Ganglioglioma with anaplastic transformation

Ganglioglioma com transformação anaplásica

IMAGES IN NEUROLOGY

Ganglioglioma with anaplastic transformation

Ganglioglioma com transformação anaplásica

Fabiano Reis^I; Guilherme Henrique Alves Vieira^II; Ricardo Schwingel^II; Vinicius Trindade Gonçalves^II; Luciano de Souza Queiroz^III

^IProfessor of the Department of Radiology of the Clinics Hospital, Faculty of Medical Sciences, Universidade Estadual de Campinas (Unicamp), Campinas SP, Brazil

^IIMedical student; Department of Radiology of the Clinics Hospital, Faculty of Medical Sciences, Unicamp, Campinas SP, Brazil

^IIIProfessor of the Department of Pathology of the Clinics Hospital, Faculty of Medical Sciences, Unicamp, Campinas SP, Brazil

^{Correspondence}

A nine year-old male had refractory seizures for four years. Diagnostic imaging (Fig 1A and B) demonstrated a cortico-subcortical focal lesion at the isthmus of the right cingulate gyrus. Biopsy yielded diagnosis of ganglioglioma (Fig 2A). Reoperation one year later showed similar features, but included atypical and multinucleated cells (Fig 2B).

At the age of 13, the patient had symptom recurrence. Diagnostic imaging (Fig 1C and D) demonstrated a heterogeneous enhanced lesion in the right parietal region, with necrosis. Histology (Fig 2C to F) revealed a malignant glial tumor with the appearance of glioblastoma multiforme. A few tumor cells were positive for chromogranin. A diagnosis of malignant transformation of ganglioglioma was made.

Gangliogliomas are rare tumors predominating in the early decades of life, with strong association with long term intractable epilepsy^1-3. They are composed of variable proportions of glial (mainly astrocytic) cells and mature or dysplastic neurons². Malignant change is a rare, but well recognized, complication. Transformation of the glial component from the low grade to a higher grade is observed in most cases. Also, there is a case of malignant transformation secondary to degeneration of the neuronal component into a neuroblastoma⁴. Some reports in literature⁵ suggest that radiation may predispose to malignant degeneration. In this case, the patient did not receive postoperative radiation.

Received 29 February 2012

Received in final form 17 May 2012

Accepted 25 May 2012

Conflict of interest: There is no conflict of interest to declare.

• 1. Miller DC, Lang FF, Epstein FJ. Central nervous system gangliogliomas. Part I: Pathology. J Neurosurg 1993;79:859-866.
• 2. Lantos PL, VandenBerg SR, Kleihues P. Tumors of the nervous system. In: Graham DI, Lantos PL (Eds). Greenfield's neuropathology. 6th ed. London, England: Arnold 1997:583-794.
• 3. Luyken C, Blümcke I, Fimmers R, et al. The spectrum of long-term epilepsy-associated tumors: long-term seizure and tumor outcome and neurosurgical aspects. Epilepsia 2003;44:822-830.
• 4. David KM, de Sanctis S, Lewis PD, Noury AM, Edwards JM. Neuroblastomatous recurrence of ganglioglioma. Case report. J Neurosurg 2000;93:698-700.
• 5. Rumana CS, Valadka AB. Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas. Neurosurgery 1998;42:1038-1043.

Publication Dates

• Publication in this collection
  12 Sept 2012
• Date of issue
  Sept 2012