Giant cell glioblastoma: review of the literature and illustrated case

Abstract

SummaryGiant cell glioblastoma is an infrequent variety of glioblastoma (5% of the cases). It has deserved a separate category in the World Health Organization classification of grade IV tumors. The clinical, imaging, histological and immunohistochemical characteristics, and the genetic alterations are reviewed. Treatment and prognosis are discussed and updated. The case of a patient that survived 19 months and died of spinal leptomeningeal metastases is illustrated.KEYWORDS: Giant cell glioblastoma. Leptomeningeal metastases. Surgery. Temozolomide. Radiotherapy.Glioblastoma de células gigantes: revisión de la literatura e ilustración de un caso Resumen El glioblastoma de células gigantes es una variante rara dentro de los glioblastomas (5% de los casos). En la clasificación de la Organización Mundial de la Salud esta entidad ha merecido una categoría aparte en los tumores de grado IV. Se revisan las características clínicas, radiológicas, histológicas, inmunohistoquímicas y las alteraciones genéticas que caracterizan este tumor. El tratamiento y el pronóstico son discutidos aportando información actualizada. Finalmente se presenta un caso clínico ilustrado, en el cual el paciente sobrevivió durante 19 meses, falleciendo como consecuencia de una diseminación espinal leptomeníngea.PALABRAS CLAVE: Glioblastoma de células gigantes. Metástasis leptomeníngeas. Cirugía. Temozolomida. Radioterapia IntroductionUnlike the "common" glioblastoma, giant cell glioblastoma is not encountered in everyday practice and it probably comes as an unexpected diagnosis most of the times. Despite certain similarities between the two disease processes, there are differences that may be of relevance for the management of the occasional patient harboring this lesion. A case of giant cell glioblastoma managed at the authors' institution is reported and the key features of giant cell glioblastoma are reviewed. Clinical caseA 54 year-old man presented with headache and motor dysphasia of short duration. MR disclosed a voluminous mass in the left temporal lobe (figure 1-A&B). The patient was operated. A frontotemporal approach was employed. The lesion had a good cleavage plane, was moderately hemorrhagic and was totally resected in a piece meal fashion. The postoperative course was uneventful. Histological examination disclosed a highly cellular neoplasm with marked pleomorphism. Prominent giant cells and numerous atypical mitotic figures were found (figure 1-C). Reticulin was abundant in the stroma. Immunohistochemistry showed positivity for GFAP, S100 protein and p53. The picture was consistent with giant cell glioblastoma. Adjuvant radiotherapy (60Gy) and chemotherapy (temozolomide) were given. There was no evidence of relapse at the 14 month follow up MR ( figure 1-D). The patient was symptom free and had no neurological deficits at that time. Three months after, the patient complained of backache, lower limb weakness and deteriorating general condition. MR disclosed spinal leptomeningeal metastases (figure 1-E). Curiously there was no ev...

Cerebellar giant cell glioblastoma multiforme in an adult

“…[1] Considering the different treatment modalities available, it is very important to make a proper differential diagnosis between infratemporal GBM and metastasis, hemangioblastoma, and also cerebellar infarct with contrast enhancement. [1,5,6] Considering malignant glial tumors, it would be always reasonable to attempt a gross-total tumor resection; however, the infiltrative nature of the GBM makes it difficult to achieve it many times. The use of radiation therapy after radical surgical resection is a well-established modality of treatment.…”

Aggressive giant cell glioblastoma with negative p53 expression: case report

“…1 Other factor leading to a more complete resection includes fibrous stroma that may contribute to a better cleavage plan. 2 Another factor that may influence prognosis is the frequently seen tumor infiltration by leukocytes, which may be indicative of an augmented immune response. 2 However, young age and extension of resection do not entirely explain the longer survival in this patient population.…”

“…2 Another factor that may influence prognosis is the frequently seen tumor infiltration by leukocytes, which may be indicative of an augmented immune response. 2 However, young age and extension of resection do not entirely explain the longer survival in this patient population. Tumor genetics and histology are important factors for prognosis and may explain the divergent clinical course of GCG and GBM.…”

“…1,3,8,10,12 Its positivity on immunohistochemistry is a criteria recommended for GCG diagnosis 2,3,7 and helps to differentiate these tumors from pleomorphic xanthoastrocytoma (PXA), a benign entity with some shared pathology features, plus p53 negative tumors. 2 Low rates of epidermal growth factor receptor (EGFR) amplification (~5% against 30%-40% of GBM) are another characteristic feature of GCG. 3,13 The current treatment strategy for GCG is similar to GBM.…”

“…Giant cell glioblastoma (GCG) accounts for 0.8% of brain tumors 1,2 and has distinguished pathological and clinical features from traditional glioblastomas (GBM) 2 including higher incidence of p53 mutations compared with GBM. 2,3 We present a case of an older patient that was diagnosed with GCG without p53 gene expression and had unusually rapid disease progression.…”