Clinical and radiological features of Forestier's disease presenting with dysphagia

Background: Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier's disease, is a rheumatologic condition characterized by ossification of the spinal ligaments and tendons. Large anterior osteophytes are typically present in the lower cervical levels, while upper cervical ossification resulting in dysphagia is very rare. Methods: Here, we presented a patient with Forestier's disease involving massive ossification of the anterior longitudinal ligament extending from C3 to C4 downward contributing to severe dysphagia. Results: A 65‑year‑old male presented with cervical pain and dysphagia. The computed tomography of the cervical spine demonstrated massive anterior longitudinal ligament ossification (DISH) extending from C3 to C7. There was an additional large osteophyte at the C3‑C4 level, and also a high‑grade intracanalicular C6‑C7 cervical stenosis due to ossification of the posterior longitudinal ligament. The patient was offered surgical intervention (e.g., resection of the C3‑C7 anterior DISH and anterior cervical discectomy/fusion at the C6‑C7 level), but he declined. Conclusions: When conservative management fails to resolve severe dysphagia for cervical DISH/Forestier's disease, anterior surgical resection is typically performed. In this case, the patient refused surgery and opted for conservative management strategies

A thoracic vertebral localization of a metastasized cutaneous Merkel cell carcinoma: Case report and review of literature

Background: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin tumor, which may be related to sun exposure. It can metastasize to lungs, liver and bone, leading to severe morbidity and mortality. Vertebral metastases from MCC are rare. The authors report the tenth case in the literature, a 59-year-old patient with MCC, which was primarily localized in the scalp, and later provoked distant metastasis to the thoracic spinal column. Case Description: A 59-year-old woman was admitted at our Unit of Neurosurgery with a 4-month history of progressive and severe dorsal back pain, without neurological signs. The patient had been surgically treated for a recidivated MCC in the occipital region in 2007, 2011, and 2013. (In 2013, the surgical treatment also included lateral cervical lymph node dissection). Chemotherapy and radiotherapy had come after the treatments. Magnetic resonance imaging (MRI) of the dorsal spine showed metastatic vertebral involvement with cord impingement of the T7-T8 levels. A total body CT scan revealed lungs and liver metastases, besides vertebral district. After a multidisciplinary consult a palliative surgery was decided and a posterior dorsal approach was employed: Radiofrequency (RF) thermoablation was followed by the injection of cement of T7 and T8 and transpedicle fixation T5-T9. The postoperative course was uneventful and followed by a further adjuvant therapy Conclusion: Spinal metastases from MCC are described in literature only exceptionally. The clinical course is presented, along with a review of literature

End-of-life care in high-grade glioma patients. The palliative and supportive perspective

High-grade gliomas (HGGs) are the most frequently diagnosed primary brain tumors. Even though it has been demonstrated that combined surgical therapy, chemotherapy, and radiotherapy improve survival, HGGs still harbor a very poor prognosis and limited overall survival. Differently from other types of primary neoplasm, HGG manifests also as a neurological disease. According to this, palliative care of HGG patients represents a peculiar challenge for healthcare providers and caregivers since it has to be directed to both general and neurological cancer symptoms. In this way, the end-of-life (EOL) phase of HGG patients appears to be like a journey through medical issues, progressive neurological deterioration, and psychological, social, and affective concerns. EOL is intended as the time prior to death when symptoms increase and antitumoral therapy is no longer effective. In this phase, palliative care is intended as an integrated support aimed to reduce the symptoms burden and improve the Quality Of Life (QOL). Palliative care is represented by medical, physical, psychological, spiritual, and social interventions which are primarily aimed to sustain patients’ functions during the disease time, while maintaining an acceptable quality of life and ensuring a dignified death. Since HGGs represent also a family concern, due to the profound emotional and relational issues that the progression of the disease poses, palliative care may also relieve the distress of the caregivers and increase the satisfaction of patients’ relatives. We present the results of a literature review addressed to enlighten and classify the best medical, psychological, rehabilitative, and social interventions that are addressed both to patients and to their caregivers, which are currently adopted as palliative care during the EOL phase of HGG patients in order to orientate the best medical practice in HGG management

Unusual case of dorsal vertebral metastases from a male breast cancer

Introduction: Breast cancer is a malignant neoplasm arising from mammary glands and is a rare entity in the male patient. Spine is a common site for skeletal metastases. Case presentation: A 53-years-old male was admitted to our Neurosurgical unit because of an untreatable dorsal pain. He had been treated 3 years before for breast cancer. A dorsal Magnetic Resonance Imaging (MRI) evidenced pathological masses at T8 level. A total body Computed Tomography (CT) scan revealed lungs and liver metastases. After a multidisciplinary consult, a posterior T8 decompression, a radiofrequency thermoablation in T8 vertebral body followed by screws and rods fixation (T7-T9) was performed. Post-operative course was uneventful and the patient experienced a significant improvement of dorsal pain. Cycles of adjuvant chemo-radiotherapy followed. After 8 months, patient is still alive and in good general conditions but a spine MRI revealed a rapid and widespread diffusion of ostelytic metastases. Conclusion: Despite the high incidence of breast cancer metastases to the spine, very few clinical reports (just eight, whose only one treated surgically) in the literature deal specifically with metastases from male breast cancer, which is rarer and with a worst prognosis than female counterpart. In spite of the poor course, surgery still plays a role in treating these kind of malignancies and in improving quality of life

New hope in brain glioma surgery: The role of intraoperative ultrasound. A review

Maximal safe resection represents the gold standard for surgery of malignant brain tumors. As regards gross-total resection, accurate localization and precise delineation of the tumor margins are required. Intraoperative diagnostic imaging (Intra-Operative Magnetic Resonance-IOMR, Intra-Operative Computed Tomography-IOCT, Intra-Operative Ultrasound-IOUS) and dyes (fluorescence) have become relevant in brain tumor surgery, allowing for a more radical and safer tumor resection. IOUS guidance for brain tumor surgery is accurate in distinguishing tumor from normal parenchyma, and it allows a real-time intraoperative visualization. We aim to evaluate the role of IOUS in gliomas surgery and to outline specific strategies to maximize its efficacy. We performed a literature research through the Pubmed database by selecting each article which was focused on the use of IOUS in brain tumor surgery, and in particular in glioma surgery, published in the last 15 years (from 2003 to 2018). We selected 39 papers concerning the use of IOUS in brain tumor surgery, including gliomas. IOUS exerts a notable attraction due to its low cost, minimal interruption of the operational flow, and lack of radiation exposure. Our literature review shows that increasing the use of ultrasound in brain tumors allows more radical resections, thus giving rise to increases in survival

With a little help from my friends: The role of intraoperative fluorescent dyes in the surgical management of high-grade gliomas

High-grade gliomas (HGGs) are the most frequent primary malignant brain tumors in adults, which lead to death within two years of diagnosis. Maximal safe resection of malignant gliomas as the first step of multimodal therapy is an accepted goal in malignant glioma surgery. Gross total resection has an important role in improving overall survival (OS) and progression-free survival (PFS), but identification of tumor borders is particularly difficult in HGGS. For this reason, imaging adjuncts, such as 5-aminolevulinic acid (5-ALA) or fluorescein sodium (FS) have been proposed as superior strategies for better defining the limits of surgical resection for HGG. 5-aminolevulinic acid (5-ALA) is implicated as precursor in the synthetic pathway of heme group. Protoporphyrin IX (PpIX) is an intermediate compound of heme metabolism, which produces fluorescence when excited by appropriate light wavelength. Malignant glioma cells have the capacity to selectively synthesize or accumulate 5-ALA-derived porphyrins after exogenous administration of 5-ALA. Fluorescein sodium (FS), on the other hand, is a fluorescent substance that is not specific to tumor cells but actually it is a marker for compromised blood-brain barrier (BBB) areas. Its effectiveness is confirmed by multicenter phase-II trial (FLUOGLIO) but lack of randomized phase III trial data. We conducted an analytic review of the literature with the objective of identifying the usefulness of 5-ALA and FS in HGG surgery in adult patients

Forecasting Molecular Features in IDH-Wildtype Gliomas: The State of the Art of Radiomics Applied to Neurosurgery

Simple Summary The prognostic expectancies of patients affected by glioblastoma have remained almost unchanged during the last thirty years. Along with specific oncological research and surgical technical alternatives, corollary disciplines are requested to provide their contributions to improve patient management and outcomes. Technological improvements in radiology have led to the development of radiomics, a new discipline able to detect tumoral phenotypical features through the extraction and analysis of a large amount of data. Intuitively, the early foreseeing of glioma features may constitute a tremendous contribution to the management of patients. The present manuscript analyzes the pertinent literature regarding the current role of radiomics and its potentialities. Background: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, marks a step forward the future diagnostic approach to these neoplasms. Alongside this, radiomics has experienced rapid evolution over the last several years, allowing us to correlate tumor imaging heterogeneity with a wide range of tumor molecular and subcellular features. Radiomics is a translational field focused on decoding conventional imaging data to extrapolate the molecular and prognostic features of tumors such as gliomas. We herein analyze the state-of-the-art of radiomics applied to glioblastoma, with the goal to estimate its current clinical impact and potential perspectives in relation to well-rounded patient management, including the end-of-life stage. Methods: A literature review was performed on the PubMed, MEDLINE and Scopus databases using the following search items: "radiomics and glioma", "radiomics and glioblastoma", "radiomics and glioma and IDH", "radiomics and glioma and TERT promoter", "radiomics and glioma and EGFR", "radiomics and glioma and chromosome". Results: A total of 719 articles were screened. Further quantitative and qualitative analysis allowed us to finally include 11 papers. This analysis shows that radiomics is rapidly evolving towards a reliable tool. Conclusions: Further studies are necessary to adjust radiomics' potential to the newest molecular requirements pointed out by the 2021 WHO classification of CNS tumors. At a glance, its application in the clinical routine could be beneficial to achieve a timely diagnosis, especially for those patients not eligible for surgery and/or adjuvant therapies but still deserving palliative and supportive care

Brain mapping-aided supratotal resection (Sptr) of brain tumors: The role of brain connectivity

Brain gliomas require a deep knowledge of their effects on brain connectivity. Understanding the complex relationship between tumor and functional brain is the preliminary and fundamental step for the subsequent surgery. The extent of resection (EOR) is an independent variable of surgical effectiveness and it correlates with the overall survival. Until now, great efforts have been made to achieve gross total resection (GTR) as the standard of care of brain tumor patients. However, high and low-grade gliomas have an infiltrative behavior and peritumoral white matter is often infiltrated by tumoral cells. According to these evidences, many efforts have been made to push the boundary of the resection beyond the contrast-enhanced lesion core on T1w MRI, in the so called supratotal resection (SpTR). SpTR is aimed to maximize the extent of resection and thus the overall survival. SpTR of primary brain tumors is a feasible technique and its safety is improved by intraoperative neuromonitoring and advanced neuroimaging. Only transient cognitive impairments have been reported in SpTR patients compared to GTR patients. Moreover, SpTR is related to a longer overall and progression-free survival along with preserving neuro-cognitive functions and quality of life