MyPACS.net: Radiology Teaching Files > Case 647376

previously visited RECURRENT GANGLIOGLIOMA
Contributed by: Dr Phillip Silberberg, Children's Hospital Omaha, Radiologist, Omaha Childrens, Creighton University and UNMC, Nebraska, USA.
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Fig. 1: CT Head without contrast, 27 May, 2003, shows a cystic tumor with a calcified mural nodule in the right cerebellum. This creates mass effect and midline shift to the left and also causing an obstructive hydrocephalus. Surrounding cerebellar edema is seen. This is a typical location and appearance of a pilocytic astrocytoma or a hemangioblastoma if this patient has Von Hippel Lindau.

Fig. 2: Axial post-contrast Brain MRI, 27 May, 2003 demonstrates a cystic lesion with rim enhancement, which is atypical for a JPA. An enhancing nodule is identified medial to the main tumor cyst, which may or may not be connected to the main tumor nodule. A ganglioglioma and DNET should also be considered.

Fig. 3: Axial post contrast Brain MRI, 27 May, 2003.

Fig. 4: Coronal Post-Contrast Brain MRI, 27 May, 2003. These images demonstrate the relationship between the main mural nodule and the other region of enhancment as previously described.

Fig. 5: Sagittal Post-Contrast Brain MRI, 27 May, 2003.

Fig. 6: Axial Pre-Contrast Brain MRI, 19 January, 2004 shows the post operative bed.

Fig. 7: Axial Post-Contrast Brain MRI, 19 January, 2004 ?enhancement.

Fig. 8: Axial Pre-Contrast Brain MRI, 19 January, 2004 A few foci of increased signal seen, espeically posteriorly, likely post operative.

Fig. 9: Axial Post-Contrast Brain MRI, 19 January, 2004; minimal enhancement anterior resection margin

Fig. 10: Sagittal Pre-Contrast Brain MRI, 19 January, 2004. Post op. Looks essentially normal.

Fig. 11: Sagittal Post-Contrast Brain MRI, 19 January, 2004 shows 2 small round areas of enhancement. This may be tumor residual or recurrence. Less likely post operative healing.

Fig. 12: Axial Pre-Contrast Brain MRI, 19 August, 2004 essentially normal signal involving the tumor bed.

Fig. 13: Axial Post-Contrast Brain MRI, 19 August, 2004 a new area of enhancement in the tumor bed or it is just better seen.

Fig. 14: Axial pre-contrast Brain MRI 19 August, 2004 demonstrates a few tiny foci of hyperintensity in the tumor bed.

Fig. 15: Axial post contrast Brain MRI 19 August,2004; 5 mm anterior nodule has enlarged from 1 mm on prior exam.

Fig. 16: low grade neoplasm with rosenthal fibers and glial component

Fig. 17: ganglion and glial components

Fig. 18: focally numerous calcifications

Fig. 19: cystic areas
Findings:

5/28/03 MRI 1. Large right cerebellar intra-axial solid and cystic mass with peripheral ring enhancement of the cyst and adjacent edema.
2. Mass effect with lateral displacement of the 4th ventricle with mild obstructive hydrocephalus and secondary mild inferior cerebellar tonsillar ectopia.

1/19/04 MRI 1. Postoperative changes involving the right cerebellar hemisphere with decreasing enhancement within the surgical bed most likely representing postsurgical changes, however followup recommended.
2. Interval resolution of pneumocephalus and right subdural fluid collection adjacent to the cerebellum.

08/19/04 MRI 1. Definite residual tumor with multiple small enhancing nodules ranging in size from 1 to 5 mm noted along the postsurgical defect in the right cerebellum. The most inferior nodule demonstrates the greatest growth since 19 Jan 04, as it previously measured 1 mm and now measures 5 mm.

Discussion:

Gangliogliomas account for 0.4%0.9% of all intracranial neoplasms and 1%4% of all pediatric neoplasms of the CNS.  Most (80%) occur in patients younger than 30 years with a peak age of incidence between 10 and 20 years. They may manifest in newborns with or without neurologic impairment and are slightly more common in males. There is a distinct predilection for the cerebral hemispheres, especially the temporal lobe. The majority occurred in the temporal lobe (38%), followed by the parietal lobe (30%) and the frontal lobe (18%). Numerous other locations have been reported, including the brainstem, cerebellum, pineal region, spinal cord, optic nerve, optic chiasm, and ventricles. A case of a ganglioglioma attached to the septum pellucidum within the lateral ventricle with an imaging appearance virtually identical to that of a subependymoma or central neurocytoma has been reported. Gangliogliomas of the temporal lobe are commonly associated with the clinical presentation of medically refractory seizures, particularly those of the partial complex type. These tumors are the most common cause (40%) of chronic temporal lobe epilepsy. Clinical associations include several reports in the literature of "hemifacial spasm" in combination with a cerebellar ganglioglioma occurring in infants and one case report of coexisting ganglioglioma and Rasmussen encephalitis in the same cerebral hemisphere. Rare locations for gangliogliomas include the pineal region and the ventricular system.

Gangliogliomas share many imaging features with other low-grade neoplasms. Cross-sectional imaging studies reveal a solid mass (43%), a cystic mass (5%), or a solid-cystic combination (52%) that is typically located in the periphery of a cerebral hemisphere. Calcification is a common finding. Reflecting a generally benign biologic nature, there is usually little associated mass effect or evidence of surrounding vasogenic edema. Gangliogliomas that occur in children tend to have greater overall tumor volume (average: eight times larger) than those arising in adults.  Gangliogliomas have variable manifestations at nonenhanced CT. A hypoattenuating mass is the most frequent manifestation (38%), followed by a mixed attenuation mass (32%) and an isoattenuating mass (15%) or hyperattenuating mass (15%) Calcification is less commonly seen in association with solid-appearing lesions .Remodeling of the skull may be seen if the neoplasm is located within the peripheral brain. Occasionally, the neoplasm may be completely undetectable at CT. The frequency of enhancement following intravenous administration of iodinated contrast media is variable, occurring in 16%80% of gangliogliomas. The MR imaging appearance of gangliogliomas is also variable and nonspecific. In general, the lesions are hypointense to isointense relative to gray matter on T1 images and hyperintense relative to gray matter on T2 imaging.  The solid-appearing components have an even more variable presentation at imaging. Some tumors may manifest as a hyperintense mass on T1-weighted images. They commonly have at least some regions of high signal intensity on T2-weighted images . Not all gangliogliomas are truly cystic despite a cystlike appearance, and the term cystic should be reserved for those that demonstrate a fluid-fluid level or pulsation artifact on MR images. Enhancement following intravenous administration of gadolinium contrast material is highly variable, ranging from nonenhancing to ringlike to intense homogeneity. Leptomeningeal spread of a ganglioglioma is rare. Gangliogliomas produce heterogeneous metabolic activity on PET images.

Gangliogliomas contain both ganglion and glial elements of varying differentiation. Gangliogliomas and gangliocytomas are part of the family of tumors composed of mature ganglion cells and dysplastic neurons. Corresponding immunoreactivity for these two cellular populations is noted with appropriate immunohistochemical stains, typically glial fibrillary acidic protein for the glial cells and synaptophysin and neurofilament protein for the neuronal group. The glial component is absent in gangliocytomas. Only gangliogliomas contain neoplastic glial cells, typically composed of astrocytes in varying states of differentiation. These glial cells directly affect the biologic behavior of the tumor. Since most gangliogliomas have astrocytes with histologic features more typical of a low-grade pilocytic astrocytoma, the biologic behavior of these lesions tends to be benign and correlates with the slow growth typically seen in these lesions. In the rare instance of a biologically aggressive ganglioglioma, the astrocytic cells are less differentiated and more similar to those seen in higher-grade glial neoplasms .Despite the presence of aggressive histologic features, the biologic behavior of these anaplastic gangliogliomas is quite variable. Some patients with these tumors will die secondary to diffuse dissemination of their disease, whereas others may have extended survival times following surgical intervention. The location of the tumor appears to have the most effect on the eventual
outcome for these patients.  Malignant degeneration of gangliogliomas is rare, with an estimated occurrence of 6%. Transformation of the glial component from the typical low-grade appearance to a higher-grade form (usually grade III or IV glioma) is present in almost all cases of malignant degeneration. Very rarely, a de novo ganglioglioma may degenerate in both glial and neuronal cell populations.Extremely rarely malignant transformation in a ganglioglioma that was entirely secondary to degeneration of the neuronal component into a neuroblastoma has been reported. Some reports in the literature suggest that postoperative radiation therapy may predispose a ganglioglioma to malignant degeneration. Others have suggested some of these cases of "malignant degeneration" may reflect differentiation or maturation of a primitive neuroectodermal tumor.

Gross total resection of gangliogliomas is recommended as the treatment of choice, resulting in resolution of seizure activity for the majority of patients. The role of radiation therapy and chemotherapy has been debated in the literature. Most authorities believe that radiation therapy and chemotherapy should be reserved for those patients with rare malignant forms of ganglioglioma (eg, anaplastic ganglioglioma), disease progression, or an unresectable ganglioglioma (eg, one located in the optic nerve, optic chiasm, or hypothalamus). The deleterious side effects on the developing nervous system of children with these tumors are cited as a strong argument against the use of radiation as a primary therapeutic modality


Desmoplastic infantile gangliogliomas (DIG),
are extremely rare neuroepithelial tumors
occurring in children less than 2 years of age.  DIG most commoly occur in the frontal or parietal lobes.  They are large at presentation, commonly involving more than one lobe. CT typically shows a large, parenchymal cystic tumor with an isodense and intensely enhancing surface mass.  The cyst contains xanthochromic fluid. The solid portion (representing the region of intense desmoplasia) is slightly hyperattenuating and typically located along the cortical margin of the mass.  It enhances intensely following intravenous administration of contrast media. Mass effect may be present. The MR imaging appearance is similar. The solid portion is generally isointense to the brain parenchyma on T1- and T2-weighted images and enhances intensely following administration of gadolinium contrast media. Extension of the enhancement to the leptomeningeal margin is characteristic on both CT and MR images and correlates with the firm dural attachment noted in many cases. Calcification is not a feature of desmoplastic infantile ganglioglioma and may aid in distinguishing this lesion from frequently calcified neoplasms seen in the 1st year of life, such as primitive neuroectodermal tumor and supratentorial ependymoma. Vasogenic edema is occasionally seen.
 Histopathology will reveal a significant desmoplastic component with occasional neoplastic astrocytes and interspersed Rosenthal fibers.  Immunohistochemistry is utilized to differentiate DIG and desmoplastic cerebral astrocytoma of infancy.  The former will stain intensely positive for Glial Fibrillary Acidic Protein, a glial marker as well as positivity for synaptophysin or neurofilament immunostain.  It is the pilocytic astrocytes and Rosenthal fibers that show cytoplasmic positivity for GFAP and the ganglion cells that react for synaptophysin.  In contrast, desmoplastic cerebral astrocytoma is GFAP positive alone. Children often present with macrocephaly, seizures, and/or psychomotor delay.  The prognosis is favorable.  This is due to the surface location and clear demarcation making them amenable to surgical excision.  The lesion has low malignant potential and requires no adjuvant therapy. 

References:

Contributed by:
Jennifer Lee, Creighton Medical Student M4,Creighton University, Omaha, Nebraska.
Dr Phillip Silberberg, Radiologist, Omaha Childrens, Creighton University and UNMC, Nebraska, USA.

References:
1. From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Koeller KK, Henry JM. Radiographics. 2001 Nov-Dec; 21(6):1533-56. http://radiographics.rsnajnls.org/cgi/content/full/21/6/1533

2. Gangliogliomas: A Report of Five Cases. By: Nair, V.; Suri, V.S.; Tatke, M.; Saran, R.K.; Malhotra, V.; Singh, D.. Indian Journal of Cancer, Jan-Mar2004, Vol. 41 Issue 1, p41-46.

3. DESMOPLASTIC INFANTILE GANGLIOGLIOMA. By: Izban, Keith F.; Thomas, Chinnamma. Pediatric Pathology & Molecular Medicine, Mar1998, Vol. 18 Issue 2, p157-172, 16p4. Desmoplastic cerebral glioblastoma of infancy. By: Al-Sarraj, S. T.; Bridges, L. R.. British Journal of Neurosurgery, Apr96, Vol. 10 Issue 2, p215-219, 5p, 9bw

5. Desmoplastic infantile ganglioglioma - A case report. By: Balasubramanian, D.; Ramesh, V.G.; Deiveegan, K.; Ghosh, Mitra; Mallikarjuna, V.S.; Annapoorneswari, T.P.; Chidambaranathan, N.; Ramani, K.V.N.. Neurology India, Sep2004, Vol. 52 Issue 3, p384-386, 3p

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Additional Details:

Case Number: 647376Last Updated: 08-20-2006
Anatomy: Cranium and Contents   Pathology: Neoplasm
Modality: MR, PathologyExam Date: 01-01-1985Access Level: Readable by all users

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