Non-infantile desmoplastic ganglioglioma: a case report

Introduction Desmoplastic infantile ganglioglioma(DIG) is a supratentorial dural-based large cystic brain tumor. It includes in WHO classification under the category of mixedglio-neural tumors and its grade is I. DIG is typically occurs in first 24 months of life. Rare tumors have been described in older subjects. The prognosis is good and it is curative by surgical total removal. We report a case of desmoplastic ganglioglioma in a 17 years old girl. Case repot A 17 years old girl suffered from headache and seizure from 4 months ago. Brain spiral CT scan demonstrated a lobulated hypodense mass M:42mm in left temporooccipital area containing a few curvilinear calcifications along with twocystic structures medial to it at projection of brain stem. Craniotomy from temporal floor was done and the mass with irregular borders excised. The specimen which was reffered in the pathology department was consist of multiple fragments of gray soft tissue totally M:4*2*1cm. Histological examination revealed a relatively low cellular glial neoplasm composed of neoplastic cells with elongated hair like process occasionally arranged in parallel bundles in fibrillary background altenating with areas of microcystic changes. Also some enlarged neoplastic ganglion cells were seen. Some Rosenthal fibers and eosinophilic bodies were present in glial area. Invasion to dura matter and areas of desmoplasia, highlighted by trichrome staining were evident. Borders of tumor and surrounding brain parenchyma show calcification. IHC staining was performed which show reactivity with GFAP and S100 in glial tumor cells and Synaptophysin in ganglion cells. The overall histomorphology and IHC findings were infavor of low grade desmoplastic ganglioglioma. Discussion DIGs are rare intracranial tumors occur mostly before the age of 2 years, but rare cases happen later. They are massive, firmly attached to the dura. CT scan show a large mass with solid-cystic areas. Histologically, the most prominent feature is desmoplasia(GFAP positive) as well as presence of ganglion cell component(synaptophysin positive) Some differential diagnoses include: -Pleomorphic xanthoastrocytoma (differentiate by lipidization of the cells and absence of neural component) -Gliofibroma (It is infratentorial and lacks neural component) -Ganglioglioma (It lacks desmoplasia) Our observation and other case reports indicate that desmoplastic ganglioglioma can occur in young adults. Like infantile cases, non-infantile gangliogliomas seem to have a favorable prognosis with surgical total removal and rarely require a chemotherapy or radiotherapy.