Desmoplastic infantile ganglioglioma: A case report and review of literature
Keywords:Desmoplastic infantile ganglioglioma, histopathology, immunohistochemistry
Background and objective: Desmoplastic infantile gangliogliomas are rare low grade supratentorial masses that are seen in the pediatric age group. Typically, they involve the superficial cerebral cortex and leptomeninges. Despite the large size of these lesions and some worrisome histological and radiological features, prognosis is generally favorable after gross total resection. To our knowledge, out of about one hundred cases reported worldwide, this is the first Desmoplastic infantile ganglioglioma reported in Kurdistan region-Iraq.
Case presentation: We report a case of Desmoplastic infantile ganglioglioma in a 2-month-old male infant, Computed tomography and magnetic resonance imaging revealed a supratentorial mixed cystic and solid temporal tumor. Near total surgical removal was done. Histopathology revealed features of desmoplastic infantile ganglioglioma, with marked desmoplastic component, glial and neuronal elements. Immunohistochemistry showed positive staining for glial fibrillary acidic protein with areas of synaptophysin positivity.
Conclusion: The diagnosis of Desmoplastic infantile ganglioglioma should be considered in differential diagnosis of tumors with a cystic component and an aggressive appearance.
Rout P, Santosh V, Mahadevan A, Kolluri VR, Yasaha TC, Shankar SK. Desmoplastic infantile ganglioglioma: clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 2002; 18:463–7.
Ganesan K, Desai S, Udwadia-Hegde A. Non-infantile variant of desmoplastic ganglioglioma: a report of 2 cases. Pediatr Radiol 2006; 36:541–5.
Pommepuy I, Delage-Corre M, Moreau JJ, Labrousse F. A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature. J Neurooncol 2006; 76:271–5.
Per H, Kontas¸ O, Kumandas¸ S, Kurtsoy A. A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. Neurosurg Rev 2009; 32:369–74.
Fan X, Larson TC, Jennings MT, Tulipan NB, Ton SA, Johnson MD. Six month old boy with 2 week history of progressive lethargy. Brain Pathol 2001; 11:265–6.
Khaddage A, Chambonniere ML, Morrison AL, Allard D, Dumollard JM, Pasquier B, et al. Desmoplastic infantile ganglioglioma: A rare tumor with an unusual presentation. Ann Diagn Pathol 2004; 8:280–3.
Duffner PK, Burger PC, Cohen ME, Sanford RA, Crischer JP. Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurg 1994; 34:583–9.
Sperner J, Gottschalk J, Neumann K, Schör- ner W, Lanksch WR, Scheffner D. Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma. Childs Nerv Syst 1994; 10:458–62.
Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N. Desmoplastic Infantile Ganglioglioma. A Report of 2 cases and a review of the Literature. Pediatr Neurosurg 2008; 44:422–5.
Brat DJ, Vandenberg SR, Figarella-Branger D, Reuss DE. Desmoplastic infantile astrocytoma and ganglioglioma. World Health Organization Classification of Tumors. Pathology and genetics of tumors of the nervous system. IARC:Lyon; 2016. P.144–6.
Burger PC, Scheithauer BW. Tumors of the central nervous system. AFIP atlas of tumor pathology; Fourth series, fascicle. AFIP: Washington D.C: Amer Registry of Pathology ; 2008. P. 218–22.
Komori T, Scheithauer BW, Parisi JE, Watterson J, Priest JR. Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow up. Mod Pathol 2001; 14:720–6.
VandenBerg SR, May EE, Rubeinstein LJ, Herman MM, Perentes E, Vinores SA, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). J Neurosurg 1987; 66:58–71.
VandenBerg SR. Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 1993; 3:275–81.
Avcı E, Öztürk A, Baba F, Torun F, Karabağ H, Yücetaş S. Desmoplastic infantile ganglioglioma: Case report. TJP 2008; 50:495–9.
Mallucci C, Lellouch-Tubiana A, Salazar C, Cinalli G, Renier D, Sainte-Rose C, et al. The management of desmoplastic neuroepithelial tumors in childhood. Childs Nerv Syst 2000; 16:8–14.
Taratuto AL, Monges J, Lylyk P, Leiguarda R. Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 1984; 54:2505–12.
Chandrashekhar TN, Mahadevan A, Vani S, Yasha TC, Sampath S, Chandramouli BA, et al. Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute. Neuropathol 2012; 32:1–12.
Setty SN, Miller DC, Camras L, Charbel F, Schmidt ML. Desmoplastic infantile astrocytoma with metastases at presentation. Mod Pathol 1997; 10:945–51.
Rutka JT, Giblin JR, Apodaca G, DeArmond SJ, Stern R, Rosenblum ML. Inhibition of growth and induction of differentiation in a malignant human glioma cell line by normal leptomeningeal extracellular matrix proteins. Cancer Res 1987; 47:3515–22.
Samarathunga AAS, Fernando C, Fernando J. Desmoplastic infantile astrocytoma; an uncommon pediatric intracranial tumor. JDP 2012; 1:59–61.
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