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DOI: 10.1055/s-0030-1267204
© Georg Thieme Verlag KG Stuttgart · New York
A Matter of the Heart: Myocardial Metastases in Neuroendocrine Tumors
Publication History
received 22.03.2010
accepted 09.09.2010
Publication Date:
22 October 2010 (online)

Abstract
The aim of the present study was to evaluate frequency, clinical spectrum, and treatment of myocardial metastases in patients with histologically proven neuroendocrine tumors by analysis of our database and literature review. The literature on cardiac metastases in patients with neuroendocrine tumors published from 1973 to the present was reviewed for age, sex, primary tumor localization, metastases, symptoms, complications, treatment, diagnostic methods, and histology. Patient records from our institution were analyzed retrospectively for cardiac metastases detected by any diagnostic means and detailed patient histories are given. 4 patients with myocardial metastases could be identified in our database (n=550) while literature review identified 41 published cases. Mean age at initial diagnosis was 57.5 years (females=13, males=28), primary tumor localizations were foregut (n=7), midgut (n=28), hindgut (n=1), or unknown (n=3). Carcinoid syndrome was reported for 28 patients. Cardiac involvement was right-ventricular only (n=10), left-ventricular only (n=11), or biventricular (n=10). Diagnosis was obtained by echocardiography (n=21), CT/MRI (n=12) and other methods (n=9), or by autopsy (n=9). We describe visualization of cardiac metastases by 68Ga-DOTATOC-PET/CT for the first time. Clinical presentation ranged from asymptomatic patients to cardiac arrest. Follow-up times ranged from <1 month up to 12 years. Clinicians treating patients with neuroendocrine tumors should be aware of the heart as a possible site of metastatic disease. Echocardiography and MRI are the methods of choice for follow-up, while PET/CT might contribute to earlier and more frequent detection. Management of cardiac metastases requires close cooperation between specialists of internal medicine, nuclear medicine, and cardiac surgery.
Key words
neuroendocrine carcinoma - octreoscan - 111In-pentetreotide SPECT/CT - 68Ga-DOTATOC-PET-CT - heart metastases
References
- 1
Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, Caplin M, Delle Fave G, Kaltsas GA, Krenning EP, Moss SF, Nilsson O, Rindi G, Salazar R, Ruszniewski P, Sundin A.
Gastroenteropancreatic neuroendocrine tumours.
Lancet Oncol.
2008;
9
61-72
Reference Ris Wihthout Link
- 2
Pape UF, Berndt U, Muller-Nordhorn J, Bohmig M, Roll S, Koch M, Willich SN, Wiedenmann B.
Prognostic factors of long-term outcome in gastroenteropancreatic neuroendocrine tumours.
Endocr Relat Cancer.
2008;
15
1083-1097
Reference Ris Wihthout Link
- 3
Oberndorfer S.
Karzinoide Tumoren des Dünndarms.
Frankf Z Pathol.
1907;
426-429
Reference Ris Wihthout Link
- 4
Williams ED, Sandler M.
Classification of Carcinoid Tumours.
Lancet.
1963;
1
238-239
Reference Ris Wihthout Link
- 5
Capella C, Heitz PU, Hofler H, Solcia E, Kloppel G.
Revised Classification of Neuroendocrine Tumors of the Lung, Pancreas and Gut.
Virchows Arch.
1995;
425
547-560
Reference Ris Wihthout Link
- 6 Heitz PU, Komminoth P, Perren A, Eng C. Tumours of the endocrine pancreas. In:, De Lellis RA, Lloyd RV, Heitz PU, editors WHO Classification of Tumours, Pathology and Genetics of Tumours of Endocrine Organs. Lyon: IARC Press; 2004: 177-182
Reference Ris Wihthout Link
- 7
Gustafsson BI, Kidd M, Modlin IM.
Neuroendocrine tumors of the diffuse neuroendocrine system.
Curr Opin Oncol.
2008;
20
1-12
Reference Ris Wihthout Link
- 8
Makridis C, Oberg K, Juhlin C, Rastad J, Johansson H, Lorelius LE, Akerstrom G.
Surgical-Treatment of Midgut Carcinoid-Tumors.
World J Surg.
1990;
14
377-385
Reference Ris Wihthout Link
- 9
Halfdanarson TR, Rubin J, Farnell MB, Grant CS, Petersen GM.
Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine
tumors.
Endocr Relat Cancer.
2008;
15
409-427
Reference Ris Wihthout Link
- 10
Hlatky R, Suki D, Sawaya R.
Carcinoid metastasis to the brain.
Cancer.
2004;
101
2605-2613
Reference Ris Wihthout Link
- 11
Patchell RA, Posner JB.
Neurologic Complications of Carcinoid.
Neurology.
1986;
36
745-749
Reference Ris Wihthout Link
- 12
Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK.
Carcinoid Heart-Disease – Clinical and Echocardiographic Spectrum in 74 Patients.
Circulation.
1993;
87
1188-1196
Reference Ris Wihthout Link
- 13
Pandya UH, Pellikka PA, Enriquez-Sarano M, Edwards WD, Schaff HV, Connolly HM.
Metastatic carcinoid tumor to the heart: Echocardiographic-pathologic study of 11
patients.
J Am Coll Cardiol.
2002;
40
1328-1332
Reference Ris Wihthout Link
- 14
Roberts MD.
A Unique Heart Disease Associated With a Unique Cancer: Carcinoid Heart Disease.
Am J Cardiol.
1997;
80
251-256
Reference Ris Wihthout Link
- 15
Maréchaux S, Dagorn J, Gaxotte V, Ennezat PV, Beregi JP, Bauters C, Deklunder G, le Tourneau T.
Myocardial metastasis of a bronchial carcinoid.
Eur Heart J.
2007;
28
391
Reference Ris Wihthout Link
- 16
Patel S, Heetun M, Gurjar SV, Szakacs S.
A rare case of intra-cardiac metastasis from an appendiceal carcinoid tumour without
liver metastases.
Int J Colorectal Dis.
2009;
24
993-994
Reference Ris Wihthout Link
- 17
Patel CN, Anthoney A, Treanor D, Scarsbrook AF.
Solitary Myocardial Metastasis From Small-Bowel Neuroendocrine Carcinoma.
J Clin Oncol.
2009;
27
1724-1726
Reference Ris Wihthout Link
- 18
Howard RJ, Drobac M, Rider WD, Keane TJ, Finlayson J, Silver MD, Wigle ED, Rakowski H.
Carcinoid heart disease: diagnosis by two-dimensional echocardiography.
Circulation.
1982;
66
1059-1065
Reference Ris Wihthout Link
- 19
Baker BJ, Mcnee VD, Scovil JA, Bass KM, Watson JW, Bissett JK.
Tricuspid Insufficiency in Carcinoid Heart-Disease – An Echocardiographic Description.
Am Heart J.
1981;
101
107-108
Reference Ris Wihthout Link
- 20
Koopmans KP, Neels ON, Kema IP, Elsinga PH, Links TP, de Vries EGE, Jager PL.
Molecular imaging in neuroendocrine tumors: Molecular uptake mechanisms and clinical
results.
Crit Rev Oncol Hematol.
2009;
71
199-213
Reference Ris Wihthout Link
- 21
Penz M, Kurtaran A, Vorbeck F, Oberhuber G, Raderer M.
Case 2: Myocardial metastases from a carcinoid tumor.
J Clin Oncol.
2000;
18
1596-1597
Reference Ris Wihthout Link
- 22
Yeung HWD, Imbriaco M, Zhang JJ, Macapinlac H, Goldsmith SJ, Larson SM.
Visualization of myocardial metastasis of carcinoid tumor by indium-111-pentetreotide.
J Nucl Med.
1996;
37
1528-1530
Reference Ris Wihthout Link
- 23
Strosberg J, Hoffe S, Hazelton T, Kvols L.
External beam irradiation of myocardial carcinoid metastases: a case report.
J Med Case Reports.
2007;
1
95-98
Reference Ris Wihthout Link
- 24
Mahmood S, De Llano SRM, Grewal RK.
Focal myocardial uptake in a patient with carcinoid tumor by indium-111-pentetreotide
on SPECT/CT.
Rev Esp Med Nucl.
2008;
27
355-357
Reference Ris Wihthout Link
- 25
Fiebrich HB, Brouwers AH, Links TP, de Vries EGE.
Myocardial metastases of carcinoid visualized by F-18-dihydroxy-phenyl-alanine positron
emission tomography.
Circulation.
2008;
118
1602-1604
Reference Ris Wihthout Link
- 26
Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R.
PROMID Study Group
.
Placebo-controlled, double-blind, prospective, randomized study of the effect of octreotide
LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut
tumors: A report from the PROMID study group.
J Clin Oncol.
2009;
27
4656-4663
Reference Ris Wihthout Link
- 27
Drake WM, Jenkins PJ, Phillips RR, Lowe DG, Grossman AB, Besser GM, Wass JAH.
Intracardiac metastases from neuroendocrine tumours.
Clin Endocrinol.
1997;
46
517-522
Reference Ris Wihthout Link
- 28
Ahlman H, Nilsson O, McNicol AM, Ruszniewski P, Niederle B, Ricke J, Jensen R, Kos-Kudla B, Oberg K, O’Connor JM, Pavel ME, Vullierme MP.
Poorly-differentiated endocrine carcinomas of midgut and hindgut origin.
Neuroendocrinology.
2008;
87
40-46
Reference Ris Wihthout Link
- 29
Schiller VL, Fishbein MC, Siegel RJ.
Unusual Cardiac Involvement in Carcinoid-Syndrome.
Am Heart J.
1986;
112
1322-1323
Reference Ris Wihthout Link
- 30
Moertel CG.
Karnofsky memorial lecture. An odyssey in the land of small tumors.
J Clin Oncol.
1987;
5
1502-1522
Reference Ris Wihthout Link
- 31
Fine SN, Gaynor ML, Isom OW, Dannenberg AJ.
Carcinoid-Tumor Metastatic to the Heart.
Am J Med.
1990;
89
690-692
Reference Ris Wihthout Link
- 32
Lund JT, Ehman RL, Julsrud PR, Sinak LJ, Tajik AJ.
Cardiac Masses – Assessment by MR Imaging.
AJR Am J Roentgenol.
1989;
152
469-473
Reference Ris Wihthout Link
- 33
Knott-Craig CJ, Schaff HV, Mullany CJ, Kvols LK, Moertel CG, Edwards WD, Danielson GK.
Carcinoid Disease of the Heart – Surgical-Management of 10 Patients.
J Thorac Cardiovasc Surg.
1992;
104
475-481
Reference Ris Wihthout Link
- 34
Davis G, Birbeck K, Roberts D, Naqvi N.
Nonvalvular myocardial involvement in metastatic carcinoid disease.
Postgrad Med J.
1996;
72
751-752
Reference Ris Wihthout Link
- 35
Hennington MH, Detterbeck FC, Szwerc MF, Fidler ME.
Invasive carcinoid tumor of the heart.
J Surg Oncol.
1997;
66
264-266
Reference Ris Wihthout Link
- 36
Schlegel PJ, Kralios AC, Terreros DA, Shami PJ.
Malignant carcinoid tumor with myocardial metastases.
Am J Med.
1999;
107
643-645
Reference Ris Wihthout Link
- 37
Kasi VS, Ahsanudin AN, Gilbert C, Orr L, Moran J, Sorrell VL.
Isolated metastatic myocardial carcinoid tumor in a 48-year-old man.
Mayo Clin Proc.
2002;
77
591-594
Reference Ris Wihthout Link
- 38
Shehata BM, Thomas JE, Doudenko-Rufforny I.
Metastatic carcinoid to the conducting system - Is it a rare or merely unrecognized
manifestation of carcinoid cardiopathy? A case report and review of the literature.
Arch Pathol Lab Med.
2002;
126
1538-1540
Reference Ris Wihthout Link
- 39
Wee JO, Sepic JD, Mihaljevic T, Cohn LH.
Metastatic carcinoid tumor of the heart.
Ann Thorac Surg.
2003;
76
1721-1722
Reference Ris Wihthout Link
- 40
Goddard MJ, Atkinson C.
Cardiac metastasis from a bronchial carcinoid: report of a case presenting with diffuse
thickening of the left ventricular wall.
J Clin Pathol.
2004;
57
778-779
Reference Ris Wihthout Link
- 41
Schiavone WA, Baker C, Prasad SK.
Imaging myocardial carcinoid with T2-STIR CMR.
J Cardiovasc Magn Reson.
2008;
10
14-18
Reference Ris Wihthout Link
Correspondence
M. PavelMD
University Medicine Berlin
Charité, Campus Virchow
Klinikum
Augustenburger Platz 1
13353 Berlin
Phone: +49/30/450 653 304
Fax: +49/30/450 553 902
Email: marianne.pavel@charite.de