Hypothalamic Hemangioma-like Pilocytic Astrocytoma in an Adult Patient: A Systematic Review with a Focus on Differential Diagnosis and Neurological Presentation.

Background: Pilocytic astrocytoma (PCA) are commonly observed as slow-growing noncancerous brain tumors in pediatric populations, but they can also occur in adults, albeit rarely. When located in diencephalic regions, particularly in the hypothalamus, they present unique diagnostic and management challenges due to their rarity and overlapping clinical and radiological features with other intracranial pathologies. This systematic review aims to provide a comprehensive understanding of hypothalamic PCA in adults, focusing on their differential diagnosis, neurological presentation, diagnostic modalities, treatment strategies. A case illustration is also described in order to better underline all the difficulties related to the diagnostic process. Material and methods: A systematic literature search was conducted in the PubMed/MEDLINE, Embase, and Scopus databases up to November 2023 to identify studies. Results: The systematic literature search identified a total of 214 articles. Following screening by title and abstract and full-text review, 12 studies were deemed eligible and are included here. Conclusions: Adult-onset PCA in diencephalic regions pose diagnostic challenges due to their rarity and overlapping features with other intracranial lesions. Advanced imaging techniques play a crucial role in diagnosis, while surgery remains the cornerstone of treatment. Multidisciplinary collaboration is essential for the optimal management and long-term follow-up of these patients.

"Single-step" resection and cranio-orbital reconstruction for spheno-orbital metastasis with custom made implant. A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Brain metastasis involving the skull base is a rare complication of malignant tumors. Besides radiotherapy, surgical treatment is a therapeutical option even though it may apply complex technical procedures that may delay complementary therapies. However, in recent days, the innovation of custom-made implants allows treating selected patients with fewer complications and better results. CASE PRESENTATION: We describe a single case of a complex fronto-orbital skull base metastasis requiring skull bone reconstruction that we treated with a single-step surgery and custom-made implant. Our procedure consists of two steps: in the first one, we perform a "virtual" craniotomy on a 3D phantom model previously built on a high-resolution bone CT scan. In the second step, the actual surgical procedure, the patient undergoes the resection and reconstruction of the cranial defect with an implant of PMMA custom-made cranioplasty. A three-month clinical and radiological follow-up is reported, which documented the extent of resection and good aesthetic results. CLINICAL DISCUSSION: In our case, we performed a skull reconstruction of cranio-orbital region with macroscopic gross-total resection of the tumor. Complementary radiotherapy was obtained after one month. Three-month follow-up showed good esthetic results and progression-free disease. A recent review of the literature is provided to discuss different reconstruction techniques. CONCLUSION: "Single-Step" resection and custom-made reconstruction is a relatively new technique that could be helpful not only for benign tumors, which remains its main application but also in selected cases of malignant tumors when immediate reconstruction and faster complementary treatments are needed.

Synchronized "One-Step" Resection and Cranio-Orbital Reconstruction for Spheno-Orbital Lesions With Custom Made Implant.

BACKGROUND: In the last few years, skull base tumors involving the fronto-orbital region have been approached with complex, invasive, and time-consuming cranial reconstruction techniques. On the other hand, recent custom-made implants allow easier and faster procedures, with excellent aesthetic results. The authors propose an easy surgical-planned protocol with a synchronized "one-step" resection and reconstruction of these complex lesions, with a preformed poly-methyilmethacrylate (PMMA) cranioplasty. METHODS: Our technique consists of a 2-phases procedure. In the first one, the so-called "virtual" phase, the authors get a tailored 3D-PMMA model based on high-resolution (HR) CT-scans of the patient's skull. Afterward, the authors perform the planned craniotomy on the 3D-PMMA model with the assistance of intraoperative navigation system. Therefore, a further CT-scan of the resected model is performed to develop the final implant. In the second phase, the "surgical phase," a single procedure consisting in a cranial resection and custom reconstruction is performed with the assistance of stealth-navigation. The authors describe 6 cases of complex fronto-orbital-sphenoidal benign tumors that required skull reconstruction. All patients were examined 3 months after surgery through cosmetic (facial and eyes symmetry and globe position) and ophthalmology tests (visual field, visual impairment, and diplopia). Postsurgical HR CT-scans of the head and MRI-scans of the brain documented a total resection of the tumor and an optimal accuracy of skull reconstruction. RESULTS: In all cases, the authors obtained a highly accurate skull reconstruction following cranio-orbital tumor resection, with a less aggressive and faster procedure compared to autologous bone graft. Final cosmetic and functional results were excellent, with good results in cases of presurgical exophthalmos and orbital asymmetry. None of the patients developed implant-related complications. CONCLUSIONS: The "one-step" resection and reconstruction of benign tumors involving the spheno-orbital region with neuronavigation assistance is a technique that allows an accurate tumor removal and a cranial-bone reconstruction within a single surgical procedure, with fewer complications and excellent cosmetic and functional results.

Contrasting microsatellite diversity in the evolutionary lineages of Phytophthora lateralis.

Following recent discovery of Phytophthora lateralis on native Chamaecyparis obtusa in Taiwan, four phenotypically distinct lineages were discriminated: the Taiwan J (TWJ) and Taiwan K (TWK) in Taiwan, the Pacific Northwest (PNW) in North America and Europe and the UK in west Scotland. Across the four lineages, we analysed 88 isolates from multiple sites for microsatellite diversity. Twenty-one multilocus genotypes (MLGs) were resolved with high levels of diversity of the TWK and PNW lineages. No alleles were shared between the PNW and the Taiwanese lineages. TWK was heterozygous at three loci, whereas TWJ isolates were homozygous apart from one isolate, which exhibited a unique allele also present in the TWK lineage. PNW lineage was heterozygous at three loci. The evidence suggests its origin may be a yet unknown Asian source. North American and European PNW isolates shared all their alleles and also a dominant MLG, consistent with a previous proposal that this lineage is a recent introduction into Europe from North America. The UK lineage was monomorphic and homozygous at all loci. It shared its alleles with the PNW and the TWJ and TWK lineages, hence a possible origin in a recent hybridisation event between a Taiwan lineage and PNW cannot be ruled out.

Differential accumulation of Phytophthora cambivora cox II gene transcripts in infected chestnut tissue.

This study provides a novel qRT-PCR protocol for specific detection and proof of viability of Phytophthora in environmental samples based on differential accumulation of cox II transcripts. Chemical and physical treatments were tested for their ability to induce in vitro the accumulation of cytochrome oxidase genes encoding subunits II (cox II) transcripts in Phytophthora cambivora. Glucose 170 mM, KNO3 0.25 mM and K3 PO3 0.5 and 0.8 mM induced the transcription of cox II in P. cambivora living mycelium while no transcription was observed in mycelium previously killed with 0.5% (p/v) RidomilGold(®) R WG. Living chestnut tissue was artificially infected with P. cambivora and treated with inducers. In vivo experiments confirmed the ability of glucose to induce the accumulation of P. cambivora cox II transcripts. Based on these results, pretreatment of environmental samples with glucose prior to nucleic acid extraction increased the accumulation of specific cox II transcripts, and therefore the sensitivity of qRT-PCR assay for detection of P. cambivora in living tissues. Furthermore, differential accumulation of transcripts between treated and untreated samples represents an unequivocal proof of inoculum viability.

Pyrosequencing of environmental soil samples reveals biodiversity of the Phytophthora resident community in chestnut forests.

Pyrosequencing analysis was performed on soils from Italian chestnut groves to evaluate the diversity of the resident Phytophthora community. Sequences analysed with a custom database discriminated 15 pathogenic Phytophthoras including species common to chestnut soils, while a total of nine species were detected with baiting. The two sites studied differed in Phytophthora diversity and the presence of specific taxa responded to specific ecological traits of the sites. Furthermore, some species not previously recorded were represented by a discrete number of reads; among these species, Phytophthora ramorum was detected at both sites. Pyrosequencing was demonstrated to be a very sensitive technique to describe the Phytophthora community in soil and was able to detect species not easy to be isolated from soil with standard baiting techniques. In particular, pyrosequencing is an highly efficient tool for investigating the colonization of new environments by alien species, and for ecological and adaptive studies coupled with biological detection methods. This study represents the first application of pyrosequencing for describing Phytophthoras in environmental soil samples.

Epilepsy in cerebral glioma: timing of appearance and histological correlations.

PURPOSE: A prospective study of patients with glioma was carried out. Special attention was paid to the first seizure, to the histology, and to the efficacy and prophylactic role of antiepileptic drugs (AEDs). Prognostic factors were analyzed. METHODS: Between February 1st 2004 and April 1st 2006 patients who underwent surgical treatment at the Neurosurgery Department of Bolzano for primary or recurrent glioma were prospectively followed until April 1st 2007. Seizures at onset occurring in the absence of interictal epileptiform abnormalities were regarded as remote symptomatic seizures and not treated. RESULTS: Sixty-four individuals were registered in the study; epilepsy diagnosis was made in 27 cases and in 24 of these seizures were the onset symptom. The correlation with histological grading showed that seizures were more frequent in low-grade glioma and in secondary glioblastoma. Although epilepsy was less frequent in high-grade glioma, in these patients seizures were more difficult to control. Poor seizure control was associated with motor and sensitive focal seizures and presence of neurological deficit. Patients without epilepsy and not taking AEDs never developed seizures during the follow-up. DISCUSSION: Our study clearly shows that epilepsy is more frequent in low-grade gliomas but seizures are more difficult to control in high-grade gliomas. In both cases seizures are a quite exclusive symptom at the onset that never appears during the stable course of the disease. Amongst glioblastoma multiforme (GBM), epilepsy is more frequent in GBM developing through progression from low-grade astrocitoma. Moreover, our study strongly indicates that the prophylactic use of AEDs in glioma is not justified.

Epilepsy in glioblastoma multiforme: correlation with glutamine synthetase levels.

PURPOSE: The hypothesis addressed by this study is that a glutamine synthetase (GS) deficiency in neoplastic astrocytes is a possible molecular basis associated with seizure generation in glioblastoma multiforme (GBM). METHODS: Quantitative Western blot analysis of GS was performed in 20 individuals operated for malignant glioma. RESULTS: The levels of GS in patients with GBM and epilepsy were significantly lower (range 0.04-1.15; mean 0.35 +/- 0.36; median 0.25) than in non-epileptic GBM individuals (range 0.78-3.97; mean 1.64 +/- 0.99; median 1.25; P = 0.002). No relationship has been found between histological features (i.e. necrosis, gliosis, stroma, inflammatory cells, giant cells, and haemosiderine) and GS expression or epilepsy. DISCUSSION: Even though the epileptogenesis in glioma is multifactorial, it is conceivable that a down-regulation of GS may have an important pro-epileptogenic role in GBM, through the slowing of glutamate-glutamine cycle. This study suggests that seizures in GBM are coupled with a highly localized enzyme deficiency. The manipulation of GS activity might constitute a novel principle for inhibiting seizures in patients with glioma epilepsy.