Gastrointestinal neuroendocrine tumours. Prospective analysis of 29 patients and literature
review
Summary
Neuroendocrine tumors are rare and can be observed in different gastrointestinal organs.
The diagnosis often is made by incidence like in carcinoids of the appendix vermiformis,
or special clinical symptoms may develop due to tumor growth. 29 consecutive patients
were evaluated prospectively during 30 months. In 21 of them, the first diagnosis
was obtained after an operative procedure. In 3 cases, the tumor was found incidentally
during another oncological procedure. In 4 patients, the histological examination
of biopsy specimens had shown an undifferentiated adenocarcinoma. In 8 of the patients,
recurrent surgery was necessary because of recurrency of the disease. The mean observation
time of all patients was 10.6 months. 6 of 29 patients died on disease-related symptoms.
The results are discussed based on the literature with special regard to organ-related
characteristics of different gastrointestinal neuroendocrine tumors.
Zusammenfassung
Neuroendokrine Tumoren des Gastrointestinaltraktes sind selten und werden in verschiedenen
Organen mit unterschiedlicher Häufigkeit beobachtet. Die Diagnose wird entweder zufällig
gestellt wie beim Befall der Appendix vermiformis, oder wenn eine Organmanifestation
zu einer definierten klinischen Symptomatik führt. Prospektiv erfaßt und ausgewertet
wurden 29 konsekutive Patienten in 30 Monaten, bei 21 davon als Erstdiagnose. Bei
3 Patienten wurde ein Zweittumor im Rahmen einer onkologischen Operation gefunden,
bei weiteren 4 Patienten deutete die Biopsie auf ein entdifferenziertes Adenokarzinom.
Bei 8 Patienten wurden Folgeoperationen durchgeführt. Bei einer mittleren Nachbeobachtungszeit
von 10,6 Monaten verstarben 6 von 29 Patienten in der Mehrzahl an den Folgen eines
Rezidivs. Die Ergebnisse des eigenen Krankengutes werden unter Berücksichtigung organtypischer
Besonderheiten mit der Literatur diskutiert.
Key words
Gastrointestinal neuroendocrine tumours - prospective observation study
Schlüsselwörter
Gastrointestinale neuroendokrine Tumoren - Prospektive Erfassung von 29 Patienten
Literatur
- 1
Ahlmann H I, Wängberg B, Nillson O, Grimelius G, Granerus G, Modlin M, Stenquist T,
Schersten T.
Aspects on diagnosis and treatment of foregut carcinoid syndrome.
Scand J Gastroenterol.
1992;
27
459-471
- 2
Ahlmann H, Kölby L, Lundell L, Olbe L, Wängberg B, Granerus G, Grimelius L, Nilsson O.
Clinical management of gastric carcinoid tumors.
Digestion.
1994;
55
77-85
- 3
Al-Khafai J B, Noffsinger A E, Miller M A, Devoe G, Stemmermann G N, Fenoglio-Preiser C.
Immunohistologic analysis of gastrointestinal and pulmonary carcinoid tumors.
Human Pathol.
1998;
29
992-999
- 4
Allison D J, Modlin I M, Jenkins W J.
Treatment of carcinoid liver metastases by hepatic-artery embolisation.
Lancet.
1997;
II
1323-1325
- 5
Arnold J C, O'Grady J G, Bird G L, Calne R Y.
Liver transplantation for primary and secondary hepatic apudomas.
Br J Surg.
1989;
76
248-249
- 6
Arnold R.
Medical treatment of metastasizing carcinoid tumors.
World J Surg.
1996;
30
203-207
- 7
Arnold R, Trautmann M E, Creutzfeldt W, Benning R, Benning M, Neuhaus C, Jürgensen R,
Stein K, Schäfer H, Bruns C, Dennler H-J. and the German Sandostatin Multicentre Study
Group .
Somatostatin analogue octreotide and inhibition of tumor growth in metastatic endocrine
gastroenteropancreatic tumours.
Gut.
1996;
38
430-438
- 8 Azoulay D, Bismuth H. Role of liver surgery and transplantation in patients with
hepatic metastases from pancreatic endocrine tumors. In: Mignon M, Jensen R T (Hrsg)
Endocrine tumors of the pancreas. Karger, Basel 1995; 461-476
- 9
Becker H D, Gabriel A.
Therapie der Karzinoide des Magens.
Arch Klin Chir.
1996;
381
18-22
- 10
von Bubnoff A Ch, Martin W R, Adamek H E, Riemann J F.
Karzinoide des Gastrointestinaltraktes.
Dtsch Med Wochenschr.
1997;
122
901-907
- 11
Caldarola V T, Jackman R J, Moertel C G.
Carcinoid tumors of the rectum.
Am J Surg.
1964;
107
844-849
- 12
Capella U, Heitz P U, Höfler H, Solcia E, Klöppel G.
Revised classification of neuroendocrine tumours of the lung, pancreas and gut.
Virchows Arch.
1995;
425
547-560
- 13
Caplin M E, Buscombe J R, Hilson A J, Jones A L, Watkinson A F, Burroughs A K.
Carcinoid tumour.
Lancet.
1998;
352
799-805
- 14
Cassidy M.
Abdominal carcinomatosis associated with vasomotor disturbances.
Proc Roy Soc Med.
1934;
27
220-221
- 15
Coupe M, Levi S, Ellis M, Clarke B, Morris J A, Alstead E A, Allison D J, Hodgson H J F.
Therapy for symptoms in the carcinoid syndrome.
Q J Med.
1989;
73
1021-1036
- 16
Creutzdeldt W.
Historical background and natural history of carcinoids.
Digestion.
1994;
55
3-10
- 17
Eller R, Frazee R, Roberts J.
Gastrointestinal carcinoid tumors.
Am Surg.
1991;
57
434-437
- 18 Encke A, Wenisch H J C. Indikation Zur Lebertransplantation. In: Caspary W F, Leuschner
U, Zeuzem S (Hrsg). Therapie von Leber- und Gallekrankheiten. Springer, Berlin, Heidelberg,
New York 1997; 329-338
- 19
Frilling A, Rogiers X, Knöfel W T, Broelsch C E.
Liver transplantation for metastatic carcinoid tumors.
Digestion.
1994;
55
104-106
- 20
Godwin J D.
Carcinoid tumors: An analysis of 2 837 cases.
Cancer.
1975;
36
560-569
- 21
Gough D B, Thompson G B, Crotty T B.
The diverse clinical and pathological features of gastric carcinoid and the relevance
of hypergastrinaemia.
World J Surg.
1994;
18
- 22
Gouzi J L. and the French Association for Surgical Research .
Indications for right hemicolectomy in carcinoid tumors of the appendix.
Surg Gynecol Obstet.
1993;
176
543-548
- 23
Jacobsen M B, Hanssen L E, Kolmannskog F, Schrumpf E, Vatn M H, Bergan A.
Interferon-a2b, with or without prior hepatic artery embolization: clinical response
and survival in mid-gut carcinoid patients.
Scand J Gastroenterol.
1995;
30
789-796
- 24
Jetmore A B, Ray J E, Gathright J B.
Rectal carcinoids: the most frequent carcinoid tumor.
Dis Colon Rectum.
1992;
35
717-725
- 25
Klöppel G.
Classification of neuroendocrine tumors.
Verh Dtsch Ges Path.
1997;
81
111-117
- 26
Kulke M H, Mayer R J.
Carcinoid tumors.
New Engl J Med.
1999;
340
858-868
- 27
Kvols L K.
Therapy of the malignant carcinoid syndrome.
Endocrinol Metab Clin North Am.
1989;
18
557-568
- 28 Kwekkeboom D J, Krenning E P, Oei H Y, Van Eyck C H J, Lamberts S W J. Use of radiolabeled
somatostatin to localize islet cell tumors. In: Mignon M, Jensen R T (Hrsg). Endocrine
tumors of the pancreas. Karger, Basel 1995; 298-308
- 29
Lamberts S W J, Van Der Lely A-J, De Herder W W, Hofland L J.
Octreotide.
New Engl J Med.
1996;
334
246-254
- 30
Lembeck F.
5-Hydroxytryptamine in a carcinoid tumor.
Nature.
1953;
172
910-911
- 31
Loftus J P, Van Heerden J A.
Surgical management of gastrointestinal carcinoid tumors.
Advanc Surg.
1995;
28
317-336
- 32
Lubarsch O.
Über den primären Krebs des Ileums. Bemerkungen über das gleichzeitige Vorkommen von
Krebs und Tuberculose.
Arch Pathol Anat.
1908;
111
280-317
- 33
Makowka M, Tsakis A G, Mazzaferro V, Tepermann L, Demetris A J, Iwatsuki S, Starzl T E.
Transplantation of the liver for metastatic endocrine tumors of the intestine and
the pancreas.
Surg Gynecol Obstet.
1989;
168
107-111
- 34
Makridis C, Oberg K, Juhlin C.
Surgical treatment of mid-gut carcinoid tumors.
World J Surg.
1990;
14
377-385
- 35
Martensen H, Nobin A, Sundler F.
Carcinoid tumors in the gastrointestinal tract - analysis of 156 cases.
Acta Chir Scand.
1983;
149
607-616
- 36
Maurer C A, Glaser C, Reubi J C, Büchler M W.
Carcinoid of the pancreas.
Digestion.
1997;
58
410-414
- 37
Moertel C G.
An odyssey in the land of small tumors.
J Clin Oncol.
1987;
5
1503-1522
- 38
Moertel C G, Sauer W G, Dockerty M B.
Life history of the carcinoid tumor of the small intestine.
Cancer.
1961;
14
901-908
- 39
Moertel C G, Weiland L H, Nagorney D L.
Carcinoid tumor of the appendix: Treatment and prognosis.
New Engl J Med.
1987;
317
1699-1701
- 40
Moertel C G, Dockerty M B, Judd E S.
Carcinoid tumors of the vermiform appendix.
Cancer.
1968;
21
270-278
- 41 Nagorney D M, Que F G. Cytoreductive hepatic surgery for metastatic gastrointestinal
neuroendocrine tumors. In: Mignon M, Jensen RT (Hrsg). Endocrine tumors of the pancreas.
Karger, Basel 1995; 416-430
- 42
Norton J A.
Surgical management of carcinoid tumors: role of debulking and surgery for patients
with advanced disease.
Digestion.
1994;
55
98-103
- 43 Norton A J, Levin B, Jensen R T. Cancer of the endocrine system. In: de Vita VTS,
Hellmann Jr SA, Rosenberg, SA (Hrsg). Cancer principles and practice of oncology.
Lippincott, Philadelphia 1992; 1 333-1 435
- 44
Oberndorfer S.
Ueber die „kleinen Dünndarmcarcinome”.
Verh Dtsch Ges Pathol.
1907;
11
1213-1216
- 45
Ormann W.
Autoimmunhepatitis, Autoimmungastritis, Hypergastrinämie und Magenkarzinoide.
Dtsch Med Wochenschr.
1995;
120
361-365
- 46
Rindi G, Luinetti O, Cornaggia M, Capella C, Solcia E.
Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma.
A clinicopathologic study.
Gastroenterol.
1993;
104
994-1006
- 47
Rothmund M, Kisker O.
Surgical treatment of carcinoid tumors of the small bowel, appendix, colon and rectum.
Digestion.
1994;
55
86-91
- 48
Scheele J.
Hepatectomy for liver metastases.
Br J Surg.
1993;
80
274-276
- 49
Sjoblom S M.
Clinical presentation and prognosis of gastrointestinal carcinoid tumours.
Scand J Gastroenterol.
1988;
23
779-787
- 50
Stinner B.
Surgical management for carcinoid tumors of the small bowel, appendix, colon, and
rectum.
World J Surg.
1996;
20
183-186
- 51
Stolte M, Sticht T, Eidt S, Ebert D, Finkenzeller G.
Frequency, location and sex distribution of various types of gastric polyp.
Endoscopy.
1994;
26
659-665
- 52
Teleky B, Herbst F, Langl F.
The prognosis of rectal carcinoid tumors.
Int J Colorectal Dis.
1992;
7
11-14
- 53
Timmermann W, Illert B, Thiede A.
Die Karzinoide des Gastrointestinaltraktes.
Chir Praxis.
1997;
52
641-650
- 54
Thompson G B, Van Heerden J A, Martin J K.
Carcinoid tumors of the gastrointestinal tract: Presentation, management, and prognosis.
Surgery.
1985;
98
1054-1062
- 55
Thorson A, Björk G, Bjorkman G, Waldenström J.
Malignant carcinoid of the small intestine with metastases to the liver, valvular
disease of the right heart (pulmonary stenosis and tricuspid regurgitation without
septal defect), peripheral vasomotor symptoms, bronchoconstriction and an unusual
type of cyanosis.
Am Heart J.
1954;
47
795-817
- 56
Tsakis A G, Todo S, Starzl T E.
Upper abdominal exenteration with liver replacement: A modification of the “cluster”
procedure.
Transplanation Proc.
1990;
22
273-274
- 57
Wenisch H J C.
Neuroendokrine Tumoren des Gastrointestinaltraktes.
Acta Chir Austriaca Suppl.
1998;
144
10
- 58
Wenisch H J C, Markus B H, Herrmann G H, Usadel K H, Encke A.
Multiviszerale Oberbauchexenteration und orthotope Lebertransplantation - ein chirurgisches
Behandlungskonzept für regionär metastasierende Tumoren des endocrinen Pancreas.
Zentralbl Chir.
1992;
117
334-342
- 59
Williams E D, Sandler M.
The classification of carcinoid tumors.
Lancet.
1963;
I
238-239
- 60
Yoshikane H, Suzuki T, Yoshioka N, Ogawa Y, Hamajima E, Hasegawa N, Hasegawa C.
Duodenal carcinoid tumors. Endoscopic imaging and endoscopic resection.
Am J Gastroenterol.
1995;
90
642-644
Prof. Dr. H. J.C. Wenisch
Chirurgische Klinik - Allgemein- und Visceralchirurgie
Klinikum Ernst von Bergmann
Charlottenstraße 72
D-14467 Potsdam
Telefon: Tel.: 03 31/2 41-52 01
Fax: Fax: 03 31/2 41-52 00