Malignant glioblastomatous transformation of a low-grade glioma in a child
Department of Pediatrics, Meram Faculty of Medicine, Selcuk University, 42080, Meram, Konya, Turkey. Child s Nervous System
(Impact Factor: 1.11).
11/2008; 24(12):1385-9. DOI: 10.1007/s00381-008-0716-3
The term of low-grade glioma addresses a favorable clinical outcome with indolent histological features in general consideration; however, recent studies underline the inconsistency, which originates from the accumulation of different histologic subtypes in this terminology. The malignant transformation of a low-grade glioma is unusual but presents a poor prognosis.
We report a case of a 12-year-old boy, who was referred for complaints of recurrent seizures. His physical examination was unremarkable, but it was learned that a peripheral mass lesion located on the left posterior parietal lobe--which had been thought to be a low-grade glioma--had been detected on a magnetic resonance imaging 2 years ago at a different hospital. The patient was then treated with valproate and carbamazepine for the seizures and advised to be followed up without any additional diagnostic and therapeutic studies for his suspected low-grade glioma. A recent magnetic resonance imaging study showed enlargements of the mass and surrounding edema with additional necrosis. Surgical excision of the tumor was performed. After the diagnosis of glioblastoma multiforme, the patient received radiation therapy and chemotherapy with a good clinical recovery without any evidence of residue or recurrence at 12-month follow-up.
The first line treatment modality in the management of low-grade glioma--especially in suitable patients--is clearly surgery. The gross total resection guarantees the distinguishing of the histological types of the low-grade gliomas and reflects the biologic behavior of these tumors. Observation without surgery must be reserved for selected inoperable cases.
Available from: Prajwal Rajappa
- "Treatment of pediatric gliomas is subject to a different set of considerations and standards as toxicity of therapy has a greater impact on the developing nervous and skeletal system. Surgery with GTR is the primary mode of therapy as this has been shown to be the most effective method for cure (Fisher et al., 2008, Unal et al., 2008). While rare, malignant transformation does occur so observation is not recommended with lesions that are amenable to surgery. "
Glioma - Exploring Its Biology and Practical Relevance, 11/2011; , ISBN: 978-953-307-379-8
Available from: Engin Gunel
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ABSTRACT: Malignant transformation of a germ cell tumor, referring to the occurrence of somatic, nongerm cell malignancy within a germ cell tumor is a rare entity. The authors report a case of malignant transformation of an unreseceted sacrococcygeal teratoma to neuroblastoma in a 6-year-old girl and discuss this case in the light of relevant literature. This case shows that complete resection of germ cell tumor is necessary to prevent malignant transformation. In addition, when malignant transformation occurs, the treatment strategy must include surgery together with histology adapted systemic chemotherapy.
Journal of Pediatric Hematology/Oncology 04/2010; 32(3):233-5. DOI:10.1097/MPH.0b013e3181d4181c · 0.90 Impact Factor
Available from: Ioannis Magras
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ABSTRACT: Pilomyxoid astrocytoma (PMA) is a recently described glial tumor with similarities to pilocytic astrocytomas, yet with distinct histopathological characteristics and a more aggressive behavior. It occurs predominantly in the hypothalamic/chiasmatic region. Only four patients with spinal cord PMA have been reported in the pediatric population. The 2007 WHO Working Group recognized PMA as a new variant and recommended an assignment to WHO grade II.
The purpose of this paper was to report a rare location, address the aggressive behavior and rapid progression, and based on the specific patient, to review the literature and discuss current treatment strategies.
A 12-year-old girl presented with motor and sensory deficits of the left side as well as gait disturbance. Imaging revealed an intramedullary tumor extending from C2 to C7. The patient improved impressively after surgical resection. Histopathological findings were consistent with PMA. Three months later, the patient presented with rapid neurological deterioration. Histopathology after the second operation was consistent with glioblastoma. The outcome was fatal 12 months after initial diagnosis, despite adjuvant therapy.
This is the fifth pediatric spinal cord PMA in literature. Furthermore, it is the only documented patient with rapid recurrence and progression within 3 months into a glioblastoma. The question of a sampling error affecting initial pathology is raised. Based on contemporary literature data, we discuss the further treatment options, as there are no guidelines yet. Efforts towards registries should be encouraged, as the documentation of PMA might lead to more evidence based treatment strategies.
Child s Nervous System 05/2010; 27(2):313-21. DOI:10.1007/s00381-010-1171-5 · 1.11 Impact Factor
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