Giant cell glioblastoma in childhood-Pathohistological and immunohistochemical analysis, forecast and complex treatment

A six year old child is presented with giant cell glioblastoma multiforme. The importance of pathohistological and immunohistochemical analysis is discussed for the diagnosis of this rare pathohistological subtype glioblastoma in childhood. The Magnetic Resonance Image Characteristics, unfavorable prognosis and high cancer cell resistance to radiotherapy (RT) and chemotherapy (Ch) are also highlighted. The risk of local recurrences and tumor progression is high, despite the complex treatment, including visibly total tumor surgery, postoperative RT and adjuvant Ch. By this pediatric clinical case of childhood giant cell glioblastoma multiforme, we emphasize the emerging need to optimize early diagnostics and the multidisciplinary healing approach.

Primary Intracranial Leiomyosarcoma Secondary to Glioblastoma: Case Report and Literature Review

BackgroundLeiomyosarcoma is a highly malignant soft-tissue sarcoma with a poor prognosis. In recent years, treatment for leiomyosarcoma has not shown much progress. Primary intracranial leiomyosarcoma (PILMS) is a much rarer type of neoplasm, which occurs more frequently in immunocompromised patients. PILMS cases reported in the literature are scarce and treatment strategy and prognosis are still under debate. In this study, a case of PILMS secondary to the total resection of giant cell glioblastoma is reported.Case DescriptionA 38-year-old male was hospitalized with a three-month history of a temporal opisthotic bump. His medical history included a total resection of a tumor located in the right temporal lobe performed 4 years earlier. Pathological examination led to a diagnosis of giant cell glioblastoma, and the patient underwent postoperative chemotherapy with temozolomide for 6 weeks plus simultaneous radiotherapy with 63.66 Gary. Four years later, during regular follow-up, a preoperative MRI brain scan resulted in a well-defined signal pointing out two nodule-like features located at the right temporal lobe and subcutaneous soft tissue, respectively, and near the area where the previous giant cell glioblastoma was located. The mass was completely removed by a transtemporal approach and postoperative pathology revealed that the mass was a leiomyosarcoma. The patient underwent postoperative radiotherapy and no recurrence occurred until now.ConclusionsTo date, research on soft-tissue sarcoma, especially PILMS, has not made much progress, and a limited number of studies have provided few details on the management of PILMS. The treatment of choice for PILMS is aggressive multimodal treatment based on total tumor resection and radiotherapy. Moreover, systemic treatment with chemotherapy and targeted therapy, such as olaratumab, as well as further research still needs to be performed as many questions are left unanswered. To our knowledge, this is the first report on a case of PILMS secondary to glioblastoma, which might serve as a potential reference for clinicians and clinical studies.

Giant Cell Variant of Glioblastoma Multiforme: Report of a Rare Variant in a Child

Malignant glial tumors are rare in children. Giant cell variant is a rare subtype of glioblastoma, accounting for about 0.8% of brain tumors and 5% of glioblastoma tumors. Giant cell glioblastoma is a male predominant tumor in children and adults. Due to the low prevalence of this variant, available information is limited. An 11-year-old female child was referred with a chief complaint of a progressive persistent headache. MRI showed a well-defined cystic lesion with a solid mural component in the right parietal lobe with a compression effect on the ipsilateral ventricular system. Surgery was done. After the pathologist reported glioblastoma multiforme, a giant cell variant, the patient received 30 sessions of radiation therapy. The patient was readmitted 18 months later with a headache, and the pathologist confirmed the recurrence of the tumor. Based on radiology, the giant cell glioblastoma cannot be distinguished from the common subtype glioblastoma. The pathologists must be aware of this entity, and histologic differential diagnoses are warranted for diagnostic, prognostic, and therapeutic purposes.

Near haploidization is a genomic hallmark which defines a molecular subgroup of giant cell glioblastoma

Background Giant cell glioblastoma (gcGBM) is a rare histologic subtype of glioblastoma characterized by numerous bizarre multinucleate giant cells and increased reticulin deposition. Compared with conventional isocitrate dehydrogenase (IDH)-wildtype glioblastomas, gcGBMs typically occur in younger patients and are generally associated with an improved prognosis. Although prior studies of gcGBMs have shown enrichment of genetic events, such as TP53 alterations, no defining aberrations have been identified. The aim of this study was to evaluate the genomic profile of gcGBMs to facilitate more accurate diagnosis and prognostication for this entity. Methods Through a multi-institutional collaborative effort, we characterized 10 gcGBMs by chromosome studies, single nucleotide polymorphism microarray analysis, and targeted next-generation sequencing. These tumors were subsequently compared to the genomic and epigenomic profile of glioblastomas described in The Cancer Genome Atlas (TCGA) dataset. Results Our analysis identified a specific pattern of genome-wide massive loss of heterozygosity (LOH) driven by near haploidization in a subset of glioblastomas with giant cell histology. We compared the genomic signature of these tumors against that of all glioblastomas in the TCGA dataset (n=367) and confirmed that our cohort of gcGBMs demonstrated a significantly different genomic profile. Integrated genomic and histologic review of the TCGA cohort identified three additional gcGBMs with a near haploid genomic profile. Conclusions Massive LOH driven by haploidization represents a defining molecular hallmark of a subtype of gcGBM. This unusual mechanism of tumorigenesis provides a diagnostic genomic hallmark to evaluate in future cases, may explain reported differences in survival, and suggests new therapeutic vulnerabilities.