Association of 5-aminolevulinic acid fluorescence guided resection with photodynamic therapy in recurrent glioblastoma: a matched cohort study.

PURPOSE: Glioblastoma is a malignant and aggressive brain tumour that, although there have been improvements in the first line treatment, there is still no consensus regarding the best standard of care (SOC) upon its inevitable recurrence. There are novel adjuvant therapies that aim to improve local disease control. Nowadays, the association of intraoperative photodynamic therapy (PDT) immediately after a 5-aminolevulinic acid (5-ALA) fluorescence-guided resection (FGR) in malignant gliomas surgery has emerged as a potential and feasible strategy to increase the extent of safe resection and destroy residual tumour in the surgical cavity borders, respectively. OBJECTIVES: To assess the survival rates and safety of the association of intraoperative PDT with 5-ALA FGR, in comparison with a 5-ALA FGR alone, in patients with recurrent glioblastoma. METHODS: This article describes a matched-pair cohort study with two groups of patients submitted to 5-ALA FGR for recurrent glioblastoma. Group 1 was a prospective series of 11 consecutive cases submitted to 5-ALA FGR plus intraoperative PDT; group 2 was a historical series of 11 consecutive cases submitted to 5-ALA FGR alone. Age, sex, Karnofsky performance scale (KPS), 5-ALA post-resection status, T1-contrast-enhanced extent of resection (EOR), previous and post pathology, IDH (Isocitrate dehydrogenase), Ki67, previous and post treatment, brain magnetic resonance imaging (MRI) controls and surgical complications were documented. RESULTS: The Mantel-Cox test showed a significant difference between the survival rates (p = 0.008) of both groups. 4 postoperative complications occurred (36.6%) in each group. As of the last follow-up (January 2024), 7/11 patients in group 1, and 0/11 patients in group 2 were still alive. 6- and 12-months post-treatment, a survival proportion of 71,59% and 57,27% is expected in group 1, versus 45,45% and 9,09% in group 2, respectively. 6 months post-treatment, a progression free survival (PFS) of 61,36% and 18,18% is expected in group 1 and group 2, respectively. CONCLUSION: The association of PDT immediately after 5-ALA FGR for recurrent malignant glioma seems to be associated with better survival without additional or severe morbidity. Despite the need for larger, randomized series, the proposed treatment is a feasible and safe addition to the reoperation.

Prevertebral Inflammatory Myofibroblastic Tumor Following COVID Vaccine Booster Dose.

We report a case of a 30-year-old previously higid female presenting with dorsal pain and persistent fever after COVID-19 vaccine booster dose. CT and MR revealed a prevertebral heterogeneous and infiltrating mass, with spontaneous regression in control imaging which biopsy confirmed an inflammatory myofibroblastic tumor.

5-Aminolevulinic acid fluorescence in brain non-neoplastic lesions: a systematic review and case series.

Fluorescence-guided surgery with 5-aminolevulinic acid (5-ALA) is used to assist brain tumor resection, especially for high-grade gliomas but also for low-grade gliomas, metastasis, and meningiomas. With the increasing use of this technique, even to assist biopsies, high-grade glioma-mimicking lesions had misled diagnosis by showing 5-ALA fluorescence in non-neoplastic lesions such as radiation necrosis and inflammatory or infectious disease. Since only isolated reports have been published, we systematically review papers reporting non-neoplastic lesion cases with 5-ALA according with the PRISMA guidelines, present our series, and discuss its pathophysiology. In total, 245 articles were identified and 12 were extracted according to our inclusion criteria. Analyzing 27 patients, high-grade glioma was postulated as preoperative diagnosis in 48% of the cases. Microsurgical resection was performed in 19 cases (70%), while 8 patients were submitted to biopsy (30%). We found 4 positive cases in demyelinating disease (50%), 4 in brain abscess (80%), 1 in neurocysticercosis (33%), 1 in neurotoxoplasmosis, infarction, and hematoma (100%), 4 in inflammatory disease (80%), and 3 in cortical dysplasia (100%). New indications are being considered especially in benign lesion biopsies with assistance of 5-ALA. Using fluorescence as an aid in biopsies may improve procedure time, number of samples, and necessity of intraoperative pathology. Further studies should include this technology to encourage more beneficial uses.

From the inside out: oculocerebrocutaneous syndrome without dermatological manifestations.

Delleman-Oorthuys or oculocerebrocutaneous syndrome (OCCS) is an extremely rare condition which relies on three pillars of manifestations: ophthalmological, neurological, and dermatological. It was first described by Delleman and Oorthuys in 1981, and since then, very few other cases have been reported. We report the case of a 13-month-old boy, referred to an ophthalmological tertiary service for investigation of a microglobe with a cystic retrobulbar mass on translucency test. MRI revealed a left microphthalmia, with extensive retrobulbar cystic lesions occupying the remaining orbit and protruding anteriorly the microglobe. Cerebral findings included polymicrogyria, subependymal nodular heterotopia at the level of the left occipital horn of the lateral ventricle, and an importantly enlarged mesencephalic tectum observed in association with an absent cerebellar vermis. The ensemble of malformations met the criteria for definite OCCS. Being a tremendously rare syndrome, OCCS will always represent a diagnostic challenge. However, its emblematic cutaneous manifestations might be an important part of the road map leading to its correct interpretation. Thus, when absent, diagnosis can be harder than usual, and, on these cases, neurologists, ophthalmologists, and radiologists play a crucial role.

Turbulent Cerebrospinal Fluid Flow in Enlarging Terminal Myelocystocele.

Terminal myelocystocele is a rare type of neural tube malformation, consisting of a skin-covered lumbosacral mass, highly associated with other complex abdominal malformations within the OIES complex (omphalocele, imperforate anus, exstrophy of the cloaca and spinal defects). We present a case of a premature female with an extensive lumbosacral mass at birth, as well as an omphalocele, cloacal exstrophy, renal abnormalities, and sacral agenesis. Lumbar magnetic resonance imaging revealed a meningocele sac herniating through the bone defects and containing a syringocele sac. Advanced imaging techniques showed turbulent cerebrospinal fluid flow. At control, 4 weeks later, the defect doubled in size. The myelocystocele sac was evacuated and closed, and the patient persisted with paraparesis. The role of cerebrospinal fluid flow analysis is well established in Chiari-type malformations, in which turbulent flow within the syrinx is related to a better outcome after surgery. It is possible that the same principle could be applied to other spinal malformations, as shown in this case of terminal myelocystocele.

Management of jugular bulb injury during retrosigmoid transmeatal resection of vestibular schwannoma.

Inadvertent laceration of the jugular bulb is a potentially serious complication of the retrosigmoid transmeatal approach to vestibular schwannomas. Here, the authors present the case of a 51-year-old woman with a right Hannover T4a vestibular schwannoma and bilateral high-riding jugular bulb, which was opened during drilling of the internal auditory canal (IAC). They highlight the immediate management of this complication, technical nuances for closing the defect without occluding the jugular bulb, and modifications of the standard technique needed to continue surgical resection. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID2155.