Case 2 -
Desmoplastic Non-Infantile Ganglioglioma
Tarik Tihan and Maher Alsughayer
UCSF School of Medicine
San Francisco, CA, and
King Hussein Cancer Center
Amman , Jordan
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A 6-year-old boy presented with a history of unsteady gait and left arm weakness of one month duration that was progressive in nature. There was no other significant medical history. Physical examination revealed horizontal nystagmus, loss of coordination on the left side and left upper extremity weakness with atrophy. Preoperative CT and MRI scans were ordered, which showed a large right cerebral contrast-enhancing mass lesion with a cystic component. Craniotomy with resection of the mass were performed.
Desmoplastic Non-Infantile Ganglioglioma
Desmoplastic supratentorial neuroepithelial neoplasms of infancy typically occur within the first 18
months of life, and are characterized by a prominent desmoplastic stroma and a divergent neuroepithelial
tumor cell population. Such tumors with predominantly astrocytic phenotype were initially described by
Taratuto et al. , while those with both glial and neuronal features were considered under the
Desmoplastic Infantile Ganglioglioma (DIG)
diagnosis by Vandenberg et al. . Both of these
neoplasms have been considered within the same spectrum of neoplasms
10]. These infantile neoplasms
are well recognized, and more than 100 cases have been reported to date.
Tumors with the typical radiological and histological features of DIG are extremely rare in the
17]. Kuchelmeister et al. first described the entity of
desmoplastic non-infantile ganglioglioma (DNIG) in one adolescent and one adult patient . Reports of
unusual "desmoplastic" pediatric tumors can also be considered in this category .
The correct diagnosis of a desmoplastic ganglion cell tumor is paramount because these neoplasms may
appear aggressive radiologically and histologically, and may be mistaken for a "malignant" glioma .
Even though the infantile tumors with similar features have an excellent prognosis and the non-infantile
cases reported to date seem to have a very favorable outcome, long term prognosis of the non-infantile
tumors and treatment options beyond surgery are still unclear.
DNIG is an exceedingly rare tumor, and the information from 11 patients form our opinion on the
characteristics of this entity
17]. Patients with DNIG commonly present with
significant neurologic deficits, seizures, or hemiparesis. The tumor typically involves the cerebral
hemisphere. The pertinent imaging features include a large cystic and solid mass, and contrast
enhancement in the solid component. Grossly, the tumors have large cysts containing small amounts of
fluid. Some tumors can be attached to the adjacent parenchyma. The main histological features are the
abundance of desmoplasia that gives the tumor a fibroblastic rather than a neuroepithelial character.
Almost all cases of DNIG reported to date displayed large ganglionic cells resembling mature neurons, but
it is possible that some examples may be predominantly of astrocytic phenotype, similar to some infantile
tumors. Eosinophilic granular bodies, and perivascular lymphocytic infiltrates can be seen in some
tumors. Despite the presence of gemistocytic cells in all tumors, the epithelioid cells with loose
papillary pattern seen in our case has not been reported.
DIG and DNIG have numerous common and rare disparate features. The similarities are
noted in the clinical presentation, the radiographic characteristics, and the profound desmoplasia.
Histologically, there are more commonalities than the simple presence of desmoplasia. The fundamental
differences between DIG and DNIG seem to be the age of onset and the rare histological features such as
epithelioid and papillary architecture peculiar to some of the non-infantile examples. These features,
like the age at presentation, may be of little or no consequence, or less likely, may be indicators of
different etiological or pathogenetic process. Another intriguing aspect of DNIG is reflected in the
case presented by Jay et al. . These authors demonstrate an increasingly desmoplastic neoplasm with an
extraordinary degree of neuronal differentiation and overlapping features between DIG and PXA. This case
along with other reports of glioneuronal tumors with overlapping, or 'hybrid' features point to the
possibility of a precursor cell with variable differentiation pathways
11]. The presence of DNIG
in nature is clear, yet a separate pathologic entity from DIG is highly debatable. The importance of
this case study is to document the clinicopathological features and comparison with cases published to
date in order to aid future studies that can address the questions on the origin of these neoplasms.
A review of desmoplastic gangliogliomas in the non-infantile age group
|Reference ||Age (yr) ||Sex ||Location ||Treatment ||Follow-up ||Outcome|
|Galatioto et al.  ||17 ||M ||Temporal ||GTR, No adjuvant therapy ||N/A ||N/A|
|Jay et al.  ||11 ||F ||Temporal ||GTR, 2nd Sx, ||9 months ||AWD|
|Kuchelmeister et al. case#1  ||15 ||M ||Parietooccipital ||GTR, No adjuvant therapy ||N/A ||N/A|
|Kuchelmeister et al. case#2 ||25 ||M ||Occipital ||GTR, No adjuvant therapy ||N/A ||N/A|
|Marti et al.  ||19 ||M ||Parietotemporal ||GTR, 2nd Sx ||N/A ||N/A|
|Onguru et al.  ||14 ||M ||Parietooccipital ||GTR, No adjuvant therapy ||N/A ||N/A|
|Pommepuy et al.  ||12 ||F ||Parietooccipital ||GTR, No adjuvant therapy ||13 years ||NED|
|Rout et al. case#4  ||6 ||F ||Frontoparietal ||STR, RT ||9 months ||NED|
|Torres et al.  ||5 ||M ||Parietal ||GTR, No adjuvant therapy ||1 year ||NED|
|Torres et al.  ||7 ||F ||Parietal ||GTR, No adjuvant therapy ||1 year ||NED|
|Woesler et al.  ||14 ||M ||Temporal ||GTR, No adjuvant therapy ||2 years ||NED|
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