Diagnosis and Management of Tumors of the Adrenal Medulla

I. Ilias; K. Pacak

doi:10.1055/s-2005-921091

Hormone and Metabolic Research, Inhaltsverzeichnis

Horm Metab Res 2005; 37(12): 717-721
DOI: 10.1055/s-2005-921091

Review

Diagnosis and Management of Tumors of the Adrenal Medulla

I. Ilias¹ , K. Pacak²

¹Department of Pharmacology, Medical School, University of Patras, Rion-Patras, Greece
²Reproductive Biology and Medicine Branch, National Institutes of Health, Bethesda, Maryland, USA

Abstract

The adrenal medulla consists of chromaffin cells, the site of catecholamine biosynthesis. Pheochromocytomas are chromaffin-cell tumors; 80 - 85 % arise from the adrenal medulla and 15 - 20 % arise from extra-adrenal chromaffin tissues (paragangliomas). Neuroblastomas are primitive tumors that derive from the same blastic precursor as in pheochromocytomas, and are distributed along the sympathetic nervous system. Pheochromocytomas account for 6.5 % of incidentally discovered adrenal tumors; they are found in 50 % of patients with multiple endocrine neoplasia 2A (MEN 2A) and 5 - 25 % of patients with von Hippel-Lindau (VHL) syndrome. Neuroblastomas are the most common solid extra-cranial tumors in children, and account for 7 - 10 % of all tumors. The diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines (the measurement of urinary fractionated metanephrines is the second choice). Measurements of homovanillic acid (HVA), norepinephrine and vanilmandelic acid (VMA) in urine are a necessity in patients with suspected neuroblastoma. Anatomical (radiological) imaging with computed tomography (CT) or magnetic resonance imaging (MRI) is necessary for both pheochromocytomas and neuroblastomas. Functional (nuclear medicine) methods are useful for both tumors. Scintigraphy with [¹²³I]-metaiodobenzylguanidine is the specific functional imaging test of first choice; if this is not available, scintigraphy with [¹³¹I]-MIBG is the second choice. Other newer specific modalities that have been used for evaluating pheochromocytomas include positron emission tomography (PET) with [¹⁸F]-F-fluorodopamine (F-DA) and [¹⁸F]-F-dihydroxyphenylalanine (DOPA). These should be used when MIBG scintigraphy is negative. Primary treatment for both types of tumor is surgical; chemotherapy is used for inoperable disease. After successful surgery, survival of patients with benign, sporadic pheochromocytomas is believed to be equal to that of the general population. Depending on the extent of disease and age, patients with neuroblastomas have cure rates of 15 - 90 %.

Key words

Pheochromocytoma - paraganglioma - neuroblastoma - metanephrines - radionuclide imaging - surgery - laparoscopy

Volltext

Referenzen

References

1 Bornstein S R, Oelkers W. Adrenal function and metabolism. Horm Metab Res. 2004; 36 341-345
2 Lack E E. Tumors of the adrenal gland and extra-adrenal paraganglia. Washington DC; Armed Forces Institute of Pathology 1997
3 Eisenhofer G, Lenders J W, Pacak K. Biochemical diagnosis of pheochromocytoma. Front Horm Res. 2004; 31 76-106
4 Ilias I, Shulkin B, Pacak K. New functional imaging modalities for chromaffin tumors, neuroblastomas and ganglioneuromas. Trends Endocrinol Metab. 2005; 16 66-72
5 van den Berg R. Imaging and management of head and neck paragangliomas. Eur Radiol. 2005; 15 1310-1318
6 Neumann H P, Bausch B, McWhinney S R, Bender B U, Gimm O, Franke G, Schipper J, Klisch J, Altehoefer C, Zerres K, Januszewicz A, Eng C, Smith W M, Munk R, Manz T, Glaesker S, Apel T W, Treier M, Reineke M, Walz M K, Hoang-Vu C, Brauckhoff M, Klein-Franke A, Klose P, Schmidt H, Maier-Woelfle M, Peczkowska M, Szmigielski C, Eng C. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002; 346 1459-1466
7 Eisenhofer G, Goldstein D S, Walther M M, Friberg P, Lenders J W, Keiser H R, Pacak K. Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. J Clin Endocrinol Metab. 2003; 88 2656-2666
8 Pacak K, Ilias I, Adams K T, Eisenhofer G. Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour. J Intern Med. 2005; 257 60-68
9 Argiris A, Mellott A, Spies S. PET scan assessment of chemotherapy response in metastatic paraganglioma. Am J Clin Oncol. 2003; 26 563-566
10 Neumann H P, Cybulla M, Shibata H, Oya M, Naruse M, Higashihara E, Terachi T, Ling H, Takami H, Shuin T, Murai M. New genetic causes of pheochromocytoma: current concepts and the clinical relevance. Keio J Med. 2005; 54 15-21
11 Boedeker C C, Ridder G J, Schipper J. Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer. 2005; 4 55-59
12 Pacak K, Linehan W M, Eisenhofer G, Walther M M, Goldstein D S. Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Ann Intern Med. 2001; 134 315-329
13 Beltsevich D G, Kuznetsov N S, Kazaryan A M, Lysenko M A. Pheochromocytoma surgery: epidemiologic peculiarities in children. World J Surg. 2004; 28 592-596
14 Elder E E, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10 % tumor. J Surg Oncol. 2005; 89 193-201
15 Brink I, Hoegerle S, Klisch J, Bley T A. Imaging of pheochromocytoma and paraganglioma. Fam Cancer. 2005; 4 61-68
16 Elsayes K M, Narra V R, Leyendecker J R, Francis I R, Lewis J S Jr, Brown J J. MRI of adrenal and extraadrenal pheochromocytoma. AJR - Am J Roentgenol. 2005; 184 860-867
17 Beck O, Fassbender W J, Beyer P, Kriener S, Neumann H P, Klingebiel T, Lehrnbecher T. Pheochromocytoma in childhood: implication for further diagnostic procedures. Acta Paediatr. 2004; 93 1630-1634
18 Opocher G, Conton P, Schiavi F, Macino B, Mantero F. Pheochromocytoma in von Hippel-Lindau disease and neurofibromatosis type 1. Fam Cancer. 2005; 4 13-16
19 Kaltsas G A, Papadogias D, Grossman A B. The clinical presentation (symptoms and signs) of sporadic and familial chromaffin cell tumours (phaeochromocytomas and paragangliomas). Front Horm Res. 2004; 31 61-75
20 Lee K L, Ma J F, Shortliffe L D. Neuroblastoma: management, recurrence, and follow-up. Urol Clin North Am. 2003; 30 881-890
21 Morgenstern B Z, Krivoshik A P, Rodriguez V, Anderson P M. Wilms’ tumor and neuroblastoma. Acta Paediatr Suppl. 2004; 93 78-84
22 Eisenhofer G, Lenders J W, Goldstein D S, Mannelli M, Csako G, Walther M M, Brouwers F M, Pacak K. Pheochromocytoma Catecholamine Phenotypes and Prediction of Tumor Size and Location by Use of Plasma Free Metanephrines. Clin Chem. 2005; 51 735-744
23 Eisenhofer G, Goldstein D S, Sullivan P, Csako G, Brouwers F M, Lai E W, Adams K T, Pacak K. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 2005; 90 2068-2075
24 Ilias I, Pacak K. Current approaches and recommended algorithm for the diagnostic localization of pheochromocytoma. J Clin Endocrinol Metab. 2004; 89 479-491
25 Ilias I, Alesci S, Pacak K. Current views on imaging of adrenal tumors. Horm Metab Res. 2004; 36 430-435
26 Kann P H. Endoscopic ultrasound imaging of the adrenals. Endoscopy. 2005; 37 244-253
27 Kann P H, Wirkus B, Behr T, Klose K J, Meyer S. Endosonographic imaging of benign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2004; 89 1694-1697
28 Taieb D, Sebag F, Hubbard J G, Mundler O, Henry J F, Conte-Devolx B. Does iodine-131 meta-iodobenzylguanidine (MIBG) scintigraphy have an impact on the management of sporadic and familial phaeochromocytoma?. Clin Endocrinol (Oxf). 2004; 61 102-108
29 Sturgeon C, Kebebew E. Laparoscopic adrenalectomy for malignancy. Surg Clin North Am. 2004; 84 755-774
30 Diner E K, Franks M E, Behari A, Linehan W M, Walther M M. Partial Adrenalectomy: The National Cancer Institute Experience. Urology, in press (doi:10.1016/j.urology.2005.01.009) . ;
31 Yip L, Lee J E, Shapiro S E, Waguespack S G, Sherman S I, Hoff A O, Gagel R F, Arens J F, Evans D B. Surgical management of hereditary pheochromocytoma. J Am Coll Surg. 2004; 198 525-534
32 Neumann H PH, Reincke M, Bender B U, Elsner R, Janetschek G. Preserved Adrenocortical Function After Laparoscopic Bilateral Adrenal Sparing Surgery for Hereditary Pheochromocytoma. J Clin Endocrinol Metab. 1999; 84 2608-2610
33 Nambirajan T, Leeb K, Neumann H P, Graubner U B, Janetschek G. Laparoscopic adrenal surgery for recurrent tumours in patients with hereditary phaeochromocytoma. Eur Urol. 2005; 47 622-626
34 Brauckhoff M, Gimm O, Brauckhoff K, Dralle H. Repeat adrenocortical-sparing adrenalectomy for recurrent hereditary pheochromocytoma. Surg Today. 2004; 34 251-255
35 Sandoval C, Strom K, Stringel G. Laparoscopy in the management of pediatric intraabdominal tumors. JSLS - J Soc Laparoend Surg. 2004; 8 115-118
36 Hwang J, Shoaf G, Uchio E M, Watson J, Pacak K, Linehan W M, Walther M M. Laparoscopic management of extra-adrenal pheochromocytoma. J Urol. 2004; 171 72-76
37 Brouwers F M, Lenders J W, Eisenhofer G, Pacak K. Pheochromocytoma as an endocrine emergency. Rev Endocr Metab Disord. 2003; 4 121-128
38 John H, Ziegler W H, Hauri D, Jaeger P. Pheochromocytomas: can malignant potential be predicted?. Urology. 1999; 53 679-683
39 Amar L, Servais A, Gimenez-Roqueplo A P, Zinzindohoue F, Chatellier G, Plouin P F. Year of Diagnosis, Features at Presentation, and Risk of Recurrence in Patients with Pheochromocytoma or Secreting Paraganglioma. J Clin Endocrinol Metab. 2005; 90 2110-2116

Dr. Ioannis Ilias

Department of Pharmacology · Medical School · University of Patras

Rion-Patras GR-26504 · Greece

Fax: +30 2610 994 720

eMail: iliasi@upatras.gr