Current treatment guidelines for the management of recurrent glioblastoma (rGBM) are far from definitive, and the prognosis remains dismal. Despite recent advancements in the pharmacological and surgical fields, numerous doubts persist concerning the optimal strategy that clinicians should adopt for patients who fail the first lines of treatment and present signs of progressive disease. With most recurrences being located within the margins of the previously resected lesion, a comprehensive molecular and genetic profiling of rGBM revealed substantial differences compared with newly diagnosed disease. In the present comprehensive review, we sought to examine the current treatment guidelines and the new perspectives that polarize the field of neuro-oncology, strictly focusing on progressive disease. For this purpose, updated PRISMA guidelines were followed to search for pivotal studies and clinical trials published in the last five years. A total of 125 articles discussing locoregional management, radiotherapy, chemotherapy, and immunotherapy strategies were included in our analysis, and salient findings were critically summarized. In addition, an in-depth description of the molecular profile of rGBM and its distinctive characteristics is provided. Finally, we integrate the above-mentioned evidence with the current guidelines published by international societies, including AANS/CNS, EANO, AIOM, and NCCN.
Background:
Spinal hemangioblastoma (HB) is a highly vascularized tumor commonly presenting in the lower thoracic and lumbar segments. It typically causes spinal compression, extensive bleeding, and/or syringomyelia.
Case Description:
A 32-year-old female presented with persistent headaches with a cervical MRI showing an intradural and extradural mass extending from the obex to C2. Following surgical tumor resection, the patient’s symptoms resolved.
Conclusion:
Resection of spinal HB requires direct removal of the tumor mass as the accompanying cystic components typically spontaneously regress.
Background:
Subdural osteomas represent an extremely rare entity with only 20 cases described to date. Despite the typical benign behavior, these tumors can grow to compress the brain and occasionally detach from the dura mater.
Methods:
A systematic search of the literature was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. After screening for duplicates, 179 publications met the eligibility criteria. Finally, 18 manuscripts were included in this review. Moreover, a detailed description of an illustrative case is provided.
Results:
The median age at diagnosis was 43.2 years, showing a female prevalence. The inner table of the frontal bone was reported as the most frequent location, and in six cases, the lesions did not show any relation with the dura, which appeared intact. Surgical resection appears to be an effective and safe management option. In the present work, the case of a 60-year-old female who presented with persistent, diffuse headaches which had first occurred 6 months earlier is described. On admission, the physical and neurological examinations were unremarkable, and her medical history disclosed no systemic disease, meningitis, or head injury. Computed tomography showed a homogeneous, high-density nodule attached to the inner table of the left middle cranial fossa.
Conclusion:
In addition to an in-depth case description, the first systematic and qualitative review of the literature on intracranial subdural osteomas using the PRISMA is provided.
Background:
Metastatic renal cell carcinoma (RCC) of the choroid plexus is an exceedingly rare condition, with only 35 reported cases to date. Surgical resection of these tumors poses a unique challenge to neurosurgeons since evidence-based treatment guidelines are yet to be designed.
Case Description:
The authors describe the case of a 58-year-old woman presenting with progressive neurological deterioration 5 years after a right nephrectomy for a WHO 2016 Stage I RCC. A head, contrast-enhanced, and magnetic resonance revealed signs of obstructive hydrocephalus and a homogeneously contrast-enhancing 5 cm mass located in the trigone of the right lateral ventricle. Furthermore, a search of the literature was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. After screening for duplicates, 35 publications met the eligibility criteria. Finally, 17 manuscripts were included for analysis. Moreover, a detailed description of an illustrative case is provided. The median age at diagnosis for intraventricular metastasis from RCC was 62.9 years, showing a slight female prevalence. The lateral ventricles were reported as the most frequent location with only one patient presenting with obstructive hydrocephalus caused by the obliteration of Monro foramen. Management options included either open craniotomy or radiosurgery.
Conclusion:
The management of choroid plexus metastasis from RCC is still controversial with various authors proposing different treatment strategies. In this article, in addition to an in-depth case description, a qualitative review of the literature on metastatic RCCs of the choroid plexus using the PRISMA is provided.
Background:
In the WHO 2016 classification of central nervous system tumors, solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) were considered part of the same category given a shared mutation. Nevertheless, since the new 2021 WHO classification, the term “hemangiopericytoma” has been retired, and SFT is considered an independent pathological entity.
Methods:
We reviewed the literature following preferred reporting items for systematic reviews and meta-analyses guidelines focusing on the treatment options and prognosis of patients with cervical SFT. We also present a 68-year-old female with spinal intradural extramedullary SFT complicated by diffuse extension into paravertebral tissues and muscles.
Results:
We found 38 cervical SFT in the literature. Patients averaged 47.3 years of age and 47.4% were female. Typically, these lesions spanned two spinal levels resulting in cord compression and most frequently exhibited benign features (i.e., diagnosed as Grade I SFTs). Interestingly, two patients exhibited distant metastases and had initial pathology consistent with grade II SFT.
Conclusion:
SFT of the cervical spine is rare and its management varies according to the histological grade and the clinical behavior, generally warranting surgical excision and adjuvant radiation therapy and/or systemic chemotherapy.
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