Desmoplastic infantile ganglioglioma
Salma Kamoun, Heifa Azouz, Marwa Zemmali, Slim Haouet, Nidhameddine Kchir
The Pan African Medical Journal. 2019;32:113. doi:10.11604/pamj.2019.32.113.12669

Innovations in Measles Elimination Innovations in Measles Elimination
"Better health through knowledge sharing and information dissemination "

Case report

Desmoplastic infantile ganglioglioma

Cite this: The Pan African Medical Journal. 2019;32:113. doi:10.11604/pamj.2019.32.113.12669

Received: 06/05/2017 - Accepted: 02/02/2019 - Published: 11/03/2019

Key words: Tumor, central nervous system, infantile desmoplastic ganglioglioma

© Salma Kamoun et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Available online at: http://www.panafrican-med-journal.com/content/article/32/113/full

Corresponding author: Salma Kamoun, Department of Pathology, Rabta Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunisia (salma_kam@yahoo.fr)


Desmoplastic infantile ganglioglioma

Salma Kamoun1,&, Heifa Azouz2, Marwa Zemmali2, Slim Haouet2, Nidhameddine Kchir2

 

1Department of Neurosurgery, National Institute of Neurology, Tunis, Tunisia, 2Department of Pathology, Rabta Hospital, Tunis, Tunisia

 

 

&Corresponding author
Salma Kamoun, Department of Neurosurgery, National Institute of Neurology, Tunis, Tunisia

 

 

Abstract

The term desmoplastic infantile ganglioglioma was coined by VandenBerg et al. in 1987. In their first report these authors referred to a rare, distinct brain tumor. About 60 cases of desmoplastic infantile ganglioglioma have been described in the literature since its first description. We report a case of a 6-year-old girl who was admitted for seizure without family history. Magnetic resonance imaging scan showed a hypodense area in the right temporal region. A right temporal craniotomy was performed and the tumor was excised. The pathologic examination revealed the diagnosis of desmoplastic infantile ganglioglioma.

 

 

Introduction    Down

The term desmoplastic infantile ganglioglioma (DIG) was coined by VandenBerg et al. in 1987 [1]. In their first report these authors referred to a rare, distinct brain tumor. About 60 cases of DIG have been described in the literature since its first description [2]. In this work, we describe the histologic, immunohistochemical and differential diagnoses of DIG.

 

 

Patient and observation Up    Down

A 6-year-old girl was admitted for seizure without family history. Neurological examination was normal. Magnetic resonance imaging scan showed a hypodense area in the right temporal region, with contrast enhancing solid as well as large cystic components (Figure 1). A right temporal craniotomy was performed and the tumor was excised. The pathologic examination revealed a markedly desmoplastic tumor, showing deposition of dense collagen fibers. The neoplasic cell population was heterogenous, composed of spindle-shaped astrocytes with a fascicular arrangement (Figure 2). These cells show intense immunoreactivity for GFAP (Figure 3). Scattered ganglion cells with copious cytoplasm and irregular nuclei were also observed, indicating neuronal differentiation (Figure 4). These neuronal cells express synaptophysin (Figure 5). No mitosis or necrosis was present.

 

 

Discussion Up    Down

DIG is a supratentorial brain tumor occurring before 2 years. It has similar clinical and neuroimaging features with desmoplastic infantile astrocytoma (DIA), including a favourable prognosis, but DIA lacks ganglion cells. DIG and DIA have been categorized together as desmoplastic infantile astrocytoma/ganglioglioma in the last editions of the WHO classification. They are WHO grade 1 tumors [3]. The main histologic differential diagnoses are pleomorphic xanthoastrocytoma, gliofibroma, ganglioglioma [4] and other small blue cell tumors of the central nervous system (medulloblastoma, central nervous system primitive neuroectodermal tumors, pineoblastomas) [5]. Total resection is the best treatment and offers long term survival [6]. Dissemination of these tumors through the cerebrospinal fluid has been reported, but is rare event [7]. Anaplastic histological features as high mitotic rate, microvascular proliferation and perinecrotic palisading tumor cells had no influence over survival [3, 8].

 

 

Conclusion Up    Down

DIA and DIG are tumors of the same family. They occur in children under 2-year-old. Their diagnosis should be rendered only after correlation between neuropathologic, clinical and neuroimaging features. They have favorable prognosis if completely resected.

 

 

Competing interests Up    Down

The authors declare no competing interest.

 

 

Authors’ contributions Up    Down

Salma Kamoun wrote the manuscript; Heifa Azouz took the photos and corrected the final version; Marwa Zemmali collected the data; Slim Haouet established the diagnosis; Nidhameddine Kchir supervised the manuscript. All the authors have read and agreed to the final manuscript.

 

 

Figures Up    Down

Figure 1: magnetic resonance imaging scan showing an area with contrast enhancing in the right temporal region

Figure 2: spindled portion of the tumor, the cells have obviously eosinophilic cytoplasm and glial appearance

Figure 3: GFAP is strongly positive in the glial portion of the tumor, but negative in blood vessel and mesenchymal cells in the desmoplastic areas

Figure 4: neuronal portion of tumor, cells range from atypical ganglionic cells to small polygonal cell types

Figure 5: synaptophysin is positive in ganglion cells

 

 

References Up    Down

  1. VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores S et al. 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. Journal of neurosurgery. 1987; 66(1): 58-71. PubMed

  2. Iwami K-i. Desmoplastic infantile ganglioglioma. Child's Nervous System. 2007; 23(6): 619-20. PubMed

  3. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. WHO Classification of tumours of the central nervous system. Acta Neuropathol. 2007 Aug; 114(2): 97-109. PubMed

  4. Craver RD, Nadell J, Nelson JS. Desmoplastic infantile ganglioglioma. Pediatr Dev Pathol. 1999 Nov-Dec; 2(6): 582-7. PubMed

  5. Prayson RA, Cohen ML, Kleinschmidt-De Masters B. Brain Tumors. 2009 Google Scholar

  6. Sugiyama K, Arita K, Shima T, Nakaoka M, Matsuoka T, Taniguchi E et al. Good clinical course in infants with desmoplastic cerebral neuroepithelial tumor treated by surgery alone. Journal of neuro-oncology. 2002; 59(1): 63-9. PubMed | Google Scholar

  7. De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G et al. Desmoplastic infantile ganglioglioma: a potentially malignant tumor. The American journal of surgical pathology. 2002; 26(11): 1515-22. PubMed | Google Scholar

  8. Kuchelmeister K, Steinhauser A, Korf B, Wagner D, Prey N, Schachenmayr W. Anaplastic desmoplastic infantile ganglioglioma. Clin Neuropathol. 1998; 17: 269-70.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 


The Pan African Medical Journal articles are archived on Pubmed Central. Access PAMJ archives on PMC here

Volume 34 (September - December 2019)

Article tools

PDF (601 Kb)
Contact the corresponding author
Download to Citation Manager
EndNote
Reference Manager
Zotero
BibTex
ProCite


Keywords

Tumor
Central nervous system
Infantile desmoplastic ganglioglioma

Rate this article

Altmetric

PAMJ is a member of the Committee on Publication Ethics
PAMJ Authors services
Next abstract

PAMJ is published in collaboration with the African Field Epidemiology Network (AFENET)
Currently tracked by: DOAJ, AIM, Google Scholar, AJOL, EBSCO, Scopus, Embase, IC, HINARI, Global Health, PubMed Central, PubMed/Medline, Ulrichsweb, More to come . Member of COPE.

ISSN: 1937-8688. © 2019 - Pan African Medical Journal. All rights reserved