Skip to main content
SpringerLink
Log in

Disseminated pilocytic astrocytoma involving brain stem and diencephalon: a history of atypical eating disorder and diagnostic delay

  • Clinical--patient studies
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Summary

The association of weight loss and pediatric brain tumors that affect the diencephalon or brain stem with weight loss is a recognized, but not fully understood phenomenon. Tumors located in the hypothalamic region may induce the diencephalic syndrome (DS), which is characterized by profound emaciation with almost complete loss of subcutaneous fatty tissue. Tumors that compress or infiltrate the brain stem rarely cause both psychological disturbance and emaciation. The clinical presentation may be different, depending on the location of the lesion and age of the patient.

In this report we present an unusual case of severe emaciation in a 49/12-year-old girl with a juvenile pilocytic astrocytoma of the hypothalamic region and brain stem with neuroaxis dissemination. This case illustrates the importance of considering intracranial mass-lesions in the differential diagnosis of weight loss, psychological disturbance and atypical eating disorder. We discuss the importance of tumor multifocality and the role of patient age in the clinical presentation with reference to the literature.

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.

Similar content being viewed by others

References

  1. Lehman RA, Krishnamurthy S, Berlin CM Weight and height deficits in children with brain stem tumorsClin Pediatr 2002;41:315–321

    Article  Google Scholar 

  2. Chipkevitch E Brain tumors and anorexia nervosa syndrome Brain Dev 1994;16:175–179

    Article  PubMed  CAS  Google Scholar 

  3. DeVile CJ, Sufraz R, Lask BD, Stanhope R Lesson of the Week: Occult intracranial tumors masquerading as early onset anorexia nervosa BMJ 1995;311:1359–1360

    PubMed  CAS  Google Scholar 

  4. Ertem D, Acar Y, Alper G, Kotiloglu E, Pehlivanoglu E An uncommon and often overlooked cause of failure to thrive: diencephalic syndrome J Pediatr Gastroenterol Nutr 2000;30:453–457

    Article  PubMed  CAS  Google Scholar 

  5. Maroon JC, Albright L “Failure to thrive” due to pontine glioma Arch Neurol. 1977;34:295–297

    PubMed  CAS  Google Scholar 

  6. Gnekow AK, Kortmann RD, Pietsch T, Emser A Low grade chiasmatic-hypothalamic glioma-carboplatin and vincristin chemotherapy effectively defers radiotherapy within a comprehensive treatment strategy – report from the multicenter treatment study for children and adolescents with a low grade glioma – HIT-LGG 1996 – of the Society of Pediatric Oncology and Hematology (GPOH). Klin Padiatr. 2004;216:331–342

    Article  PubMed  CAS  Google Scholar 

  7. Russel A A diencephalic syndrome of emaciation in infancy and childhood Arch Dis Child 1951;26:274–249

    Google Scholar 

  8. Andler W, Stolecke H, Sirang H Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy Helv Paediatr Acta 1978;33:393–400

    PubMed  CAS  Google Scholar 

  9. Vlachopapadopoulou E, Tracey KJ, Capella M, Gilker C, Matthews DE Increased energy expenditure in a patient with diencephalic syndrome J Pediatr 1993;122:922–924

    Article  PubMed  CAS  Google Scholar 

  10. Gallet M, Leke L, Kremp O, Kochert F, Rosat P, Laurent C, Deramont H, Risbourg B A case report of Russel’s diencephalic cachexia Ann Pediatr 1992;39:189–193

    CAS  Google Scholar 

  11. Ward A, Tiller J, Treasure J, Russell G Eating disorders: psyche or soma? Int J Eat Disord 2000;27:279–287

    Article  PubMed  CAS  Google Scholar 

  12. Neary NM, Goldstone AP, Bloom SR Appetite regulation: from the gut to the hypothalamus Clin Endocrinol 2004;60:153–160

    Article  Google Scholar 

  13. Janisch W, Schreiber D, Martin H, Gerlach H Diencephalic pilocytic astrocytoma with clinical onset in infancy. Biological behavior and pathomorphological findings in 11 children Zentralbl Allg Pathol1985;130:31–43

    PubMed  CAS  Google Scholar 

  14. Gropman AL, Packer RJ, Nicholson HS, Vezina LG, Jakacki R, Geyer R, Olson JM, Phillips P, Needle M, Broxson EH, Reaman G, Finlay J Treatment of diencephalic syndrome with chemotherapy Cancer 1998;83:166–172

    Article  PubMed  CAS  Google Scholar 

  15. Chipkevitch E, Fernandes AC Hypothalamic tumor associated with atypical forms of anorexia nervosa and diencephalic syndrome Arq Neuropsiquiatr 1993;51:270–274

    PubMed  CAS  Google Scholar 

  16. Perilongo G, Carollo C, Salviati L. Diencephalic syndrome and disseminated juvenile pilocytic astrocytoma of the hypothalamo-optic chiasm region Cancer 1997;80:142–146

    Article  PubMed  CAS  Google Scholar 

  17. Kocks W, Kalff R, Reinhardt V, Grote W, Hilke J Spinal metastasis of pilocytic astrocytoma of the chiasma opticum. Childs Nerv Syst1989;5:118–120

    Article  PubMed  CAS  Google Scholar 

  18. Kageji T, Nagahiro S, Horiguchi H, Watanabe T, Suzuya H, Okamoto Y, Kuroda Y Successful high-dose chemotherapy for widespread neuroaxis dissemination of an optico-hypothalamic juvenile pilocytic astrocytoma in an infant: a case report J Neurooncol2003;62:281–287

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors would like to thank Thomas Olson, Pediatric Oncology, Emory University, Atlanta, USA for critically proofreading our manuscript.

Author information

Authors and Affiliations

  1. Department of General Pediatrics, Heinrich-Heine-University, Düsseldorf, Germany

    Felix Distelmaier, Ertan Mayatepek & Thorsten Rosenbaum

  2. Department of Pediatric Oncology, Hematology and Immunology, Heinrich-Heine-University, Düsseldorf, Germany

    Gisela Janssen & Ulrich Göbel

  3. Department of Diagnostic Radiology, Heinrich-Heine-University, Düsseldorf, Germany

    Jörg Schaper

  4. Department of General Pediatrics, University Children’s Hospital, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, D-40225, Düsseldorf, Germany

    Felix Distelmaier

Authors
  1. Felix Distelmaier
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gisela Janssen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ertan Mayatepek
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jörg Schaper
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ulrich Göbel
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Thorsten Rosenbaum
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Felix Distelmaier.

Additional information

Felix Distelmaier and Gisela Janssen contributed equally to this paper.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Distelmaier, F., Janssen, G., Mayatepek, E. et al. Disseminated pilocytic astrocytoma involving brain stem and diencephalon: a history of atypical eating disorder and diagnostic delay. J Neurooncol 79, 197–201 (2006). https://doi.org/10.1007/s11060-006-9125-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-006-9125-1

Keywords

Search

Navigation