Gangliozytisches Paraganglinonom der Papilla Vateri

Zusammenfassung

Es wird über ein blutendendes gangliozytisches Paraganglionom (GPG) der Papilla Vateri eines 47-jährigen Mannes berichtet, das erfolgreich durch eine partielle Duodenopankreatektomie entfernt wurde. GPG des Duodenums sind sehr seltene Tumoren, derzeit wurden 81 Fälle in der Literatur beschrieben. Histologisch finden sich typischerweise drei Zelltypen: epithelioide-, spindelige- und ganglionzell-ähnliche Zellen. Überwiegend handelt es sich um benigne Tumoren. Tumorrezidive und Metastasierung der GPG sind jedoch beschrieben. Das in diesem Fall beobachtete infiltrative Wachstum unterstreicht das maligne Potenzial der GPG.

Abstract

A case of a bleeding duodenal gangliocytic paraganglioma (GPG) of the Papilla Vateri is reported. The 47 year old patient was treated successfully by partial pancreaticoduodenectomy. Histologically the GPG is characterized by three types of cells: epitheloid cells, ganglion-like cells and spindle cells. So far 81 cases are reported in the literature. Most cases of GPGs are benign, metastasis or recurrence is possible. This case showed an infiltrative growth pattern indicating the true neoplastic nature of this tumor.

Schlüsselwörter

Gangliozytisches Paragaglinonom - Duodenum

Key words

Gangliocytic paraganglioma - duodenum

Literatur

• 1 Anders K H, Glasgow B J, Lewin K J. Gangliocytic paraganglioma associated with duodenal adenocarcinoma. Case report with immunohistochemical evaluation.  Arch Pathol Lab Med. 1987;  111 49-52
• 2 Barbareschi M. et al . Duodenal gangliocytic paraganglioma. Report of a case and review of the literature.  Virchows Arch A Pathol Anat Histopathol. 1989;  416 81-89
• 3 Barbaryka I. Polypoid nonchromaffine paraganglioma of the duodenum. Virchows Arch 1978; 377: 181-187
• 4 Branum G D, Pappas T N, Meyers W C. The management of tumors of the ampulla of Vater by local resection.  Ann Surg. 1996;  224 621-627
• 5 Büchler M, Malfertheimer P, Baczako K, Krautzberger W, Beger H C. A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction: malignant paraganglioma?.  Digestion. 1985;  31 54-59
• 6 Burke A P, Helwig E B. Gangliocytic paraganglioma.  Am J Clin Pathol. 1989;  92 1-9
• 7 Caceres M, Mosquera L F, Shih J A, O'Leary J P. Paraganglioma of the bile duct.  South Med J. 2001;  94 515-518
• 8 Cahen D L, Fockens P, Wit L, Offerhausen G. et al . Local resection or pancreaticoduodenectomy for villous adenoma of the ampulla of Vater diagnosed before operation.  Br J Surg. 1997;  84 948-951
• 9 Dahl E, Waugh J M, Dahlin D C. Gastrointestinal ganglioneuromas. Brief reviews with report of a duodenal ganglioneuroma.  Am J Pathol. 1957;  33 953-966
• 10 Dookhan D B, Miettinen M, Finkel G, Gibas Z. Recurrent duodenal gangliocytic paraganglioma with lymph node metastases.  Histopathology. 1993;  22 399-401
• 11 Duff C A, Segesser L K, Bino M, Schmid E R. et al . Das duodenale Paragangliom.  Chirurg. 1991;  62 144-147
• 12 Evans J D, Wilson P G, Barber P C, Neoptolemos J P. Duodenal gangliocytic paraganglioma presenting as ampullary tumor.  Int J Pancreatol. 1996;  20 131-134
• 13 Gemer M, Feuchtwanger M. Ganglioneuroma of the duodenum.  Gastroenterology. 1966;  51 689-693
• 14 Gibbs E R, Walton G F, Kent R B, Laws H L. Villous tumor of the ampulla of Vater.  Am Surg. 1997;  63 467-471
• 15 Hashimoto S, Kawasaki S, Matsuzawa K, Harada H, Makuuchi M. Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis.  Am J Gastroenterol. 1992;  87 1216-1218
• 16 Hironaka M. et al . Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.  Am Surg Pathol. 2001;  25 688-693
• 17 Inai K, Kobulce T, Yonehura S, Tokooka S. Duodenal gangliocytic paraganglioma with lymph node metastases in a 17-year-old boy.  Cancer. 1989;  15 2540-2545
• 18 Kawaguchi K, Takizawa T, Koike M, Tabata I, Goseki N. Multiple paragangliomas.  Virchows Arch A Pathol Anat Histopathol. 1985;  406 373-380
• 19 Kepes J J, Zacharias D L. Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination.  Cancer. 1971;  27 61-70
• 20 Kheir S, Halpern N. Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.  Cancer. 1984;  53 2491-2496
• 21 Konok G P, Sanchez-Cassis G. Gangliocytic paraganglioma of the duodenum.  Can J Surg. 1979;  22 173-175
• 22 Korbi S, Kapanci Y, Widgren S. Malignant paraganglioma of the duodenum. Immunohistochemical and ultrastructural study of a case.  Ann Pathol. 1987;  7 47-55
• 23 Kotsis T, Voros D, Paphiti A, Frangou M, Mallas E. Duodenal gangliocytic paraganglioma as a radiological moving defect.  Dig Surg. 2000;  17 636-640
• 24 Min K W. Gangliocytic paraganglioma of the duodenum: report of a case with immunocytochemical and ultrastructural investigation.  Ultrstruct Pathol. 1997;  21 587-595
• 25 Motton G, Veraldi G F, Fracastoro G, Ricci F. et al . Vater's papilla and periampullary area adenoma: personal experience about nine cases and review of the literaur.  Hepato Gastroenterol. 1996;  43 448-455
• 26 Neoptolemos J P, Talbot I C, Shaw D C, Carr-Locke D L. Long-term survival after resection of ampullary carcinoma is associated independently with tumor grade and new staging classification that assesses local invasiveness.  Cancer. 1988;  61 1403-1407
• 27 Niedergethmann M, Richter A, Wendl K, Schmidt B. et al . Rare Indications for a Kausch-Whipple Procedure.  Eur J Surg. 2001;  167 115-11
• 28 Perrone T, Sibley R K, Rosai J. Duodenal gangliocytic paraganglioma.  An immunohistochemical and ultrastructural study and a hypothesis concerning its origin.. 1985;  9 31-41
• 29 Reed R J, Duroca P J, Harkin J C. Gangliocytic paraganglioma.  Am J Surg Pathol. 1977;  1 207-216
• 30 Scheithauer B W, Nora F E, LeChago J, Wick M R. et al . Duodenal gangliocytic paraganglioma. Clinicopathologic and immunocytochemical study of 11 cases.  Am J Clin pathol. 1986;  86 559-565
• 31 Taylor H B, Vincent J, Hyams C DR, Nielsen O F. Benign nonchromaffin paragangliomas of the duodenum.  Virchows Arch. 1996;  335 356-366
• 32 Tomic S, Warner T. Pancreatic somatostatin- secreting gangliocytic paraganglioma with lymph node metastases.  Am J Gastroenterol. 1996;  91 607-608
• 33 Witzigmann H, Möbius C h, Uhlmann D, Geißler F. et al . Behandlungskonzept von Adenomen der Papilla Vateri.  Chirurg. 2000;  71 196-201