scholarly journals Complex Form Variant of Dysembryoplastic Neuroepithelial Tumor of the Cerebellum

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Jesús Vaquero ◽  
Cristobal Saldaña ◽  
Santiago Coca ◽  
Mercedes Zurita
Keyword(s):  
Pilocytic Astrocytoma ◽  
Complex Form ◽  
Benign Neoplasm ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Cerebellar Tumor ◽  
Pathological Study ◽  
Contrast Administration ◽  
Light Enhancement ◽  
Gadolinium Administration ◽  
Form Variant

Dysembryoplastic neuroepithelial tumor (DNT) is a benign neoplasm with typical supratentorial location, but the possibility of these rare tumors can also be located in the posterior fossa must be taken into account. We report a 21-year-old woman that suffered gait instability, headache, and diplopia. On CT-scan, an intraparenchymatous cerebellar tumor was disclosed. It was isodense, showing light enhancement after contrast administration. On MRI (T1-weighted image) the tumor was isointense, showing inhomogeneous hyperintensity after-gadolinium administration. On T2-weighted MRI, the tumor was inhomogenously hyperintense. At surgery, a solid and hypervascularized tumor was completely removed. Two years after surgery, the patient is symptom-free. Pathological study showed coexistence of areas of pilocytic astrocytoma with areas in which small rounded oligodendrocyte-like cells (OLC), with strong synaptophysin expression were identified. These neurocytic areas showed an eosinophilic matrix forming microcysts, and cells with aspect of “floating neurons” were occasionally identified. A complex form variant of DNT was diagnosed. Our case suggests that in presence of a cerebellar tumor with features of pilocytic astrocytoma, the possibility of a complex form variant of DNT should be considered.

Complex Dysembryoplastic Neuroepithelial Tumor With Pilocytic Astrocytoma Component

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S44-S45
Author(s):  
Sandhyarani Dasaraju ◽  
Krutika Patel ◽  
Shalla Akbar ◽  
Osama Elkadi
Keyword(s):  
Contrast Enhancement ◽  
Pilocytic Astrocytoma ◽  
Early Recognition ◽  
Complex Form ◽  
Surgical Excision ◽  
Biological Behavior ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Who Grade ◽  
Complete Surgical Excision

Abstract Objectives Dysembryoplastic neuroepithelial tumor (DNET) is an uncommon mixed glioneuronal tumor. DNET is classified as WHO grade I neoplasm and commonly seen in the temporal lobe of children and young adults with features of pharmacoresistant seizures. The term complex DNET defines those tumors associated with additional features, including a pilocytic astrocytoma (PA) component, a finding that is rarely described in the literature. Methods Here we report a 16-year-old male who presented with seizures. On imaging, a 5.5 × 2.2 × 2.1-cm multiloculated mass was centered in the left frontoparietal gray matter with solid and cystic components and focal areas of contrast enhancement. Results Microscopic examination revealed a noninfiltrative glioneuronal neoplasm composed of a bland population of round to oval cells arranged around pools of mucinous material. Occasional large neuronal cells were noted. Focal areas composed of elongated astrocytic cells demonstrating piloid morphology were also present. No high-grade features were demonstrated. The findings are consistent with DNET, complex form in addition to a second component with piloid features resembling pilocytic astrocytoma. Conclusion The early recognition of DNET is important as it has an excellent prognosis on complete surgical excision without use of adjunct therapies. Unresected or incompletely removed DNET and secondary radiochemotherapy may give rise to recurrence and malignancy. The development of the secondary neoplasm challenges the stability in terms of biological behavior and highlights the importance of complete surgical excision and close follow-up. Our patient had neither undergone previous surgery nor received any adjuvant therapy, and hence this case is a de novo development of PA in a DNET. The association of contrast enhancement with DNET is an unusual finding in this case.

Malignant transformation of a dysembryoplastic neuroepithelial tumor

2000 ◽  
Vol 92 (4) ◽  
pp. 722-725 ◽  
Author(s):  
Robert R. Hammond ◽  
Neil Duggal ◽  
John M. J. Woulfe ◽  
John P. Girvin
Keyword(s):  
Malignant Transformation ◽  
Complex Form ◽  
Ct Scanning ◽  
Subtotal Resection ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Content Type ◽  
Recent Onset ◽  
Fibrillary Astrocytoma ◽  
Fine Print ◽  
Glial Nodules

✓ A 29-year-old man presented in 1984 with a recent onset of partial seizures marked by speech arrest. Electroencephalography identified a left frontotemporal dysrhythmia. Computerized tomography (CT) scanning revealed a superficial hypodense nonenhancing lesion in the midleft frontal convexity, with some remodeling of the overlying skull. The patient was transferred to the London Health Sciences Centre for subtotal resection of what was diagnosed as a “fibrillary astrocytoma (microcystic).” He received no chemotherapy or radiation therapy and remained well for 11 years.The patient presented again in late 1995 with progressive seizure activity. Both CT and magnetic resonance imaging demonstrated a recurrent enhancing partly cystic lesion. A Grade IV astrocytoma was resected, and within the malignant tumor was a superficial area reminiscent of a dysembryoplastic neuroepithelial tumor (DNT). Data on the lesion that had been resected in 1984 were reviewed, and in retrospect the lesion was identified as a DNT of the complex form. It was bordered by cortical dysplasia and contained glial nodules, in addition to the specific glioneuronal element. The glial nodules were significant for moderate pleomorphism and rare mitotic figures. The Ki67 labeling index averaged 0.3% in the glial nodules and up to 4% focally. Cells were rarely Ki67 positive within the glioneuronal component. This case is the first documented example of malignant transformation of a DNT. It serves as a warning of the potential for malignant transformation in this entity, which has been traditionally accepted as benign. This warning may be especially warranted when confronted with complex forms of DNT. The completeness of resection in the benign state is of paramount importance.

RAPIDLY GROWING DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR

Neurosurgery ◽  
2006 ◽  
Vol 59 (6) ◽  
pp. E1337-E1338 ◽  
Author(s):  
Oltea Sampetrean ◽  
Taketoshi Maehara ◽  
Nobutaka Arai ◽  
Tetsuo Nemoto
Keyword(s):  
Complex Form ◽  
Interesting Case ◽  
Labeling Index ◽  
Pathological Examination ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Complex Partial Seizures ◽  
Ki 67 ◽  
The Past ◽  
Short Period ◽  
Postoperative Diagnosis

Abstract OBJECTIVE During the past 15 years, the concept of dysembryoplastic neuroepithelial tumors has continued to evolve. We present an interesting case of dysembryoplastic neuroepithelial tumor that showed rapid growth during a short period of time. CLINICAL PRESENTATION A 9-year-old boy had been experiencing intractable complex partial seizures since the age of 7 years. Magnetic resonance imaging scans demonstrated a well-demarcated 3.5-cm lesion with a 1.5-cm ring-enhanced core in the left temporal lobe. One month later, the lesion had rapidly grown to occupy three times more space than on the first evaluation, with the ring-enhanced core reaching approximately five times its initial volume. INTERVENTION A combined tumor removal and epileptogenic focus resection surgery was performed immediately. In the pathological examination, the presence of the specific glioneuronal element with a Ki-67 labeling index of lower than 1%, as well as the glial component with a Ki-67 labeling index of 8%, led to a postoperative diagnosis of dysembryoplastic neuroepithelial tumor, complex form. No adjuvant therapy was performed. Five years after surgery, there is no evidence of any recurrence and the boy continues to be seizure free without antiepileptic drugs. CONCLUSION The lesion did not behave as a stable benign entity as it is generally accepted, and is, therefore, presented as an argument in favor of an early and complete resection.

scholarly journals Clinical characteristics and surgical outcome of patients with temporal lobe tumors and epilepsy

2000 ◽  
Vol 58 (4) ◽  
pp. 1002-1008 ◽  
Author(s):  
CARMEN LISA JORGE ◽  
SUELY K. NAGAHASHI-MARIE ◽  
CHRISTIANE C. PEDREIRA ◽  
SÉRGIO ROSEMBERG ◽  
ROSA M.F. VALÉRIO ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Temporal Lobe ◽  
Pilocytic Astrocytoma ◽  
Clinical Characteristics ◽  
Pathological Diagnosis ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Precise Location ◽  
Histological Characteristics ◽  
Mri Characteristics

This is a retrospective study of 21 surgically treated patients with temporal lobe tumors and epilepsy. Evaluation included clinical data, EEG findings, structural scans, pathological diagnosis and post-surgical follow-up. There were 9 cases of ganglioglioma, 5 pilocytic astrocytoma, 3 ganglioneuroma, 2 dysembryoplastic neuroepithelial tumor, 1 pleomorphic xantoastrocytoma, and 1 meningioangiomatosis. Mean follow-up time was 22 months and outcome was evaluated according to Engel's classification; 76.2% were classified in class I and 23.8% in II and III. All patients classes II and III had been submitted to mesial and neocortical resections. There were no differences related to clinical characteristics, pathological diagnosis or duration of follow-up in patients seizure-free or not. All patients had abnormal MRI and ten of these had normal CT; the MRI characteristics were compared to pathological diagnosis and specific histological characteristics of the tumors were not discernible by MRI. We concluded that MRI was essential for the diagnosis and precise location of TL tumors. Ganglioglioma was the most frequent tumor and lesionectomy associated to mesial resection doesn't guarantee a better prognosis.

scholarly journals Leptomeningeal dissemination of pilocytic astrocytoma via hematoma in a child

2002 ◽  
Vol 13 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Masaru Kanda ◽  
Hidenobu Tanaka ◽  
Soji Shinoda ◽  
Toshio Masuzawa
Keyword(s):  
Spinal Cord ◽  
Pilocytic Astrocytoma ◽  
Spinal Cord Tumor ◽  
Cerebellar Tumor ◽  
Leptomeningeal Dissemination ◽  
Original Tumor ◽  
Initial Surgery ◽  
Spinal Region ◽  
Lumbar Spinal ◽  
Mib 1

A case of recurrent pilocytic astrocytoma with leptomeningeal dissemination (LMD) is described. A cerebellar tumor was diagnosed in a 3-year-old boy, in whom resection was performed. When the boy was 6 years of age, recurrence was treated with surgery and local radiotherapy. At age 13 years, scoliosis was present, but the patient was asymptomatic. Twelve years after initial surgery LMD was demonstrated in the lumbar spinal region without recurrence of the original tumor. This tumor also was subtotally removed. During the procedure, a hematoma was observed adjacent to the tumor, but the border was clear. Histological examination of the spinal cord tumor showed features similar to those of the original tumor. There were no tumor cells in the hematoma. The MIB-1 labeling index indicated no malignant change compared with the previous samples. Radiotherapy was performed after the surgery. The importance of early diagnosis and management of scoliosis is emphasized, and the peculiar pattern of dissemination of the pilo-cytic astrocytoma and its treatment are reviewed.

Development of a pilocytic astrocytoma in a dysembryoplastic neuroepithelial tumor

2007 ◽  
Vol 106 (6) ◽  
pp. 509-512 ◽  
Author(s):  
Vivek Josan ◽  
Paul Smith ◽  
Andrew Kornberg ◽  
Christian Rickert ◽  
Wirginia Maixner
Keyword(s):  
Pilocytic Astrocytoma ◽  
Dysembryoplastic Neuroepithelial Tumor

Radical Resection of Cerebellar Juvenile Pilocytic Astrocytoma - A 22-Year Survival: A Case Report

2020 ◽  
Vol 13 (1) ◽  
pp. 59-62
Author(s):  
Iliya V. Valkov ◽  
Mladen E. Ovcharov ◽  
Milan N. Mladenovski ◽  
Nikolay V. Vasilev ◽  
Iliya I. Duhlenski
Keyword(s):  
Survival Rate ◽  
Pilocytic Astrocytoma ◽  
Right Hemisphere ◽  
Young Patients ◽  
Gross Total Resection ◽  
Low Grade ◽  
Radical Cure ◽  
Cerebellar Tumor ◽  
Total Resection ◽  
Juvenile Pilocytic Astrocytoma

Summary Juvenile pilocytic astrocytoma (JPA) is a low-grade glioma, a most common astrocytoma in young patients. It is a tumor with relatively well-defined margins. Pilocytic astrocytomas (PA) comprise approximately 5-6% of all gliomas. Gross total resection ensures a radical cure of patients and long-time survival. In the literature, the data on the survival rate of more than 20 years is scarce. A 5.5-year old boy with a history of 3-month complaints of headache, dizziness, and vomiting was diagnosed after CT to harbour a hypodense cerebellar tumor mass, situated in the midline-right hemisphere. The compression of the fourth ventricle resulted in rostral hydrocephalus with transependymal resorption. Within a week, a VP shunt was applied, followed by a radical Nafziger-Town operation. Gross total resection of the tumor was achieved. Profound clinical improvement was observed immediately after the operation. Postoperative CT scans, including the ones 22 years after the operation, remained practically normal. The patient is now 28-year old and is a perspective economist now. He leads a healthy working life. In general, the prognosis is excellent. If the tumor is completely removed by surgery, the chances of being “cured” are very high. Pilocytic astrocytoma has a five-year survival rate in over 96 percent in children and young adults, which is one of the highest survival rates of any brain tumor. However, there is even a small percent possibility for malignant transformation (1-4%).

Metachronous, multicentric glioma of pilocytic astrocytoma with oligodendroglioma-like component and oligodendroglioma through distinct genetic aberrations

2013 ◽  
Vol 118 (4) ◽  
pp. 854-858 ◽  
Author(s):  
Atsushi Kanoke ◽  
Masayuki Kanamori ◽  
Toshihiro Kumabe ◽  
Ryuta Saito ◽  
Mika Watanabe ◽  
...  
Keyword(s):  
Pilocytic Astrocytoma ◽  
De Novo ◽  
Histological Diagnosis ◽  
Gross Total Resection ◽  
Cerebellar Tumor ◽  
Total Resection ◽  
Different Types ◽  
Multicentric Gliomas ◽  
The Right ◽  
R132h Mutation

This patient presented with a rare case of metachronous, multicentric gliomas first manifesting as headache and nausea in 1983 when he was an 8-year-old boy. Computed tomography revealed a cerebellar tumor and the tumor was subtotally resected. The histological diagnosis was pilocytic astrocytoma, and radiation therapy to the posterior fossa and chemotherapy consisting of nimustine hydrochloride and fluorouracil were performed. In 1989, at age 14 years, the patient presented with local recurrence. He underwent gross-total resection of the tumor, and histological examination revealed that the tumor consisted of classic pilocytic astrocytoma with a biphasic pattern and a small oligodendroglioma-like component. In 2011, at age 36 years, he presented with seizure. Magnetic resonance imaging revealed a mass lesion in the right middle frontal gyrus. Gross-total resection of the tumor was performed, and the histological diagnosis was oligodendroglioma. Genetic analyses revealed amplification of the BRAF gene in both the primary cerebellar pilocytic astrocytoma and the recurrent tumor with biphasic features, as well as a BRAF V600E missense mutation in the oligodendroglioma-like component. On the other hand, the IDH1 R132H mutation, instead of aberrations of the BRAF gene, was identified in the oligodendroglioma arising in the right frontal lobe. Different types of aberrations of the BRAF gene in the classic and oligodendroglioma-like component in the recurrent pilocytic astrocytoma suggest that they had different cell origins or that amplification of BRAF was negatively selected under the de novo BRAF V600E mutation. In addition, the aberration profiles of IDH1 and BRAF suggest that the oligodendroglioma arose independent of cerebellar pilocytic astrocytoma.

Extensive cerebrospinal fluid enhancement following gadolinium chelate administration: possible pathogenesis

2005 ◽  
Vol 46 (5) ◽  
pp. 523-527 ◽  
Author(s):  
H.-L. Hsu ◽  
C.-J. Chen
Keyword(s):  
Cerebrospinal Fluid ◽  
Renal Clearance ◽  
Pulse Sequences ◽  
Contributing Factors ◽  
Contrast Administration ◽  
Delayed Imaging ◽  
Barrier Disruption ◽  
Gadolinium Chelate ◽  
Gadolinium Administration

Diffuse cerebrospinal fluid (CSF) enhancement following gadolinium administration is a rarely recognized phenomenon, and its mechanism is not fully understood. We report two cases of diffuse CSF enhancement following gadolinium administration and review the literature. We conclude that the contributing factors of this phenomenon include blood-CSF barrier disruption, increased dosage, impair renal clearance, delayed imaging after contrast administration, and use of different pulse sequences.

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