AP, van Seters AP, Sandler MP. Pheochromocytomas are relatively rare catecholamine-secreting neuroendocrine tumors that arise from the chromaffin cells of the adrenal medulla or extraadrenal paraganglia. In response to the comments, a SPECT/CT and MIBG scan were obtained. Magnetic resonance imaging of functioning paragangliomas. The peak incidence is between the third and the fifth decades of life. 1A and 1B), 10% are malignant, 10% occur in children, and 10% are extraadrenal.The extraadrenal lesions are also referred to as paragangliomas []. 2013;200 (2): 370-8. Careers. 13. Prevention and treatment information (HHS). Clipboard, Search History, and several other advanced features are temporarily unavailable. Extra-Adrenal Pheochromocytoma: An Unusual Location By Gary E. Hartman, Raymond Hintz, William Northway, Jr, and Frederick G. Mihm Stanford, California A 14-year-old boy with a retrohepatic para-aortic pheochromocytoma demonstrates the potential difficulties of localization in an unusual extra-adrenal site of pheochro- mocytoma. had undergone surgery for ectopic pheochromocytoma. 18. Ros PR, Mortele KJ. 2005;4(1):61-8. doi: 10.1007/s10689-004-2155-y. Solitary lesions inexplicably favor the right side. Pheochromocytomas may occur in persons of any age. 2007;188 (4): 970-4. Clinical experience over 48 years with pheochromocytoma. Baid SK, Lai EW, Wesley RA, Ling A, Timmers HJ, Adams KT, Kozupa A, Pacak K. Brief communication: radiographic contrast infusion and catecholamine release in patients with pheochromocytoma. Our review was undertaken to describe CT and MRI features of unusual extra-adrenal paragangliomas (pheochromocytomas). However, pheochromocytoma originating from the pancreas is rare. DISCUSSION: a rare, catecholamine-secreting tumor that may precipitate life-threatening hypertension. FDG PET-CT scan showed huge bilateral intense FDG uptake in the adrenal glands with no extra-abdominal … -, AJR Am J Roentgenol. MRI demonstrated the greatest variability in the appearances of larger tumours, provided additional information compared to CT for surgical planning and is a useful screening tool for patients at high risk of extra-adrenal paragangliomas. When a tumor composed of the same cells as a pheochromocytoma develops outside the adrenal gland, it is referred to as a paraganglioma. (2006) ISBN:0781772370. Epub 2019 Apr 1. Shapiro B, Copp JE, Sisson JC, et al: … 3, Fig. Lynn M, Shapiro B, Sisson J, et al: Pheochromocytoma and the normal adrenal medulla: Improved visualization with 1-123 MIBG Scintigraphy. Combined functional/anatomical imaging ( 68 Ga-DOTA-TOC PET/CT) enables exact tumour extension … 68 Ga-DOTATOC PET/CT in the localization of metastatic extra-adrenal paraganglioma and pheochromocytoma … Weakness. 2019 Jun;404(4):385-401. doi: 10.1007/s00423-019-01768-z. CT is the first imaging modality to be used, with an overall sensitivity of 89%. Pheochromocytomas and paragangliomas may be ... Higher-than-normal amounts of certain catecholamines may be a sign of pheochromocytoma. when Vena caval catecholamine … important for safe excision, and CT, 131iodine scintigraphy and magnetic resonance imaging are useful modalities.1, 2 Preoperative and intraoperative management is the same as for adrenal pheochromocytoma but one should explore the retroperitoneum to detect multifocal extra-adrenal pheochro- mocytoma. DIAGNOSIS: Pheochromocytoma of left adrenal gland. 1995 Aug;165(2):260 Locations of extra-adrenal pheochromocytomas include the organ of Zuckerkandl which is close to the origin of the inferior mesenteric artery, bladder wall, heart, mediastinum, and carotid and glomus jugulare bodies. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. 2002;222 (2): 507-12. Extra-adrenal pheochromocytomas, or gangliomas, develop in the paraganglion chromaffin cells of the sympathetic nervous system. They may be malignant in up to 40% of the cases, though conflicting data add to the uncertainty of this point. Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas. Pheochromocytoma, Diagnosis, Localization, and Treatment. The focus of our report was to show the striking imaging features of the tumor. These tumors also arise in the paraganglia near the aorta and the ganglia of the sympathetic chains. CT is equally sensitive to MRI, and may be used as first line imaging. (see adrenal anatomy for more info). Extra-adrenal pheochromocytomas (EAPs) may arise in any portion of the paraganglion system, though they most commonly occur below the diaphragm, frequently in the organ of Zuckerkandl. All 39 paragangliomas were shown on MRI. Imaging tests, especially CT or MRI, help localize tumors. Hypertension 1997; 29:1133. Preoperative medical management is essential in reducing the risk of intraoperative hypertensive crises and typically consists of the non-competitive alpha-adrenergic blockade (e.g. Over 70% of tumors express somatostatin receptors. After controlling blood pressure, surgery was performed, and the tumour was successfully removed. 40 (4): 1041-1060. W B Saunders Co. (2005) ISBN:0721601871. CT imaging of the abdomen and (123)I-labeled metaiodobenzylguanidine scintigraphy revealed a right pheochromocytoma and left adrenal incidentaloma. As a general rule, tumors in the adrenal region tend to be large at presentation, usually >3 cm, with an average size of approximately 5 cm. Washington, DC: Armed Forces Institute of Pathology,1997 : … Extra-adrenal pheochromocytomas may arise in any portion of the paraganglion system, although they most commonly occur below the diaphragm. Pheochromocytomas are uncommon tumors arising from pheochromocytes, the predominant cells of adrenal medulla. Headaches, palpitations and diaphoresis with hypertension, frequently episodic. HOOD 1993-03-01 00:00:00 Extra-Adrenal 1992; i47:i-iO. 11. This site needs JavaScript to work properly. The overall sensitivity is said to be 98% 6. He was clinically diagnosed with an extra-adrenal abdominal ectopic pheochromocytoma. Zuber SM, Kantorovich V, Pacak K. Hypertension in pheochromocytoma: characteristics and treatment. Computed tomography (CT) imaging of his abdomen revealed a heterogeneously enhancing soft tissue mass within the retroperitoneum between the infrarenal abdominal aorta and the inferior vena cava, which measured approximately 4.0 × 4.4 × 4.3 cm. Our review of extra-adrenal paragangliomas highlights their unusual sites and appearances. Havekes B, van der Klaauw AA, Weiss MM, Jansen JC, van der Mey AG, Vriends AH, Bonsing BA, Romijn JA, Corssmit EP. FOIA A right 6 cm pheochromocytoma seen on a CT scan (arrow) and after removal. (2007) ISBN:354029354X. AJR Am J Roentgenol. The term “pheochromocytoma” has been used to refer to the paraganglioma of the adrenal medulla. The gross and microscopic morphology of paragangliomas is practically the same irrespective of location. eCollection 2017 Jul 1. many neuroendocrine tumors demonstrate uptake with MIBG, extramedullary hematopoiesis in adrenal gland, Management of Incidental Adrenal Masses: ACR White Paper, multiple endocrine neoplasia type II (MEN II): both, usually large, heterogeneous masses with areas of necrosis and cystic change, may wash out similar to an adrenal adenoma, but they tend to have a greater enhancement in an arterial or portal venous contrast phase, tend to enhance more on the portal venous phase than the arterial phase, 110 HU of enhancement on the arterial phase is compatible with pheochromocytoma; hypervascular metastases could be considered in an appropriate setting, up to 7% demonstrate areas of calcification, slightly hypointense to the remainder of the adrenal, if necrotic and/or hemorrhagic then the signal will be more heterogeneous, ~ 1/3 do not have marked T2 hyperintensity, areas of necrosis/hemorrhage/calcification will alter the signal, enhancement is prolonged, persisting for as long as 50 minutes, clinical suspicion is almost always present before imaging: secondary hypertension and positive urinary catecholamines, usually large and heterogeneous adrenal masses, with cystic and necrotic components, vivid enhancement of arterial/portal venous phase: >110-120 HU.
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