Skip to main content


Log in

Desmoplastic infantile astrocytoma and ganglioglioma: a series of 12 patients treated at a single institution

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript



Desmoplastic infantile astrocytomas and gangliogliomas (DIA/DIG) usually present with a large size, large cystic component, large dural implant, encasement of big vessels, clinical presentation within 18 months of life, high incidence of seizures and overall good prognosis, even if tumour surgery can be very challenging at first procedure.


We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019.


The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm3. Cystic components were predominant ranging from 0 to 295 cm3. Active hydrocephalus was pre-operatively evident in 5 cases. Eight patients (66.6%) received total or subtotal removal, three of them (25%) underwent partial removal, and one patient (8.3%) received a biopsy. One patient died within 24 h after surgery due to severe hypotension, as a consequence of significant intraoperative blood loss. Overall, seven (58.3%) patients were reoperated on the tumour after the first procedure: 4 patients were operated twice; 3 patients were operated 3 times. Two patients presented remote localizations and underwent chemotherapy. At last follow-up, 7 patients were tumour-free, 2 are alive with stable disease, and 2 are alive with progressive disease (leptomeningeal seeding).


Desmoplastic infantile tumours are rare giant neonatal tumours. Total removal is the goal of treatment, but prognosis remains good even if total removal is not achieved. In case of tumour progression or epilepsy from residual tumour, reoperation is the first option, with chemotherapy reserved to unresectable or disseminated cases with mixed results, while, to date, radiotherapy still plays no role.

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

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others


  1. Abuharbid G, Esmaeilzadeh M, Hartmann C, Hermann EJ, Krauss JK (2015) Desmoplastic infantile astrocytoma with multiple intracranial and intraspinal localizations at presentation. Childs Nerv Syst 31:959–964.

    Article  PubMed  Google Scholar 

  2. Bächli H, Avoledo P, Gratzl O, Tolnay M (2003) Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst 19:359–366.

    Article  PubMed  Google Scholar 

  3. Beppu T, Sato Y, Uesugi N, Kuzu Y, Ogasawara K, Ogawa A (2008) Desmoplastic infantile astrocytoma and characteristics of the accompanying cyst. Case report J Neurosurg Pediatr 1:148–151.

    Article  PubMed  Google Scholar 

  4. Bhardwaj M, Sharma A, Pal HK (2006) Desmoplastic infantile ganglioglioma with calcification. Neuropathology 26:318–322.

    Article  PubMed  Google Scholar 

  5. Blessing MM, Blackburn PR, Balcom JR, Krishnan C, Harrod VL, Zimmermann MT, Barr Fritcher EG, Zysk CD, Jackson RA, Nair AA, Jenkins RB, Halling KC, Kipp BR, Ida CM (2018) Novel BRAF alteration in desmoplastic infantile ganglioglioma with response to targeted therapy. Acta Neuropathol Commun 6:118.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Blessing MM, Blackburn PR, Krishnan C, Harrod VL, Barr Fritcher EG, Zysk CD, Jackson RA, Milosevic D, Nair AA, Davila JI, Balcom JR, Jenkins RB, Halling KC, Kipp BR, Nageswara Rao AA, Laack NN, Daniels DJ, Macon WR, Ida CM (2019) Desmoplastic infantile ganglioglioma: a MAPK pathway-driven and microglia/macrophage-rich neuroepithelial tumor. J Neuropathol Exp Neurol 78:1011–1021.

    Article  CAS  PubMed  Google Scholar 

  7. Blount J (2006) Desmoplastic infantile gangliogliomas in: Neuro-oncology of CNS tumors, Springer-Verlag. Tonn, JC; Westphal, JT; Grossman SA, Berlin Heidelberg

  8. Chacko G, Chandi SM, Chandy MJ (1995) Desmoplastic low grade astrocytoma: a case report and review of literature. Clin Neurol Neurosurg 97:32–35.

    Article  CAS  PubMed  Google Scholar 

  9. Darwish B, Arbuckle S, Kellie S, Besser M, Chaseling R (2007) Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci 14:498–501.

    Article  CAS  PubMed  Google Scholar 

  10. Dudley RWR, Torok MR, Gallegos DR, Mulcahy-Levy JM, Hoffman LM, Liu AK, Handler MH, Hankinson TC (2015) Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. Neurosurgery 76:313–319; discussion 319; quiz 319–320.

    Article  PubMed  Google Scholar 

  11. Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A (2010) Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev 34:151–158.

    Article  PubMed  Google Scholar 

  12. Gessi M, Zur Mühlen A, Hammes J, Waha A, Denkhaus D, Pietsch T (2013) Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. J Neuropathol Exp Neurol 72:807–815.

    Article  CAS  PubMed  Google Scholar 

  13. Greer A, Foreman NK, Donson A, Davies KD, Kleinschmidt-DeMasters BK (2017) Desmoplastic infantile astrocytoma/ganglioglioma with rare BRAF V600D mutation. Pediatr Blood Cancer 64.

  14. Hummel TR, Miles L, Mangano FT, Jones BV, Geller JI (2012) Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. J Pediatr Hematol Oncol 34:e232–e236.

    Article  PubMed  Google Scholar 

  15. Izban KF, Thomas C (1998) Desmoplastic infantile ganglioglioma. Pediatric Pathology & Molecular Medicine 18:157–172.

    Article  Google Scholar 

  16. Jones DTW, Hutter B, Jäger N, Korshunov A, Kool M, Warnatz H-J, Zichner T, Lambert SR, Ryzhova M, Quang DAK, Fontebasso AM, Stütz AM, Hutter S, Zuckermann M, Sturm D, Gronych J, Lasitschka B, Schmidt S, Seker-Cin H, Witt H, Sultan M, Ralser M, Northcott PA, Hovestadt V, Bender S, Pfaff E, Stark S, Faury D, Schwartzentruber J, Majewski J, Weber UD, Zapatka M, Raeder B, Schlesner M, Worth CL, Bartholomae CC, von Kalle C, Imbusch CD, Radomski S, Lawerenz C, van Sluis P, Koster J, Volckmann R, Versteeg R, Lehrach H, Monoranu C, Winkler B, Unterberg A, Herold-Mende C, Milde T, Kulozik AE, Ebinger M, Schuhmann MU, Cho Y-J, Pomeroy SL, von Deimling A, Witt O, Taylor MD, Wolf S, Karajannis MA, Eberhart CG, Scheurlen W, Hasselblatt M, Ligon KL, Kieran MW, Korbel JO, Yaspo M-L, Brors B, Felsberg J, Reifenberger G, Collins VP, Jabado N, Eils R, Lichter P, Pfister SM, International Cancer Genome Consortium PedBrain Tumor Project (2013) Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. Nat Genet 45:927–932.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Kim J-H, Kim I-O, Kim WS, Kim KH, Park CM, Yeon K-M (2003) MR findings of desmoplastic cerebral astrocytoma of infancy. Acta Radiol 44:688–690.

    Article  PubMed  Google Scholar 

  18. Koelsche C, Sahm F, Paulus W, Mittelbronn M, Giangaspero F, Antonelli M, Meyer J, Lasitschka F, von Deimling A, Reuss D (2014) BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol 40:337–344.

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  20. Louis DN, von Deimling A, Dickersin GR, Dooling EC, Seizinger BR (1992) Desmoplastic cerebral astrocytomas of infancy: a histopathologic, immunohistochemical, ultrastructural, and molecular genetic study. Hum Pathol 23:1402–1409.

    Article  CAS  PubMed  Google Scholar 

  21. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131:803–820.

    Article  PubMed  Google Scholar 

  22. Lundar T, Due-Tønnessen BJ, Fric R, Egge A, Krossnes B, Due-Tønnessen P, Stensvold E, Brandal P (2018) Neurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients. Acta Neurochir 160:1207–1214.

    Article  PubMed  Google Scholar 

  23. 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 16:8–14.

    Article  CAS  PubMed  Google Scholar 

  24. 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 45:986–990.

    Article  PubMed  Google Scholar 

  25. Nikas I, Anagnostara A, Theophanopoulou M, Stefanaki K, Michail A, Hadjigeorgi C (2004) Desmoplastic infantile ganglioglioma: MRI and histological findings case report. Neuroradiology 46:1039–1043.

    Article  CAS  PubMed  Google Scholar 

  26. Osborn AG, Salzman KL, Jhaveri MD, Barkovich AJ (2015) Desmoplastic infantile astrocytoma and ganglioglioma in: diagnostic imaging: Brain E-Book. Elsevier Health Sciences

    Google Scholar 

  27. 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 Neuro-Oncol 76:271–275.

    Article  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  29. Rumana CS, Valadka AB (1998) Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas. Neurosurgery 42:1038–1043.

    Article  CAS  PubMed  Google Scholar 

  30. Rumana CS, Valadka AB, Contant CF (1999) Prognostic factors in supratentorial ganglioglioma. Acta Neurochir 141:63–68; discussion 68-69.

    Article  CAS  PubMed  Google Scholar 

  31. Santhosh K, Kesavadas C, Radhakrishnan VV, Abraham M, Gupta AK (2008) Multifocal desmoplastic noninfantile astrocytoma. J Neuroradiol 35:286–291.

    Article  CAS  PubMed  Google Scholar 

  32. Sugiyama K, Arita K, Shima T, Nakaoka M, Matsuoka T, Taniguchi E, Okamura T, Yamasaki H, Kajiwara Y, Kurisu K (2002) Good clinical course in infants with desmoplastic cerebral neuroepithelial tumor treated by surgery alone. J Neuro-Oncol 59:63–69.

    Article  Google Scholar 

  33. 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; discussion 983-984.

    Article  PubMed  Google Scholar 

  34. 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 

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

    Article  CAS  PubMed  Google Scholar 

  36. Trehan G, Bruge H, Vinchon M, Khalil C, Ruchoux MM, Dhellemmes P, Ares GS (2004) MR imaging in the diagnosis of desmoplastic infantile tumor: retrospective study of six cases. AJNR Am J Neuroradiol 25:1028–1033

    PubMed  PubMed Central  Google Scholar 

  37. Ulu MO, Tanriöver N, Biçeroğlu H, Oz B, Canbaz B (2008) A case report: a noninfantile desmoplastic astrocytoma. Turk Neurosurg 18:42–46

    PubMed  Google Scholar 

  38. Uro-Coste E, Ssi-Yan-Kai G, Guilbeau-Frugier C, Boetto S, Bertozzi AI, Sevely A, Lolmede K, Delisle MB (2010) Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation. J Neuro-Oncol 98:143–149.

    Article  CAS  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  40. VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins VP, Park TS (1987) Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:58–71.

    Article  CAS  PubMed  Google Scholar 

  41. Wang AC, Jones DTW, Abecassis IJ, Cole BL, Leary SES, Lockwood CM, Chavez L, Capper D, Korshunov A, Fallah A, Wang S, Ene C, Olson JM, Geyer JR, Holland EC, Lee A, Ellenbogen RG, Ojemann JG (2018) Desmoplastic infantile ganglioglioma/astrocytoma (DIG/DIA) are distinct entities with frequent BRAFV600 mutations. Mol Cancer Res 16:1491–1498.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Pietro Spennato.

Ethics declarations

Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee (Comitato Etico Cardarelli-Santobono) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

For this retrospective study, formal consent is not required.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Imperato, A., Spennato, P., Mazio, F. et al. Desmoplastic infantile astrocytoma and ganglioglioma: a series of 12 patients treated at a single institution. Childs Nerv Syst 37, 2187–2195 (2021).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: