Skip to main content
SpringerLink
Log in

Duodenal gangliocytic paraganglioma

Report of a case and review of the literature

  • Case Report
  • Published:
Virchows Archiv A Aims and scope Submit manuscript

Summary

A case of gangliocytic paraganglioma (GP) of the ampulla of Vater is reported and the literature reviewed, with special attention to immunohistochemical studies. The present case, which occurred in a 56-year-old woman, shows the typical histological admixture of epithelioid, ganglion and spindle cells. Immunohistochemistry reveals strong reactivity for synaptophysin, Leu-7, somatostatin, S-100 protein and vimentin. A few ganglion cells are reactive for neurofilaments. Chromogranin A, myelin basic protein, desmin and cytokeratin are absent.

Immunohistochemical data from literature regarding the cytoskeletal composition of GPs are not unequivocal: cytokeratin and neurofilament positivity is reported by some authors and denied by others. More uniformity is reported concerning the peptides produced by GPs: somatostatin and pancreatic polypeptide are the most frequently found antigens, followed by serotonin. General neuroendocrine markers like neuron specific enolase and protein gene product 9.5 are always positive, whereas chromogranins are rarely found. S-100 protein is always positive in the spindle cell component.

Our data are in keeping with those previously reported and add the diffuse positivity for the Leu-7 antigen and the positivity of ganglion cells for synaptophysin.

The nature of the tumour is still a matter of debate and it is difficult to agree with either of the proposed hypotheses —hamartoma/choristoma versus true neoplasm. However the recent reports of the occasional malignant evolution of GPs may support their true neoplastic nature.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Adam A, Hochholzer L (1981) Ganglioneuroblastoma of the posterior mediastinum: A clinicopathologic review of 80 cases. Cancer 47:373–381

    Google Scholar 

  • Anders KH, Glasgow BJ, Lewin KJ (1987) Gangliocytic paraganglioma associated with duodenal adenocarcinoma. Case report with immunohistochemical evaluation. Arch Pathol Lab Med 111:49–52

    Google Scholar 

  • Barbareschi M, Floresta AM, Mariani C, Allegranza A, Mosca L (1984) The nature of perineuronal cells in human gastrointestinal tract. Acta Neurol 6:326–327

    Google Scholar 

  • Barbareschi M, Mauri MF, Muscara' M, Mauri FA, Lo Re V (1987) S-100 protein in human lung neuroendocrine neoplasms. Immunohistochemical study of 14 cases and review of the literature. Histol Histopathol 2:185–192

    Google Scholar 

  • Barbareschi M, Frigo B, Ferrero S, Mariscotti C, Mosca L (1988) Bronchial carcinoid with S-100 positive sustentacular cells. Tumori 74:699–705

    Google Scholar 

  • Buchler M, Malfertheiner P, Baczako K, Krautzberger W, Beger HG (1985) A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction: malignant paraganglioma? Digestion 31:54–59

    Google Scholar 

  • Cremer H, Addicks K (1985) Polypoid gangliocytic paraganglioma of the duodenum. Pathologe 6:211–216

    Google Scholar 

  • Christensen WN, Strong EW, Bains MS, Woodruff JM (1988) Neuroendocrine differentiation in the glandular peripheral nerve sheath tumor. Am J Surg Pathol 12:417–426

    Google Scholar 

  • Dante S, Viale G, Dalla Palma P (1987) Gangliocytic paraganglioma of the duodenum: case report. Tumori 73:425–429

    Google Scholar 

  • El-Salhy M, Lundqvist M, Wilander E (1986) Bronchial carcinoids and phaeochromocytomas. A comparative study with special emphasis on S-100 protein, serotonin and neuron-specific enolase (NSE) immunoreactivity. Acta Path Microbiol Immunol Scand Sect A 94:229–235

    Google Scholar 

  • Grouls V, Pickartz H, Reis HE (1987) Peduncolated polypoid gangliocytic paraganglioma of the afferent jejunal loop of a Billroth II stomach. Leber Magen Darm 17:327–333

    Google Scholar 

  • Guarda LA, Ordonez NG, Del Junco GW, Luna MA (1983) Gangliocytic paraganglioma of the duodenum: An immunocytochemical study. Am J Gastroenterol 78:794–798

    Google Scholar 

  • Guarda LA, Ordonez NG (1987) Gangliocytic paraganglioma of the duodenum: report of cytologic, histologic,immuno-histochemical and ultrastructural features of a case. Diagn Cytopathol 3:314–319

    Google Scholar 

  • Hamid QA, Bishop AE, Rode J, Dhillon AP, Rosemberg BF, Reed RJ, Sibley RK, Polak JM (1986) Duodenal gangliocytic paragangliomas: A study of 10 cases with immunocytochemical neuroendocrine markers. Hum Pathol 17:1151–1157

    Google Scholar 

  • Hsu SM, Raine L, Fanger H (1981) Use of avidin-biotin-peroxi-dase complex (ABC) in immunoperoxidase technique. A comparison between ABC and unlabeled antibody (PAP) procedures. J Histochem Cytochem 29:577–580

    Google Scholar 

  • Ironside JW, Royds JA, Taylor CB, Timperley WR (1985) Paraganglioma of the cauda equina: A histological ultrastructural and immunocytochemical study of two cases with a review of the literature. J Pathol 145:195–201

    Google Scholar 

  • Johnson TL, Zarbo RJ, Lloyd RV, Crissman JD (1988) Paragangliomas of the head and neck: Immunohistochemical neuroendocrine and intermediate filament typing. Modern Pathol 1:216–223

    Google Scholar 

  • Kawaguchi K, Takizawa T, Koike M, Tabata I, Goseki N (1985) Multiple paraganglioneuromas. Virchows Arch [A] 406:373–380

    Google Scholar 

  • Kheir SM, Halpern NB (1984) Paraganglioma of the duodenum in association with congenital neurofibromatosis. Cancer 53:2491–2496

    Google Scholar 

  • Korbi S, Kapanci Y, Widgren S (1987) Le paragangliome malin du duodénum. Etude immunohistochimique et ultrastruturale d'un cas. Ann Pathol 7:47–55

    Google Scholar 

  • Lauzon A, Cadotte M (1972) Paragangliome gangliocytaire du duodénum. Union Med Can 101:1584–1586

    Google Scholar 

  • Lloyd RV, Cano M, Rosa P, Hille A, Huttner WB (1988) Distribution of chromogranin A and secretogranin I (chromogranin B) in neuroendocrine cells and tumors. Am J Pathol 130:296–304

    Google Scholar 

  • Lukash WM, Hyams VJ, Nielsen OF (1966) Neurogenic neo-plasms of the small bowel: Benign nonchromaffin paraganglioma of the duodenum: report of a case. Am J Dig Dis 11:575–579

    Google Scholar 

  • Martin JME, Maung RT (1987) Differential immunohisto-chemical reactions of carcinoid tumors. Hum Pathol 18:941–945

    Google Scholar 

  • Masson P (1930) The significance of the muscolar “stroma” of argentaffin tumors (carcinoids). Am J Pathol 6:499–514

    Google Scholar 

  • Mayol Belda MJ, Sanchez Ramos MA, Van der Hofstad Alberola C, Vasquez Echarri J (1988) Duodenal gangliocytic paraganglioma. Rev Esp Enferm Apar Dig 73:214–216

    Google Scholar 

  • Michels S, Swanson PE, Robb JA, Wick MR (1987) Leu-7 in small cell neoplasms. An immunohistochemical study with ultrastructural correlations. Cancer 60:2958–2964

    Google Scholar 

  • Mosca L, Barbareschi M, Mauri MF, Munguia-Barrera J, Frigo B, Muscarà M, Valentini L, Mariscotti C(1988) Neuroendocrine lung structures and tumours: immunohisto-chemical study by specific markers. Histol Histopathol 3:367–376

    Google Scholar 

  • Ortiz-Rivas LA, Longo J, Juanco C, Silvan M, Guerra I (1986) Gangliocytic paraganglioma of the duodenum. Presentation of a case and review of the literature. Rev Esp Enferm Apar Dig 69:363–365

    Google Scholar 

  • Perrone T, Sibley RK, Rosai J (1985) Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. Am J Surg Pathol 9:31–41

    Google Scholar 

  • Perrone T (1986) Duodenal gangliocytic paraganglioma and carcinoid. Am J Surg Pathol 10:147–150

    Google Scholar 

  • Reed RJ, Daroca PJ, Harkin JC (1977) Gangliocytic paraganglioma. Am J Surg Pathol 1:207–216

    Google Scholar 

  • Riddell K, Tippens BS, Gown AM (1987) PHE-5 a new monoclonal antibody to a unique neuroendocrine granule protein. Lab Invest 56:64A

    Google Scholar 

  • Sando WC, Mills SE, Rodgers BM (1986) Duodenal gangliocytic paraganglioma occurring in adolescence. J Pediatr Gastroenterol Nutr 5:659–664

    Google Scholar 

  • Savio OS, Ganzales BN, Cortés FG, Barry HG, Rodriguez MMA, Rodriguez NA (1974) Paraganglioma no cromafinico del yeyuno. Rev Cubana Cir 13:497–505

    Google Scholar 

  • Scheithauer BW, Nora FE, LeChago J, Wick MR, Crawford BG, Weiland LH, Carney JA (1986) Duodenal gangliocytic paraganglioma. Clinicopathologic and immunohistochemical Study of 11 cases. Am J Clin Pathol 86:559–565

    Google Scholar 

  • Schroder HD, Johannsen L (1986) Demonstration of S-100 protein in sustentacular cells of phaechromocytomas and paragangliomas. Histopathology 10:1023–1033

    Google Scholar 

  • Seshi B, True L, Carter D, Rosai J (1988) Immunohistochemical characterization of a set of monoclonal antibodies to human neuron-specific enolase. Am J Pathol 131:258–269

    Google Scholar 

  • Sonneland PR, Scheithauer BW, LeChago J, Crawford BG, Onofrio BM (1986) Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure. Cancer 58:1720–1735

    Google Scholar 

  • Stephens M, Williams GT, Jasani B, Williams ED (1987) Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease-a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid. Histopathology 11:1331–1340

    Google Scholar 

  • Sternberger LA (1979) Immunocytochemistry, 2nd ed. John Wiley & Sons, New York

    Google Scholar 

  • Tsutsumi Y (1984) Leu7 immunoreactivity as histochemical marker for paraffin-embedded neuroendocrine tumors. Acta Histochem Cytochem 17:15–21

    Google Scholar 

  • Tischler AS, Mobtaker H, Mann K, Nunnemacher G, Jason WJ, Dayal Y, De Lellis RA, Adelman L, Wolfe HJ (1986) Anti-lymphocyte monoclonal antibody HNK-1 (Leu7) recognizes a constituent of neuroendocrine granule matrix. Lab Invest 54:64A

    Google Scholar 

  • Wilander E, Lundqvist M, Movin T (1985) S-100 protein in carcinoid tumors of appendix. Acta Neuropathol 66:306–310

    Google Scholar 

  • Wittaker MH, Sandusky GE (1988) Synaptophysin, Chromogranin A, PHE-5 and Chromogranin (LK2H10). Immunoreactivity in abdominal carcinoids. Modern Pathol 1:102A

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. 3rd Chair of Morbid Anatomy and Histopathology, School of Medicine, University of Milan, Milano, Italy

    M. Barbareschi, B. Frigo & M. Falleni

  2. 5th Chair of Morbid Anatomy and Histopathology, School of Medicine, University of Milan, Milano, Italy

    S. Cristina

  3. Department of Morbid Anatomy and Histopathology, “S. Chiara” Hospital, Trento, Italy

    D. Aldovini & E. Leonardi

Authors
  1. M. Barbareschi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. B. Frigo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. D. Aldovini
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. E. Leonardi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Cristina
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. Falleni
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Barbareschi, M., Frigo, B., Aldovini, D. et al. Duodenal gangliocytic paraganglioma. Vichows Archiv A Pathol Anat 416, 81–89 (1989). https://doi.org/10.1007/BF01606473

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01606473

Key words

Search

Navigation