Anaplastic ganglioglioma in a middle-aged woman: a case report with a review of the literature

Abstract

We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant radiation therapy and chemotherapy were given after gross total resection of the tumor. Histological and immunohistochemical studies showed an anaplastic ganglioglioma. Gangliogliomas of the central nervous system are rather uncommon tumors, and anaplastic ones are extremely rare. The pertinent literature regarding gangliogliomas is reviewed.

Fig. 1

Preoperative MRI showing an irregular strong enhancing mass in the left frontal lobe extending to the cingulate gyrus (A). MRI on the 12th postoperative day showing multiple irregularly shaped enhancing lesions along the resection margin of the left frontal lobe and cingulate gyrus (B). After chemotherapy and radiation therapy, MRI 5 months postoperatively revealed that the irregular enhancing lesions in the resected margin of the left frontal lobe and cingulate gyrus had disappeared (C). And, the last follow-up MRI at 35 months postoperatively shows no evidence of recurrence (D, E).

Fig. 2

Photomicrographs showing the histopathology of the ganglioglioma in our case. (A) The tumor cells infiltrated the gray matter and subarachnoid space. The tumor cells in the gray matter consisted of neoplastic ganglion cells and glial cells. Those in the subarachnoid space (asterisk) are mainly glial cells and show increased cellularity, nuclear atypism, and occasional mitosis (hematoxylin and eosin, original magnification, ×40). (B) Atypical glial cells (arrow) and scattered ganglion cells (white arrow) constitute the ganglioglioma. Note the increased cellularity and nuclear atypism of the glial cells (hematoxylin and eosin, original magnification, ×400). (C) Neoplastic ganglion cells (arrow) are clustered abnormally and lack orientation and polarity. Most have large nuclei with prominent nucleoli (hematoxylin and eosin, original magnification, ×400).

Fig. 3

Immunostaining features suggestive of anaplastic gangliogliomas. Neoplastic ganglion cells are positive for NSE (A) and synaptophysin (B). (C) Glial components are positive for GFAP, and scattered ganglion cells are negative. (D) The Ki-67 LI is high (20%) in the region of anaplastic transformation.