Diagnostik und operative Therapie von Phäochromozytomen und Paragangliomen (original) (raw)

Zentralbl Chir 2010; 135(3): 226-232
DOI: 10.1055/s-0030-1247315

Übersicht

© Georg Thieme Verlag Stuttgart ˙ New York

Diagnosis of and Surgical Therapy for Pheochromocytoma and Paraganglioma

• 1Johannes Gutenberg-Universität Mainz, Endokrine Chirurgie, Klinik für Allgemein- und Abdominalchirurgie, Mainz, Deutschland

Further Information

Publication History

Publication Date:
14 June 2010 (online)

Zusammenfassung

Phäochromozytome und Paragangliome sind seltene chromaffine Tumoren, welche aufgrund ­einer begleitenden Katecholamin-Produktion eine besondere Herausforderung für den Chirurgen darstellen. Neue Erkenntnisse zur Genese insbesondere hereditärer Tumoren haben die alte Regel des 10 %-Tumors relativiert und die Anforderungen an die notwendige Diagnostik sowie die operative Strategie wesentlich verändert. Die frühzeitige Erkennung eines malignen Prozesses oder synchroner hereditärer Tumoren sind Ziel verschiedener bildgebender Verfahren wie CT / MRT, 123J-MIBG-(SPECT) oder 18F-DOPA-PET. Ohne Vorliegen von Metastasen ist eine sichere Einschätzung der Dignität präoperativ und sogar histopathologisch selten eindeutig möglich. Wesentlic­he Voraussetzung für eine risikoarme Operation ist die adäquate präoperative α-Blockade, welche üblicherweise auf 3–5 mg / kg KG / Tag langsam gesteigert werden sollte. Einen Sonderfall stellen ausschließlich dopaminsezernierende Para­gang­lio­me dar. Therapie der Wahl ist für singuläre ­benigne Phäochromozytome die minimalinvasive Resektion, welche retroperitoneoskopisch oder transabdominell erfolgen kann. Auch abdomi­nelle Paragangliome können überwiegend minimalinvasiv entfernt werden; sie erfordern in der Regel jedoch eine besondere Expertise. Für sehr große oder malignitätsverdächtige Tumoren sowie ggf. bei multifokalen Prozessen haben sich transabdominelle Resektionen bewährt. Auch bei fortgeschrittenen malignen Prozessen sind Debulking-Resektionen unter palliativen Aspekten sinnvoll und tragen zur Verbesserung der Lebensqualität und der Prognose der Patienten bei.

Abstract

Pheochromocytomas and paragangliomas are rare chromaffin tumours that represent an exceptional challenge for the surgeon because of the concomitant secretion of catecholamines. Recent findings on the genetic background of hereditary tumours have challenged the rule of the 10 % ­tumour and significantly changed the requirements for preoperative work-up and surgical strat­egy. Early detection of malignant growth or multiple hereditary tumours is the goal of imag­ing techniques such as CT / MRI, 123I-MIBG-(SPECT) or 18F-DOPA-PET. However, in the absence of metastasis, reliable differentiation between ­benign and malignant growth is preoperatively and even histopathologically rarely possible. An essential precondition for successful surgical ther­apy with low operative risks is an ade­quate pretreatment with α-adrenergic antag­o­nists which should slowly be increased to 3–5 mg / kg BW / day prior to resection. Dopamine-secreting paragangliomas represent the sole exception. Minimally invasive techniques using a transabdominal or retroperitoneal approach have become the gold standard for the resection of unifocal benign pheochromocytomas. In addition, most paragangliomas located below the dia­phragm can be resected with a minimally invasive approach which, however, demands exceptional expertise. Open transabdominal resections are an approved therapy for large or potentially ­malignant tumours and for settings with multi­focal tumour sites. Even for advanced malignant tumours, surgical debulking may be reasonable to improve the patient’s quality of life and prognosis.

Schlüsselwörter

Phäochromozytom - Paragangliom - MEN - PGL - endokrine Chirurgie - Viszeralchirurgie

Key words

pheochromocytoma - paraganglioma - MEN - PGL - endocrine surgery - abdominal surgery

Literatur

• 1 Fränkel F. Ein Fall von doppelseitigem völlig latent verlaufendem Neben­nierentumor und gleichzeitiger Nephritis mit Veränderungen am Circulationsapparat und Retinitis. Virchows Arch Pathol Physiol. 1886; 103 224-263
• 2 McNicol A M, Young W F, Kawashima A et al. Benign pheochromocytoma. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds. World Health Organization Classification of Tumors pathology and genetics: Tumors of Endocrine Organs. Lyon: IARC Press; 2004: 151–155
• 3 Kimura N, Chetty R, Capella C et al. Extra-adrenal paraganglioma: ­ca­rotid body, jugulotympanic, vagal, laryngeal, aortico-pulmonary. In: ­DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds. World Health Organization Classification of Tumors pathology and genetics: Tumors of Endocrine Organs. Lyon: IARC Press; 2004: 159–161
• 4 Neumann H P, Eng C. The approach to the patient with paraganglioma. J Clin Endocrinol Metab. 2009; 94 2677-2683
• 5 Tischler A S, Kimura N, McNicol A M. Pathology of pheochromocytoma and extra-adrenal paraganglioma. Ann N Y Acad Sci. 2006; 1073 557-570
• 6 Stenstrom G, Svardsudd K. Pheochromocytoma in Sweden 1958–1981. An analysis of the National Cancer Registry Data. Acta Med Scand. 1986; 220 225-232
• 7 Beard C M, Carney J A, Sheps S G et al. Incidence of phaeochromocytoma. J Hum Hypertens. 1989; 3 481
• 8 McNichol A M. Differential diagnosis of pheochromocytomas and paragangliomas. Endocr Pathol. 2001; 12 407-415
• 9 Erickson D, Kudva Y C, Ebersold M J et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endo­crinol Metab. 2001; 86 5210-5216
• 10 Kopetschke R, Slisko M, Kilisli A et al. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol. 2009; 161 355-361
• 11 Karagiannis A, Mikhailidis D P, Athyros V G et al. Pheochromocytoma: an update on genetics and management. Endocr Relat Cancer. 2007; 14 935-956
• 12 Kloppel G. Tumoren des Nebennierenmarks und der Paraganglien. ­Pathologe. 2003; 24 280-286
• 13 Neumann H P, Bausch B, McWhinney S R et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002; 346 1459-1466
• 14 Amar L, Bertherat J, Baudin E et al. Genetic testing in pheochromo­cytoma or functional paraganglioma. J Clin Oncol. 2005; 23 8812-8818
• 15 Gimm O. Pheochromocytoma-associated syndromes: genes, proteins and functions of RET, VHL and SDHx. Fam Cancer. 2005; 4 17-23
• 16 Waldmann J, Bartsch D K, Kann P H et al. Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. Langenbecks Arch Surg. 2007; 392 437-443
• 17 van der Harst E, Bruining H A, Jaap Bonjer H et al. Proliferative index in phaeochromocytomas: does it predict the occurrence of metastases?. J Pathol. 2000; 191 175-180
• 18 Thompson L DR, Young W F, Kawashima A et al. Malignant adrenal phaeochromocytoma. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds. World Health Organization Classification of Tumors pathology and ­genetics: Tumors of Endocrine Organs. Lyon: IARC Press; 2004: 147–150
• 19 Scholz T, Eisenhofer G, Pacak K et al. Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab. 2007; 92 1217-1225
• 20 Shen W T, Sturgeon C, Clark O H et al. Should pheochromocytoma size influence surgical approach? A comparison of 90 malignant and 60 benign pheochromocytomas. Surgery. 2004; 136 1129-1137
• 21 Wu D, Tischler A S, Lloyd R V et al. Observer variation in the application of the Pheochromocytoma of the Adrenal Gland Scaled Score. Am J Surg Pathol. 2009; 33 599-608
• 22 Wangberg B, Muth A, Khorram-Manesh A et al. Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci. 2006; 1073 512-516
• 23 Eisenhofer G, Goldstein D S, Walther M M et al. Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. J Clin Endocrinol Metab. 2003; 88 2656-2666
• 24 Jimenez C, Cote G, Arnold A et al. Review: Should patients with appar­ently sporadic pheochromocytomas or paragangliomas be screened for hereditary syndromes?. J Clin Endocrinol Metab. 2006; 91 2851-2858
• 25 Greenblatt D Y, Shenker Y, Chen H. The utility of metaiodobenzylguanidine (MIBG) scintigraphy in patients with pheochromocytoma. Ann Surg Oncol. 2008; 15 900-905
• 26 Mihai R, Gleeson F, Roskell D et al. Routine preoperative (123)I-MIBG scintigraphy for patients with phaeochromocytoma is not necessary. Langenbecks Arch Surg. 2008; 393 725-727
• 27 Taieb D, Tessonnier L, Sebag F et al. The role of 18F-FDOPA and 18F-FDG-PET in the management of malignant and multifocal phaeochromo­cytomas. Clin Endocrinol (Oxf). 2008; 69 580-586
• 28 Apgar V, Papper E M. Pheochromocytoma. Anesthetic management during surgical treatment. AMA Arch Surg. 1951; 62 634-648
• 29 Graham J B. Pheochromocytoma and hypertension; an analysis of 207 cases. Int Abstr Surg. 1951; 92 105-121
• 30 Weismann D, Fassnacht M, Weinberger F et al. Intraoperative haemo­dynamic stability in patients with phaeochromocytoma – minimally invasive vs conventional open surgery. Clin Endocrinol (Oxf). 2006; 65 352-358
• 31 Gray D K, Thompson N W. Pheochromocytoma. In: Doherty GM, Skogseid B, eds. Surgical Endocrinology. Philadelphia: Lippincott Wil­liams & Wil­kins; 2001: 247–261
• 32 Prichard B N, Ross E J. Use of propranolol in conjunction with alpha ­receptor blocking drugs in pheochromocytoma. Am J Cardiol. 1966; 18 394-398
• 33 Proye C, Fossati P, Fontaine P et al. Dopamine-secreting pheochromocytoma: an unrecognized entity? Classification of pheochromocytomas according to their type of secretion. Surgery. 1986; 100 1154-1162
• 34 Walz M K, Petersenn S, Koch J A et al. Endoscopic treatment of large primary adrenal tumours. Br J Surg. 2005; 92 719-723
• 35 Brauckhoff M, Gimm O, Dralle H. Preoperative and surgical therapy in sporadic and familial pheochromocytoma. Front Horm Res. 2004; 31 121-144
• 36 Walz M K, Peitgen K, Neumann H P et al. Endoscopic treatment of solitary, bilateral, multiple, and recurrent pheochromocytomas and paragangliomas. World J Surg. 2002; 26 1005-1012
• 37 Musholt T J. Surgical management of advanced malignant adrenal ­tumors. Eur Surg. 2003; 35 97-101
• 38 Hanazawa K, Koizumi K, Tanaka M et al. Total replacement of the aorta and the inferior vena cava after resection of an extra-adrenal malignant pheochromocytoma. Urol Int. 2000; 64 108-110
• 39 Chrisoulidou A, Kaltsas G, Ilias I et al. The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer. 2007; 14 569-585
• 40 Forrer F, Riedweg I, Maecke H R et al. Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging. 2008; 52 334-340
• 41 Averbuch S D, Steakley C S, Young R C et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988; 109 267-273
• 42 Huang H, Abraham J, Hung E et al. Treatment of malignant pheochromocytoma / paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer. 2008; 113 2020-2028
• 43 Eisenhofer G, Bornstein S R, Brouwers F M et al. Malignant pheochromocytoma: current status and initiatives for future progress. Endocr Relat Cancer. 2004; 11 423-436
• 44 Pacak K, Linehan W M, Eisenhofer G et al. Recent advances in genet­ics, diagnosis, localization, and treatment of pheochromocytoma. Ann Intern Med. 2001; 134 315-329

Prof. Thomas J. Musholt

Johannes Gutenberg-Universität Mainz · Endokrine Chirurgie, Klinik für Allgemein- und Abdominalchirurgie

Langenbeckstr. 1

55101 Mainz

Deutschland

Phone: +49 / 61 31 / 17 71 79

Fax: +49 / 61 31 / 17 47 71 79

Email: musholt@uni-mainz.de