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Published in: Journal of Gastrointestinal Surgery 1/2011

01-01-2011 | Original Article

Neuroendocrine Tumors of Meckel’s Diverticulum: Lessons from a Single Institution Study of Eight Cases

Authors: Gilles Poncet, MD, Valérie Hervieu, MD, Thomas Walter, MD, Florian Lépinasse, BSc, Laurence Chardon, MD, Frank Pilleul, MD, Catherine Lombard-Bohas, MD, Jean-Alain Chayvialle, MD, Christian Partensky, MD, Jean-Yves Scoazec, MD

Published in: Journal of Gastrointestinal Surgery | Issue 1/2011

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Abstract

Introduction

Endocrine tumors of Meckel’s diverticulum are rare. Their clinical and pathological characteristics are not well known, making it difficult to assess the best strategy for therapeutic management.

Materials and Methods

Eight cases of endocrine tumors of Meckel’s diverticulum, submitted to surgical resection in our institution between 1977 and 2009, were studied. Clinical charts were reviewed; classification, grading, and staging were performed according to recent international recommendations. Five cases, including two associated with the carcinoid syndrome, were revealed by mesenteric mass or liver metastases; three cases were diagnosed incidentally at laparotomy or laparoscopy.

Results

All cases presented as typical well-differentiated midgut endocrine tumors. Five cases were associated with mesenteric lymph node metastases; three presented with liver metastases. Seven cases were classified as well-differentiated endocrine carcinomas, one as well-differentiated endocrine tumor of benign behavior.

Discussion

All tumors >1 cm, but one, had regional or distant disease. All patients had complete surgical resection of the primary. One patient deceased after 25 months; the others were alive after 12–101 months.

Conclusion

In conclusion, endocrine tumors of Meckel’s diverticulum are rarely symptomatic and often diagnosed at an advanced stage. All tumors measuring more than 1 cm in diameter must be resected according to oncological principles.
Literature
1.
go back to reference Stone PA, Hofeldt MJ, Campbell JE, Vedula G, DeLuca JA, Flaherty SK. Meckel diverticulum: ten-year experience in adults. South Med J 2004;97:1038–1041.PubMedCrossRef Stone PA, Hofeldt MJ, Campbell JE, Vedula G, DeLuca JA, Flaherty SK. Meckel diverticulum: ten-year experience in adults. South Med J 2004;97:1038–1041.PubMedCrossRef
2.
go back to reference Ciardo LF, Agresta F, Bedin N. Meckel’s diverticulum: a neglected (or deliberately ignored) entity. Chir Ital 2004;56:689–692.PubMed Ciardo LF, Agresta F, Bedin N. Meckel’s diverticulum: a neglected (or deliberately ignored) entity. Chir Ital 2004;56:689–692.PubMed
3.
go back to reference Modlin IM, Shapiro MD, Kidd M. An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg 2005;29:92–101.PubMedCrossRef Modlin IM, Shapiro MD, Kidd M. An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg 2005;29:92–101.PubMedCrossRef
4.
go back to reference Solcia E, Klöppel G, Sobin L. Histological typing of endocrine tumours. 2nd edition. New York:Springer, 2000 Solcia E, Klöppel G, Sobin L. Histological typing of endocrine tumours. 2nd edition. New York:Springer, 2000
5.
go back to reference Rindi G, Kloppel G, Alhman H, Caplin M, Couvelard A, de Herder WW, Erikssson B, Falchetti A, Falconi M, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2006;449:395–401.PubMedCrossRef Rindi G, Kloppel G, Alhman H, Caplin M, Couvelard A, de Herder WW, Erikssson B, Falchetti A, Falconi M, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2006;449:395–401.PubMedCrossRef
6.
go back to reference Rindi G, Kloppel G, Couvelard A, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2007;451:757–762.PubMedCrossRef Rindi G, Kloppel G, Couvelard A, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2007;451:757–762.PubMedCrossRef
7.
go back to reference Sobin LH, Gospodarowicz MK, Wittekind C. TNM Classification of Malignant Tumours, 7th Edition. New York: Wiley, 2009. Sobin LH, Gospodarowicz MK, Wittekind C. TNM Classification of Malignant Tumours, 7th Edition. New York: Wiley, 2009.
8.
go back to reference Arnold C, Marjoniemi V. Islet of Langerhans heterotopia in Meckel’s diverticulum. Pathology 2006;38:452–454.PubMedCrossRef Arnold C, Marjoniemi V. Islet of Langerhans heterotopia in Meckel’s diverticulum. Pathology 2006;38:452–454.PubMedCrossRef
9.
go back to reference Grossmann I, Akkersdijk GJ. Carcinoid tumor in a Meckel’s diverticulum: hypothesis on mutual embryological origin. Int Surg 2003;88:41–46.PubMed Grossmann I, Akkersdijk GJ. Carcinoid tumor in a Meckel’s diverticulum: hypothesis on mutual embryological origin. Int Surg 2003;88:41–46.PubMed
10.
go back to reference Moyana TN. Carcinoid tumors arising from Meckel’s diverticulum. A clinical, morphologic, and immunohistochemical study. Am J Clin Pathol 1989;91:52–56.PubMed Moyana TN. Carcinoid tumors arising from Meckel’s diverticulum. A clinical, morphologic, and immunohistochemical study. Am J Clin Pathol 1989;91:52–56.PubMed
11.
go back to reference Carpenter SS, Grillis ME. Meckel’s diverticulitis secondary to carcinoid tumor: an unusual presentation of the acute abdomen in an adult. Curr Surg 2003;60:301–303.PubMedCrossRef Carpenter SS, Grillis ME. Meckel’s diverticulitis secondary to carcinoid tumor: an unusual presentation of the acute abdomen in an adult. Curr Surg 2003;60:301–303.PubMedCrossRef
12.
go back to reference Coyne JD, Dervan PA. Ileal intussuseptus containing a Meckel’s diverticulum showing florid localized mucosal angiogenesis and microcarcinoidosis. Histopathology 2003;43:608–609.PubMedCrossRef Coyne JD, Dervan PA. Ileal intussuseptus containing a Meckel’s diverticulum showing florid localized mucosal angiogenesis and microcarcinoidosis. Histopathology 2003;43:608–609.PubMedCrossRef
13.
go back to reference Green M, Oratz R, Muggia FM. Carcinoid syndrome from a tumor of Meckel’s diverticulum. Am J Med 1987;12:184–186.CrossRef Green M, Oratz R, Muggia FM. Carcinoid syndrome from a tumor of Meckel’s diverticulum. Am J Med 1987;12:184–186.CrossRef
14.
go back to reference Pyke CM, Lancaster BA, van Heerden JA, Kvols LK. Carcinoid syndrome secondary to a primary tumour in a Meckel’s diverticulum. Aust N Z J Surg 1993;63:732–734.PubMedCrossRef Pyke CM, Lancaster BA, van Heerden JA, Kvols LK. Carcinoid syndrome secondary to a primary tumour in a Meckel’s diverticulum. Aust N Z J Surg 1993;63:732–734.PubMedCrossRef
15.
go back to reference La Rosa S, Rigoli E, Uccella S, Chiaravalli AM, Capella C. CDX2 as a marker of intestinal EC-cells and related well-differentiated endocrine tumors. Virchows Arch 2004;445:248–254.PubMedCrossRef La Rosa S, Rigoli E, Uccella S, Chiaravalli AM, Capella C. CDX2 as a marker of intestinal EC-cells and related well-differentiated endocrine tumors. Virchows Arch 2004;445:248–254.PubMedCrossRef
16.
go back to reference Saqi A, Alexis D, Remotti F, Bhagat G. Usefulness of CDX2 and TTF-1 in differentiating gastrointestinal from pulmonary carcinoids. Am J Clin Pathol 2005;123:394–404.PubMedCrossRef Saqi A, Alexis D, Remotti F, Bhagat G. Usefulness of CDX2 and TTF-1 in differentiating gastrointestinal from pulmonary carcinoids. Am J Clin Pathol 2005;123:394–404.PubMedCrossRef
17.
go back to reference Jaffee IM, Rahmani M, Singhal MG, Younes M. Expression of the intestinal transcription factor CDX2 in carcinoid tumors is a marker of midgut origin. Arch Pathol Lab Med 2006;130:1522–1526.PubMed Jaffee IM, Rahmani M, Singhal MG, Younes M. Expression of the intestinal transcription factor CDX2 in carcinoid tumors is a marker of midgut origin. Arch Pathol Lab Med 2006;130:1522–1526.PubMed
18.
go back to reference Lundqvist M, Wilander E. Subepithelial neuroendocrine cells and carcinoid tumours of the human small intestine and appendix. A comparative immunohistochemical study with regard to serotonin, neuron-specific enolase and S-100 protein reactivity. J Pathol 1986;148:141–147.PubMedCrossRef Lundqvist M, Wilander E. Subepithelial neuroendocrine cells and carcinoid tumours of the human small intestine and appendix. A comparative immunohistochemical study with regard to serotonin, neuron-specific enolase and S-100 protein reactivity. J Pathol 1986;148:141–147.PubMedCrossRef
19.
go back to reference Rasmussen OO, Rafiolsadat Z, Berg J. Carcinoid and pancreas tissue in a macroscopically normal Meckel’s diverticulum. Ugeskr Laeger 1987;83:2538. Rasmussen OO, Rafiolsadat Z, Berg J. Carcinoid and pancreas tissue in a macroscopically normal Meckel’s diverticulum. Ugeskr Laeger 1987;83:2538.
20.
go back to reference Scognamiglio F, Panico L, Petrillo O, Fusco B, Terracciano LM, Ferrara G. Carcinoid associated with pancreatic heterotopia in Meckel’s diverticulum. The clinical, morphological and ultrastructural aspects of a case. Minerva Chir 1990;45:1043–1047.PubMed Scognamiglio F, Panico L, Petrillo O, Fusco B, Terracciano LM, Ferrara G. Carcinoid associated with pancreatic heterotopia in Meckel’s diverticulum. The clinical, morphological and ultrastructural aspects of a case. Minerva Chir 1990;45:1043–1047.PubMed
21.
go back to reference Weber JD, McFadden DW. Carcinoid tumors in Meckel’s diverticula. J Clin Gastroenterol 1989;11:682–686.PubMedCrossRef Weber JD, McFadden DW. Carcinoid tumors in Meckel’s diverticula. J Clin Gastroenterol 1989;11:682–686.PubMedCrossRef
22.
go back to reference Nies C, Zielke A, Hasse C, Ruschoff J, Rothmund M. Carcinoid tumors of Meckel’s diverticula. Report of two cases and review of the literature. Dis Colon Rectum 1992;35:589–596PubMedCrossRef Nies C, Zielke A, Hasse C, Ruschoff J, Rothmund M. Carcinoid tumors of Meckel’s diverticula. Report of two cases and review of the literature. Dis Colon Rectum 1992;35:589–596PubMedCrossRef
23.
go back to reference Plockinger U, Couvelard A, Falconi M, Sundin A, Salazar R, Christ E, de Herder WW, Gross D, Knapp WH, Knigge UP, Kulke MH, Pape UF. Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma. Neuroendocrinology 2008;87:20–30.PubMedCrossRef Plockinger U, Couvelard A, Falconi M, Sundin A, Salazar R, Christ E, de Herder WW, Gross D, Knapp WH, Knigge UP, Kulke MH, Pape UF. Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma. Neuroendocrinology 2008;87:20–30.PubMedCrossRef
24.
go back to reference Eriksson B, Kloppel G, Krenning E, Ahlman H, Plockinger U, Wiedenmann B, Arnold R, Auernhammer C, Korner M, Rindi G, Wildi S. Consensus guidelines for the management of patients with digestive neuroendocrine tumors-well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology 2008;87:8–19.PubMedCrossRef Eriksson B, Kloppel G, Krenning E, Ahlman H, Plockinger U, Wiedenmann B, Arnold R, Auernhammer C, Korner M, Rindi G, Wildi S. Consensus guidelines for the management of patients with digestive neuroendocrine tumors-well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology 2008;87:8–19.PubMedCrossRef
25.
go back to reference Poncet G, Faucheron JL, Walter T. Recent trends in the treatment of well-differentiated endocrine carcinoma of the small bowel. World J Gastroenterol 2010;16:1696–1706.PubMedCrossRef Poncet G, Faucheron JL, Walter T. Recent trends in the treatment of well-differentiated endocrine carcinoma of the small bowel. World J Gastroenterol 2010;16:1696–1706.PubMedCrossRef
26.
go back to reference Eriksson B, Kloppel G, Krenning E, Ahlman H, Plockinger U, Wiedenmann B, Arnold R, Auernhammer C, Korner M, Rindi G, Wildi S. Consensus guidelines for the management of patients with digestive neuroendocrine tumors—well-differentiated jejunal–ileal tumor/carcinoma. Neuroendocrinology 2008;87:8–19.PubMedCrossRef Eriksson B, Kloppel G, Krenning E, Ahlman H, Plockinger U, Wiedenmann B, Arnold R, Auernhammer C, Korner M, Rindi G, Wildi S. Consensus guidelines for the management of patients with digestive neuroendocrine tumors—well-differentiated jejunal–ileal tumor/carcinoma. Neuroendocrinology 2008;87:8–19.PubMedCrossRef
27.
go back to reference Silk YN, Douglass HO Jr, Penetrante R. Carcinoid tumor in Meckel’s diverticulum. Am Surg 1988;83:664–667. Silk YN, Douglass HO Jr, Penetrante R. Carcinoid tumor in Meckel’s diverticulum. Am Surg 1988;83:664–667.
28.
go back to reference Robijn J, Sebrechts E, Miserez M. Management of incidentally found Meckel’s diverticulum a new approach: resection based on a Risk Score. Acta Chir Belg 2006;106:467–470.PubMed Robijn J, Sebrechts E, Miserez M. Management of incidentally found Meckel’s diverticulum a new approach: resection based on a Risk Score. Acta Chir Belg 2006;106:467–470.PubMed
29.
go back to reference Anderson DJ. Carcinoid tumor in Meckel’s diverticulum: laparoscopic treatment and review of the literature. J Am Osteopath Assoc 2000;100:432–434.PubMed Anderson DJ. Carcinoid tumor in Meckel’s diverticulum: laparoscopic treatment and review of the literature. J Am Osteopath Assoc 2000;100:432–434.PubMed
30.
go back to reference Bona D, Schipani LS, Nencioni M, Rubino B, Bonavina L. Laparoscopic resection for incidentally detected Meckel diverticulum. World J Gastroenterol 2008;14:49614963.PubMedCrossRef Bona D, Schipani LS, Nencioni M, Rubino B, Bonavina L. Laparoscopic resection for incidentally detected Meckel diverticulum. World J Gastroenterol 2008;14:49614963.PubMedCrossRef
31.
go back to reference Palanivelu C, Rangarajan M, Senthilkumar R, Madankumar MV, Kavalakat AJ. Laparoscopic management of symptomatic Meckel’s diverticula: a simple tangential stapler excision. JSLS 2008;12:66–70.PubMed Palanivelu C, Rangarajan M, Senthilkumar R, Madankumar MV, Kavalakat AJ. Laparoscopic management of symptomatic Meckel’s diverticula: a simple tangential stapler excision. JSLS 2008;12:66–70.PubMed
Metadata
Title
Neuroendocrine Tumors of Meckel’s Diverticulum: Lessons from a Single Institution Study of Eight Cases
Authors
Gilles Poncet, MD
Valérie Hervieu, MD
Thomas Walter, MD
Florian Lépinasse, BSc
Laurence Chardon, MD
Frank Pilleul, MD
Catherine Lombard-Bohas, MD
Jean-Alain Chayvialle, MD
Christian Partensky, MD
Jean-Yves Scoazec, MD
Publication date
01-01-2011
Publisher
Springer-Verlag
Published in
Journal of Gastrointestinal Surgery / Issue 1/2011
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI
https://doi.org/10.1007/s11605-010-1312-x

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