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Paraneoplastic syndromes in ganglioneuroblastoma: contrasting symptoms of constipation and diarrhoea

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Abstract

A paraneoplastic syndrome is occasionally the first clinical symptom seen with tumours. We report on two children who initially presented with paraneoplastic syndromes due to ganglioneuroblastomas: the first with severe watery diarrhoea caused by a ganglioneuroma producing vasoactive intestinal peptide, the second with non-treatable constipation, caused by ganglioneuroma-produced anti-neuronal nuclear antibodies. Conclusion: Either severe diarrhoea or chronic constipation may represent rare paraneoplastic syndromes in ganglioneuroblastomas.

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Abbreviations

ANNA :

anti-neuronal nuclear antibodies

VIP :

vasoactive intestinal peptide

WDHA :

watery diarrhoea-hypokalaemia-achlorhydria

References

  1. Altermatt HJ, Gerber HA, Scheithauser BW (1993) Neurologic paraneoplastic syndromes. Anti-Purkinje cell antibodies and anti-neuronal nuclear antibodies as serologic markers. Dtsch med Wschr 118: 1735–1741

    Article  CAS  PubMed  Google Scholar 

  2. Briellmann RS, Sturzenegger M, Gerber HA, Schaffner T, Hess CW (1996) Autoantibody-associated sensory neuronopathy and intestinal pseudo-obstruction without detectable neoplasia. Eur Neurol 36: 369–373

    Article  CAS  PubMed  Google Scholar 

  3. Debas HT, Gittes G (1993) Somatostatin analog therapy in functioning neuroendocrine gut tumors. Digestion 54: 68–71

    Article  PubMed  Google Scholar 

  4. Dropcho EJ (1995) Autoimmune central nervous system paraneoplastic disorders: mechanisms, diagnosis, and therapeutic options. Ann Neurol 37: 203–113

    Article  Google Scholar 

  5. Kimura N, Yamamoto H, Okamoto H, Wakasa H, Nagura H (1993) Multiple-hormone gene expression in ganglioneuroblastoma with watery diarrhea, hypokalemia, and achlorhydria syndrome. Cancer 71: 2841–2846

    Article  CAS  PubMed  Google Scholar 

  6. Kulling D, Reed CE, Verne GN, Cotton PB, Tarnasky PR (1997) Intestinal pseudo-obstruction as a paraneoplastic manifestation of malignant thymoma. Am J Gastroenterol 92: 1564–1566

    CAS  PubMed  Google Scholar 

  7. Lautenbach E, Lichtenstein GR (1995) Retroperitoneal leiomyosarcoma and gastroparesis. A new association and review of tumor-associated intestinal pseudo-obstruction. Am J Gastroenterol 90: 1338–1341

    CAS  PubMed  Google Scholar 

  8. Lee H, Lennon VA, Camilleri M, Prather, CM (2001) Paraneoplastic gastrointestinal motor dysfunction: clinical and laboratory characteristics. Am J Gastroenterol 96: 373–379

    Article  CAS  PubMed  Google Scholar 

  9. Lennon VA, Sas DF, Busk MF, Scheithauer B, Malagelada JR, Camilleri M, Miller LJ (1991) Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma. Gastroenterol 100: 137–142

    Article  CAS  Google Scholar 

  10. Liang BC, Albers JW, Sima AF, Nostrant T (1994) Paraneoplastic pseudo-obstruction, mononeuropathy multiplex, and sensory neuronopathy. Muscle Nerve 17: 91–96

    Article  CAS  PubMed  Google Scholar 

  11. Plantaz D, Michon J, Valteau-Couanet D, Coze C, Chastagner P, Bergeron C, Nelken B, Martelli H, Peyroulet MC, Carpentier AF, Armari-Alla C, Pagnier A, Rubie H (2000) Opsoclonus-myoclonus syndrome associated with non-metastatic neuroblastoma. Long-term survival. Study of the French society of Pediatric Oncologists. Arch Pediatr 7: 621–628

    Article  CAS  PubMed  Google Scholar 

  12. Quak SH, Prabhakaran K, Kwok R, O'Reilly AP (1988) Vasoactive intestinal peptide secreting tumours in children: a case report with literature review. Aust Paediatr J 24: 55–58

    CAS  PubMed  Google Scholar 

  13. Said SI, Mutt V (1970) Polypeptide with broad biologic activity. Isolation from small intestine. Science 169: 1217–1218

    Article  CAS  PubMed  Google Scholar 

  14. Schobinger-Clément S, Gerber HA, Stallmach T (1999) Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. Am J Surg Pathol 23: 602–606

    Article  PubMed  Google Scholar 

  15. Schuler D, Koos R, Peter A (1977) Paraneoplastic syndrome in children. Acta Paediatr Acad Sci Hung 18: 31–40

    CAS  PubMed  Google Scholar 

  16. Smith VV, Gregson N, Foggensteiner L, Neale G, Milla PJ (1997) Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other involvement. Gastroenterology 112: 1366–1371

    Article  CAS  PubMed  Google Scholar 

  17. Tora M, Graus F, de Bolos C, Real FX (1997) Cell surface expression of paraneoplastic encephalomyelitis/sensory neuronopathy-associated Hu antigens in small-cell lung cancers and neuroblastomas. Neurology 48: 735–741

    Article  CAS  PubMed  Google Scholar 

  18. Verner JV, Morrison AB (1958) Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med 25: 374–380

    Article  CAS  PubMed  Google Scholar 

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Author notes

  1. Barbara Wildhaber

    Present address: Section of Pediatric Surgery, University of Michigan Hospitals, Mott F3970, 0245, MI 48109, Ann Arbor, USA

Authors and Affiliations

  1. Department of Surgery, University Children's Hospital, Zurich, Switzerland

    Barbara Wildhaber & Peter Sacher

  2. Department of Oncology, University Children's Hospital, Zurich, Switzerland

    Felix Niggli & Eva Bergsträsser

  3. Department of Pathology, University Hospital of Zurich, Zurich, Switzerland

    Thomas Stallmach

Authors
  1. Barbara Wildhaber
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  2. Felix Niggli
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  3. Eva Bergsträsser
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  4. Thomas Stallmach
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  5. Peter Sacher
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Corresponding author

Correspondence to Barbara Wildhaber.

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Wildhaber, B., Niggli, F., Bergsträsser, E. et al. Paraneoplastic syndromes in ganglioneuroblastoma: contrasting symptoms of constipation and diarrhoea. Eur J Pediatr 162, 511–513 (2003). https://doi.org/10.1007/s00431-003-1212-0

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  • DOI: https://doi.org/10.1007/s00431-003-1212-0

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