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Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation

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Abstract

Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are rare intracranial tumors that mostly occur in the first 2 years of life and involve superficial cerebral cortex. Despite the large size of these lesions and some worrisome histological and radiological features, prognosis is generally favorable after gross total resection. We report an original observation of a desmoplastic infantile astrocytoma in a 5-year-old boy with multiple localizations on initial presentation, including the unusual subtentorial region. Magnetic resonance imaging showed a temporal tumor with prepontine and interpeduncular extension, and two other distinct localizations in cisterna magna and left cerebellar hemisphere. Leptomeningeal enhancements were present around the basal cistern. The surgical samples, corresponding exclusively to subtentorial lesions, were devoid of anaplastic features; the temporal lesion was untouched because of the interpeduncular extension. Adjuvant chemotherapy was applied, with shrinkage of lesions. DIA and DIG are more generally unifocal at initial presentation. When the tumor is large, multilobular involvement is common, but multiple location of DIG is, on the contrary, very rare. Previously, only five cases of DIG/DIA located in two or more separate locations have been published. We report the sixth, and first noninfantile, case of DIA/DIG with multifocal initial presentation.

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References

  1. Rout P, Santosh V, Mahadevan A, Kolluri VR, Yasaha TC, Shankar SK (2002) Desmoplastic infantile ganglioglioma: clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 18:463–467

    Article  PubMed  Google Scholar 

  2. Ganesan K, Desai S, Udwadia-Hegde A (2006) Non-infantile variant of desmoplastic ganglioglioma: a report of 2 cases. Pediatr Radiol 36:541–545

    Article  PubMed  Google Scholar 

  3. Pommepuy I, Delage-Corre M, Moreau JJ, Labrousse F (2006) A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature. J Neurooncol 76:271–275

    Article  CAS  PubMed  Google Scholar 

  4. Kuchelmeister K, Bergmann M, Von Wild K et al (1993) Desmoplastic ganglioglioma: report of two non infantile case. Acta Neuropathol (Berl) 85:199–204

    Article  CAS  Google Scholar 

  5. Per H, Kontaş O, Kumandaş S, Kurtsoy A (2009) A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. Neurosurg Rev 32:369–374

    Article  PubMed  Google Scholar 

  6. Brat DJ, Vandenberg SR, Figarella-Branger D, Taratuto AL (2008) Desmoplastic infantile astrocytoma and ganglioglioma. World Health Organization Classification of Tumours. Pathology and genetics of tumours of the nervous system. IARC, Lyon, pp 96–98

  7. Burger PC, Scheithauer BW (2007) Tumors of the central nervous system. AFIP atlas of tumor pathology; Fourth series, fascicle 7, pp 218–222

  8. Komori T, Scheithauer BW, Parisi JE et al (2001) Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow up. Mod Pathol 14:720–726

    Article  CAS  PubMed  Google Scholar 

  9. Vandenberg SR, May EE, Rubinstein LJ et al (1987) Desmoplastic supratentorial neuroepithelial tumours of infancy with divergent differentiation potential (desmoplastic ganglioglioma). Report on 11 cases of a distinctive embryonal tumour with favourable prognosis. J Neurosurg 66:58–71

    Article  CAS  PubMed  Google Scholar 

  10. VandenBerg SR (1993) Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3:275–281

    Article  CAS  PubMed  Google Scholar 

  11. Setty SN, Miller DC, Camras L, Charbel F, Schmidt ML (1997) Desmoplastic infantile astrocytoma with metastases at presentation. Mod Pathol 10(9):945–951

    CAS  PubMed  Google Scholar 

  12. Taratuto AL, Monges J, Lylyk P et al (1984) Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 54:2505–2512

    Article  CAS  PubMed  Google Scholar 

  13. De Munnynck K, Van Gool S, Van Calenbergh F et al (2002) Desmoplastic infantile ganglioglioma: a potentially malignant tumour? Am J Surg Pathol 26:1515–1522

    Article  PubMed  Google Scholar 

  14. Milanaccio C, Nozza P, Ravegnani M, Rossi A, Raso A, Gambini C, Garrè ML, Pietsch T (2005) Cervico-medullary desmoplastic infantile ganglioglioma: an unusual case with diffuse leptomeningeal dissemination at diagnosis. Pediatr Blood Cancer 45(7):986–990

    Article  PubMed  Google Scholar 

  15. Tseng JH, Tseng MY, Kuo MF et al (2002) Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma. Pediatr Neurosurg 36:29–32

    Article  PubMed  Google Scholar 

  16. Lönnrot K, Terho M, Kähärä V, Haapasalo H, Helén P (2007) Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol 68(3):304–308

    Article  PubMed  Google Scholar 

  17. Duffner PK, Burger PC, Cohen ME et al (1994) Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 34:583–589

    Article  CAS  PubMed  Google Scholar 

  18. Tenreiro-Picon OR, Kamath SV, Knorr JR et al (1995) Desmoplastic infantile ganglioglioma: CT and MRI features. Pediatr Radiol 25:540–543

    Article  CAS  PubMed  Google Scholar 

  19. Taranath A, Lam A, Wong CK (2005) Desmoplastic infantile ganglioglioma: a questionably benign tumour. Australas Radiol 49(5):433–437

    Article  CAS  PubMed  Google Scholar 

  20. Khaddage A, Chambonniere ML, Morrison AL et al (2004) Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation. Ann Diagn Pathol 8:280–283

    Article  PubMed  Google Scholar 

  21. Mayes DA, Hu Y, Teng Y, Siegel E, Wu X, Panda K, Tan F, Yung WK, Zhou YH (2006) PAX6 suppresses the invasiveness of glioblastoma cells and the expression of the matrix metalloproteinase-2 gene. Cancer Res 66:9809–9817

    Article  CAS  PubMed  Google Scholar 

  22. Snuderl M, Chi SN, De Santis SM, Stemmer-Rachamimov AO, Betensky RA, De Girolami U, Kieran MW (2008) Prognostic value of tumor microinvasion and metalloproteinases expression in intracranial pediatric ependymomas. J Neuropathol Exp Neurol 67:911–920

    Article  PubMed  Google Scholar 

  23. Kucheilmeister K, Schonmeyr R, Albani M, Schachenmayr W (1998) Anaplastic desmoplastic infantile ganglioglioma. Clin Neuropathol 17:269–270

    Google Scholar 

  24. Tamburrini G, Colosimo C, Giangaspero F, Riccardi R, Di Rocco C (2003) Desmoplastic infantile ganglioglioma. Childs Nerv Syst 19:292–297

    Article  CAS  PubMed  Google Scholar 

  25. Mallucci C, Lellouch-Tubiana A, Salazar C, Cinalli G, Renier D, Sainte-Rose C, Pierre-Kahn A, Zerah M (2000) The management of desmoplastic neuroepithelial tumours in childhood. Childs Nerv Syst 16(1):8–14

    Article  CAS  PubMed  Google Scholar 

  26. Takeshima H, Kawahara Y, Hirano H, Obara S, Niiro M, Kuratsu J (2003) Postoperative regression of desmoplastic infantile gangliogliomas: report of two cases. Neurosurgery 53:979–983

    Article  PubMed  Google Scholar 

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Acknowledgments

We thank Alexandra A. Lao for proofreading the manuscript.

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Authors and Affiliations

  1. Department of Pathology, Rangueil University Hospital, Toulouse, France

    E. Uro-Coste, G. Ssi-Yan-Kai, C. Guilbeau-Frugier & M. B. Delisle

  2. INSERM U858, Toulouse, France

    E. Uro-Coste, C. Guilbeau-Frugier, K. Lolmede & M. B. Delisle

  3. Department of Neurosurgery, Purpan University Hospital, Toulouse, France

    S. Boetto

  4. Department of Haematology-Oncology, Children University Hospital, Toulouse, France

    A. I. Bertozzi

  5. Department of Radiology, Purpan University Hospital, Toulouse, France

    A. Sevely

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  1. E. Uro-Coste
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  2. G. Ssi-Yan-Kai
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  3. C. Guilbeau-Frugier
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  8. M. B. Delisle
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Correspondence to E. Uro-Coste.

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Uro-Coste, E., Ssi-Yan-Kai, G., Guilbeau-Frugier, C. et al. Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation. J Neurooncol 98, 143–149 (2010). https://doi.org/10.1007/s11060-009-0075-2

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  • DOI: https://doi.org/10.1007/s11060-009-0075-2

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