2. Desmoplastic infantile ganglioglioma
Desmoplastic infantile ganglioglioma (DIG) is a rare tumor that
typically occurs in infants under the age of 24 months. These
tumors commonly have a good prognosis after surgical
resection despite their aggressive radiological appearances.
Desmoplastic infantile ganglioglioma (DIG) was first described
by Vandenberg et al. in 1987.
DIG is a rare supratentorial tumor with dense desmoplastic
tissue and divergent astrocytic and ganglionic differentiation.
It occurs with a male:female ratio of 1.7:1.0.
It is classified as a benign tumor and coded as WHO grade 1.
3. Desmoplastic infantile ganglioglioma
Astrocyte-like cells had large, round and vesicular nuclei with surrounding large
amphophilic (staining with both acidic and basic dyes) cytoplasm. Neuronal cells
had vesicular chromatin and polygonal nuclei with large perikarya. The cells
were surrounded by diffuse reticulin (Gomori stain) and collagen fibers (Masson
Trichome – MTC stain).
Tumor cells were stained for glial fibrillary acidic protein (GFAP), synaptophysin,
chromogranin, S-100 and epithelial membrane antigen (EMA), and they were
negative for CD34, NFP and progesterone receptor. Nearly 2% of tumor cells
were labeled with Ki-67/MIB-1.
There are a few cases in the literature regarding CSF dissemination and
malignant transformation. Thus, CSF examination is recommended in every
patient with deeply located and subtotally resected DIGs.
4. Desmoplastic ganglioglioma
The radiological differential diagnosis of DIG includes
dysembryoblastic neuroepithelial tumor/DNET, primitive
neuroectodermal tumor/PNET and ependymoma.
Dysembryoblastic neuroepithelial tumor is a supratentorial cortical
lesion with absence of mass effect, perilesional edema, and small
cystic component.
Primitive neuroectodermal tumors are located within the deep white
matter and have both cystic and solid components.
Ependymomas are primarily in a paraventricular location along the
parietal white matter.
5. Most of DIGs have a favorable prognosis after gross total
resection, despite their frequent large size, their cytological
characteristics and their infiltrating pattern… We report a new
case of desmoplastic ganglioglioma in a 12-year-old patient
with a follow-up of 13 years.
6. At surgery, the superficial
component of the tumor was
firmly attached to the dura.
Surgical resection was
macroscopically complete. No
adjuvant treatment was given.
Thirteen years after surgery, the
patient is symptom free.
7. Electron microscopic study
revealed in the desmoplastic
component, elongated cells
with a lobulated nucleus and
an abundant cytoplasm
containing intermediate
filaments. The cytoplasmic
membrane and
the cell processes were
partially covered by a basal
lamina, that was often
adjacent to collagen fibrils.
In the cortical cell
component, neuronal cells
with dense-core granules and
synapses were identified.
8. DNIG can be located in parietal, temporal or occipital lobes but no frontal location has been reported
contrary to DIG. Neuro-imaging of DIG and DNIG is similar. Suggesting features are (i) massive superficial
heterogeneous tumor with solid and cystic portions, (ii) iso- or slightly hyperattenuating solid portion with
focal enhancement after contrast injection and (iii) extension of enhancement to the leptomeninges.
9. DIG may have exceptionally an aggressive behavior. In two cases, on account of an unusual deep
location, a total resection could not be performed, and tumor progression occurred even after
complementary chemo- or radiotherapy.
De Munnynck et al. reported a DIG with an aggressive behavior in a 2-year-old girl.
Histologically, this tumor showed prominent anaplasia with a Ki67 labeling index of 45%.
Radiological evidence of malignancy was present with intracerebral and pial metastases.
In the cerebrospinal fluid, circulating malignant cells were found. Chemotherapy with
vincristine (0.15 mg/m2) and carboplatinum (550 mg/m2) was given. After one cycle of
chemotherapy, there was a definite decrease of all intracranial lesions and, to a lesser extent,
the spinal lesions. She received three additional courses of carboplatinum (200 mg/m2 for 24
for hours, days 1–4) and etoposide (100 mg/m2 for 24 hours, days 1–4), and one course of high-
dose carboplatinum (500 mg/m2 for 24 hours, days 1–4) and etoposide (200 mg/m2 for 24 hours,
days 1–4) with autologous stem cell rescue. Because of very slow platelet recovery, a second
high-dose course was not given. Further tumor shrinkage was observed on the following MR
scan.
She remained stable on a maintenance chemotherapy of vincristine (1.5 mg/m2) and cis-
platinum (80 mg/m2) each 4 weeks (6–8 months after surgery). Because of hearing loss and
renal tubular dysfunction, cessation of therapy was mandatory. Because of tumor progression 11
months after surgery, an attempt with tamoxifen (120 mg/m2) was started, yet the patient died
after six weeks.
10.
11.
12. Postoperative course was not eventful. No adjuvant
treatment was given. 18 months after surgery, the patient was symptom free.
13.
14.
15.
16.
17. In all Grade I tumors radiotherapy is not indicated. However,
in this child therapy in the form of 54 Gy was administered,
since only a part of the tumor was removed. A few months
later the child presented with seizures which were well
controlled with antiepileptic drugs. The patient showed no
neurological abnormality and the visual evoked responses
were unremarkable.
Repeat imaging revealed a large heterogeneous cystic mass
with solid enhancing components in the left temporoparietal
region and ventricular trigone.
18.
19. Deaply Seated Desmoplastic Gangliogliomas Have Poorer
Prognosis
Proliferation rates in DIGs are usually low with reported Ki-67 labeling indices ranging between
0.5 and 5%. However DIGs with overt anaplastic and malignant histological features, including
necrosis with pseudopalisading of tumor cells, vascular proliferation, high mitotic rate and
high Ki-67 labeling indices have also been described.
It is of interest that the outcome of these cases seems to be dependent on the location of the
tumor. Thus the two anaplastic DIG cases with superficially located tumors reported by
Kuchelmeister et al did not show recurrence after gross complete removal without further
adjuvant therapy.
In contrast, the unusual deeply seated anaplastic DIG case reported by De Munnynck et al,
presenting with intracerebral and pial metastases, showed an aggressive behavior after
incomplete resection and adjuvant chemotherapy. A DIG case with deep tumor location but no
anaplastic features was also reported, which finally led to death after incomplete resection
and adjuvant chemotherapy.
20.
21.
22. Additional chemotherapy cycles were administered. These repetitive
cycles of high-dose chemotherapy included treatment with
cyclophosphamide, cisplatin, etoposide, vincristine, and thiotepa,
and peripheral blood stem cell rescue (according to protocol
Children Cancer Group 99703).
MR imaging showed a promising response to chemotherapy. Just
after concluding the chemotherapy with peripheral stem cell
reinfusion, the patient died of sepsis at the age of 16 months.
23.
24.
25.
26.
27. PHH3 highlighted mitotic figures (25 mitoses/10 HPFs) and a
Ki-67 (MIB-1) labeling index was 21%. Gross total resection
of the tumor and overlying dura were achieved.
Sixmonth follow-up MRI showed no evidence of recurrence.
The patient was able to return to work.
A high Ki-67 index, the presence of necrosis, endothelial
vascular proliferation, and increased mitosis are not
associated with poorer outcome.
39. We describe a 9-year-old child with a cerebellar
desmoplastic ganglioglioma with co-existent congenital
anomalies including macrocephaly, an arteriovenous
malformation (AVM) of the right clavicle and a large axillary
hyperpigmented area suggestive of a port wine stain (PWS).
This is the first report of a non-infantile patient with a solid
desmoplastic ganglioglioma in the posterior fossa.
