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Background: Intramedullary spinal cord metastasis (ISCM) is uncommon and usually occurs in advanced malignancies. Effective management methods are not clearly defined, and the outcomes of current treatments vary. Currently, there is no universal strategy for managing patients with intramedullary spinal metastases., Case: To the best of our knowledge, we present a case of ovarian adenocarcinoma that was managed surgically in a 70-year-old woman with metastasis to the upper thoracic spinal cord, and we assess pertinent literature and deliberate management approaches., Conclusions: Progression in early diagnosis and advanced therapeutic methods for ISCMs have contributed to the increased incidence and prevalence of this condition. There is no established consensus regarding the definitive patient management methods. Consequently, we offer multidisciplinary management with individualization based on the patient's functional status, requirement for a definitive diagnosis for potential additional adjuvant therapies, and assessment of the extent of systemic disease, which can influence the desired quality of life and survival duration., (© 2024 The Author(s). Cancer Reports published by Wiley Periodicals LLC.)

Objective: Intradural spinal metastases are considered rare. At present, limited information is available on incidence, surgical management, and outcomes., Methods: We conducted a retrospective patient chart review from 2002 to 2024, identifying all patients surgically treated for intradural spinal metastases. Clinical, surgical and survival data were collected and compared to literature data for patients surgically treated for extradural spinal metastases., Results: A total of 172 patients with spinal metastases were identified with 13 patients meeting inclusion criteria (7.6%). The mean age at diagnosis of intradural spinal metastases was 52 ± 22 years, with diverse primaries including lung ( n = 3), breast ( n = 2), sarcoma ( n = 2), and six unique entities. Intradural spinal metastasis was diagnosed on average of 3.3 years after primary diagnosis. In total, we observed five (38%) intradural-extramedullary and eight (62%) intramedullary metastases, located in the cervical (38.5%), thoracic (46.1%) and lumbar spine (15.4%). The most common preoperative symptoms were pain, sensory changes, and gait ataxia (each 76.9%). Gross total resection was achieved in 54%, and local tumor control in 85%. Postoperatively, 92% exhibited clinical improvement or stability. Most frequent adjuvant treatment was radio- and/or chemotherapy in 85%. The average survival after operation for spinal intradural metastases was 5 months, ranging from 1 month to 120 months. The location of the intradural metastasis in the cervical spine was associated with a significantly more favorable survival outcome (compared to thoracic/lumbar location, p = 0.02)., Conclusions: Intradural location of spinal metastases is rare (7.6%). Even so, surgical resection is safe and effective for neurological improvement, and survival appears lower compared to the reported survival of extradural spinal metastases.

Brain metastases (BMs) arising from ovarian cancer remain rare. Spinal cord metastases are even rarer, accounting for just 0.4% of total metastatic spinal cord compressions. In this report, we describe a case of a woman in her 70s who developed sequential brain and spinal cord metastases during her treatment for high-grade serous ovarian cancer, without a germline or somatic BRCA mutation. Following completion of neoadjuvant chemotherapy, interval debulking surgery and adjuvant chemotherapy, relapsed disease was ultimately identified as a single BM, curiously mimicking an acoustic neuroma. Subsequently, spinal cord metastases rapidly developed. Throughout, multidisciplinary team meetings guided decisions on patient management. In this report, we highlight the rarity of such a presentation and discuss the possible role of disease pathophysiology, associated systemic anticancer therapy resistance, and treatment possibilities for both cerebral and spinal metastases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Intramedullary spinal cord metastases (ISCM) are a rare and challenging manifestation of metastatic cancer that have devastating impacts on the individual's neurological function, survival expectancy and overall quality of life. Given the rarity and poor prognosis, there is a lack of consensus in management. Uterine carcinosarcoma itself is a rare cancer, accounting for less than 3% of all uterine cancers. It carries a poor prognosis, with only one-third of patients surviving beyond 5 years. There are no previous reports of uterine carcinosarcoma metastases to the spinal cord. Here, we present the case of a woman in her late 70s with a uterine carcinosarcoma intramedullary metastasis that was refractory to radiotherapy treatment and responded favourably to surgical debulking., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Intramedullary metastases (IMM) lead to severe neurological symptoms. They are extremely rare in clinical practice. Optimal management of such patients is not clearly defined. Surgery, chemo- and radiotherapy are the main options., Objective: To study the results of stereotactic high-dose irradiation for IMM considering local control of metastases, neurological status and life expectancy., Material and Methods: Nineteen patients with 29 IMMs underwent high-dose irradiation (Cyber Knife G4 and TrueBeam STx devices) between 2016 and 2022. There were 1-10 fractions, radiation dose 14.0-35.0 Gy with isodose 70-100%. Local control and new spinal cord metastases were assessed using contrast-enhanced MRI. Clinical status and neurological functions were assessed using the Karnofsky and McCormick scales., Results: Local control rates were 91% and 78% after 6 and 12 months, respectively. Overall survival after treatment was 7.3 months, 6- and 12-month overall survival - 58% and 37%, respectively. In addition, 8 patients were available for follow-up. Of these, 6 ones demonstrated improvement or stabilization of neurological function after treatment. The main cause of death was progression of the underlying disease. No significant treatment-related toxic effects were observed., Conclusion: High-dose irradiation is a safe, effective and time-saving treatment for IMM. Considering unadvisable surgical methods and chemotherapy for this pathology, we can recommend high-dose irradiation for patients with spinal cord metastases.

Background: Breast cancer is currently the most diagnosed cancer globally. For patients who complete breast cancer treatment, developing a survivorship plan is important, including serial history, physical examinations, and annual mammograms to look for recurrence and metastasis., Case Report: This is a case report of a 76-year-old female with a history of recurrent breast cancer who presented with lower-back pain and found to have MRI findings initially concerning for intradural extramedullary metastatic disease. Biopsy was later found to be consistent with benign spinal schwannomas., Conclusion: We present a unique case of spinal masses in the setting of breast cancer initially concerning for leptomeningeal spread, later found to have benign schwannomas that mimicked leptomeningeal spread on imaging. To our knowledge, this is the first reported case of schwannomas mimicking leptomeningeal spread in a patient with a history of recurrent breast cancer. After metastasis is excluded, schwannomas should be considered in the differential of benign spinal lesions., (Copyright © 2023, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Abstract: Chest CT images were acquired in a 79-year-old man to evaluate a right lung mass revealed by chest radiography. The image findings suggested possible pulmonary malignancy. FDG PET/CT was performed for staging, which displayed not only a hypermetabolic mass in the upper lobe of right lung but also increased FDG uptake of the spinal cord at T11 to T12 level. A lesion corresponding to the hypermetabolic region of the spinal cord was revealed by MRI. The intramedullary spinal cord metastasis from lung squamous cell carcinoma was confirmed through the pathological examination after the spinal cord lesion was resected., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Tumors of the central nervous system (CNS) include primary tumors - itraaxial, growing from brain and spinal cord cells (neuroepithelial tumors) or extraaxial, growing from surrounding structures (brain and spinal cord, nerve sheaths, vascular structures, lymphatic tissue, germ cells, malformations, pituitary glands). Much more often they are located in the intracranial space a solitary or multiple metastatic spread of malignancy originating from another organ (eg lung, breast, malignant melanoma, Grawitzs tumor). The occurrence of metastases of solid tumors is then in the intraaxial or extraaxial region, leptomeningeal or dural. Even morphologically benign tumors with their occurrence in a closed CNS compartment can have malignant behaviour and cause severe slowly developing to acute neurological symptoms, including intracranial hypertension. Primary tumors of the central nervous system present 1-2% of all cancers, with a higher incidence in adults after the age of 60, with a slight predominance in men, with higher mortality in men than in women. About 5% of CNS tumors are hereditary (e.g., Li-Fraumeni syndrome, neurofibromatosis type I, II). The causes of most brain and spinal cord tumors are unclear, the effect of radiation has been definitely demonstrated, there is an increased risk in transplant patients and AIDS (Acquired Immune Deficiency Syndrome) patients, and the potentiating effects of some chemicals and viruses on the development of CNS neoplasms are uncertain. The effectiveness of treatment of brain and spinal cord tumors is influenced by the existence of the so-called hematoencephalic barrier, which protects the brain from the penetration of toxic substances, but at the same time prevents the penetration of most cytostatics to the tumor target. Another obstacle may be the localization of the tumor in areas difficult to access for histological verification (brain stem, optical chiasma) due to the high risk of complications even after stereotactic biopsy. In some cases, in an effort not to cause an irreversible neurological deficit by inconsiderate tissue collection, the sample of histological material can then become inconclusive to tumor cells, i.e., tumor cells are not captured. Last but not least, the radiosensitivity of some brain structures is also limiting, which makes it impossible to apply a higher dose of ionizing radiation to a tumor affecting sensitive tissues or located near of these sensitive tissues. The rapid development of immunohistochemical (IHC) and molecular genetic analysis methods has significantly refined diagnostics and thus theoretically facilitates the choice of the optimal treatment procedure for the individual patient. While advances in modern conformal photon and particle (currently the most frequently proton) radiotherapy, stereotactic radiosurgery has enabled accurately targeted irradiation of the CNS tumor site and at the same time spare the high-risk brain structures, thereby significantly reduce the risk of acute and late neurotoxicity, pharmacotherapy options are still limited. Just molecular-genetic knowledge already provides us with predictive and prognostic information. They should increasingly stratify patients for targeted therapy.

Background/aim: Leptomeningeal metastases (LMs) of the spine have complex management. We reviewed the literature on spine LMs., Materials and Methods: PubMed, EMBASE, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of spine LMs., Results: We included 46 studies comprising 72 patients. The most frequent primary tumors were lung (19.4%) and breast cancers (19.4%). Median time from primary tumors was 12 months (range=0-252 months). Cauda equina syndrome occurred in 34 patients (48.6%). Nodular spine LMs (63.6%) were more frequent. Concurrent intracranial LMs were present in 27 cases (50.9%). Cerebrospinal fluid cytology was positive in 31 cases (63.6%). Cases were managed using palliative steroids (73.6%) with locoregional radiotherapy (55.6%) chemotherapy (47.2%), or decompressive laminectomy (8.3%). Post-treatment symptom improvement (32%) and favorable radiological response (28.3%) were not different based on treatment (p=0.966; p=0.727). Median overall-survival was 3 months (range=0.3-60 months), not significantly different between radiotherapy and chemotherapy (p=0.217)., Conclusion: Spine LMs have poor prognoses. Radiotherapy, chemotherapy, and surgery are only palliative, as described for intracranial LMs., (Copyright © 2022 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Introduction: Central nervous system (CNS) metastasis from nasopharyngeal carcinoma (NPC) is rare and presents with high morbidity and mortality. As a rare entity, a few patients with CNS metastasis from NPC have been reported, and no studies were available on treatment and prognosis. Based on our clinical experience, early diagnosis with incorporation of a clear palliative plan is imperative in providing holistic care for patients with locally-invasive and metastatic nasopharyngeal carcinoma., Case Report: Our study reports a case of a 48-year-old Filipino male with known NPC Stage IVB who developed acute symptoms of constipation, urinary retention, and bilateral lower limb weakness and numbness. Magnetic resonance imaging showed intramedullary lesions in multiple segments of the spinal cord. Steroid and radiotherapy of the spine were initiated with noted transient improvement of the motor strength. Subsequently, he developed cancer-related stroke. The patient progressively deteriorated despite best medical care., Competing Interests: Declaration of competing interest None., (Copyright © 2021. Published by Elsevier Inc.)

Background: Intramedullary metastasis (IMM) is a rare disease with poor prognosis. The incidence of IMMs has increased, which has been linked to improved systemic treatment in many cancers. Surgery and/or radiotherapy are the most commonly used treatments; only small-sample retrospective studies and case reports on stereotactic body radiotherapy (SBRT) have reported acceptable results in terms of local control and clinical improvement, with no reported toxicity. Thus, we performed this monocentric retrospective study on five cases treated with SBRT for IMMs, which we supplemented with a systematic review of the literature., Methods: We included all patients treated for IMM with SBRT. The target tumor volume, progression-free survival, prescription patterns in SBRT, survival without neurological deficit, neurological functional improvement after treatment, and overall survival were determined., Results: Five patients treated with a median dose of 30 Gy in a median number of fractions of 5 (prescribed at a median isodose of 86%) included. The median follow-up duration was 23 months. Two patients showed clinical improvement. Three patients remained stable. Radiologically, 25% of patients had complete response and 50% had stable disease. No significant treatment-related toxicity was observed., Conclusion: SBRT appears to be a safe, effective, and rapid treatment option for palliative patients., (© 2021. The Author(s).)

Background: Pituitary carcinomas (PCs), defined as distant metastases of pituitary neoplasms, are very rare malignancies. Because the clinical presentation of PCs is variable, early diagnosis and management remain challenging. PCs are always refractory to comprehensive treatments, and patients with PCs have extremely poor prognoses., Case Presentation: We describe one case of a prolactin-secreting pituitary adenoma (PA) refractory to conventional therapy that evolved into a PC with intraspinal metastasis. A 34-year-old female was diagnosed with an invasive prolactin-secreting PA in 2009 and was unresponsive to medical treatment with bromocriptine. The tumor was gross totally removed via transsphenoidal surgery (TSS). However, the patient experienced multiple tumor recurrences or regrowth despite comprehensive treatments, including medical therapy, two gamma knife radiosurgeries (GKSs), and four frontal craniotomies. In 2016, she was found to have an intradural extramedullary mass at the level of the fourth lumbar vertebra. The intraspinal lesion was completely resected and was confirmed as a metastatic PC based on histomorphology and immunohistochemical staining. The literature on the diagnosis, molecular pathogenesis, treatment, and prognosis of patients with prolactin-secreting PCs was reviewed., Conclusion: PCs are very rare neoplasms with variable clinical features and poor prognosis. Most PCs usually arise from aggressive PAs refractory to conventional therapy. There is no reliable marker to identify aggressive PAs with a risk for progression to PCs; thus, it is difficult to diagnose these PCs early until the presence of metastatic lesions. It is still very challenging to manage patients with PCs due to a lack of standardized protocols for diagnosis and treatment. Establishing molecular biomarkers and the pathobiology of PCs could help in the early identification of aggressive PAs most likely to evolve into PCs., (© 2021. The Author(s).)

Epithelioid glioblastoma multiforme (eGBM) is a rare and aggressive variant of glioblastoma multiforme (GBM) that predominantly affects younger patients and can be difficult to distinguish from other gliomas. Data on how patients with eGBM might be best treated are limited, although genomic analyses have shown that almost half of tumours harbour activating BRAF gene mutations. Here we present the case of a young female with BRAF V600E-mutant eGBM who had a prolonged response to targeted therapy with the BRAF and MEK1/2 inhibitors dabrafenib and trametinib. We review current knowledge about eGBM, including the emerging role for BRAF- ± MEK1/2- targeted therapy., (Copyright © 2021. Published by Elsevier Inc.)

Purpose/aim: Intradural extramedullary (IDEM) ependymomas are very rare, and IDEM ependymomas with craniospinal disseminated metastasis are exceptionally rare; only 2 preoperative cases have been confirmed, and postoperative cases have not been reported., Case Report: We present a case of a 21-year-old female with an IDEM ependymoma of the craniocervical junction who experienced head and neck pain for more than 1 month. Magnetic resonance imaging (MRI) of the cervical spine revealed a large IDEM cystic lesion located in the medulla oblongata and the upper cervical spinal cord. The patient underwent surgery without complications, and the tumor was completely removed. Histopathological examination revealed a diagnosis of aplastic ependymoma, World Health Organization (WHO) grade III. The patient failed to follow-up with radiotherapy for one month after discharge. Nearly three months after surgery, craniospinal disseminated metastasis was found in the patient; subsequently, chemoradiotherapy was administered to prolong the survival time of the patient. Unfortunately, the patient underwent radiotherapy and chemotherapy for only 7 days; then, the patient gave up treatment and died 5 months later., Conclusions: To the best of our knowledge, no other cases of craniocervical junction anaplastic ependymomas with craniospinal disseminated metastasis have been reported in the literature. Total resection does not completely prevent recurrence and metastasis, and MRI of the entire neuraxis and timely postoperative craniospinal radiotherapy are necessary for the treatment of this disease.

Purpose: Peripheral enhancement characteristics on magnetic resonance imaging (MRI), namely the rim and flame signs, are specific for intramedullary spinal cord metastases (ISCM) compared to primary cord masses. The study compared the frequency of a novel finding-the central dot sign-in ISCMs versus primary intramedullary masses., Methods: In this study 45 patients with 64 ISCMs and 64 control patients with 64 primary intramedullary cord masses were investigated and 2 radiologists blinded to lesion type independently evaluated MR images for the presence of a central dot sign: a punctate focus of enhancement in/near the center of an enhancing intramedullary mass. The frequency of this sign in the two patient groups was compared., Results: A total of 63 enhancing ISCMs in 44 patients and 54 enhancing primary cord masses in 54 patients were included. The central dot sign was identified in 6% (4/63) of enhancing ISCMs in 9% (4/44) of patients and in none (0/54) of the enhancing primary cord masses (p = 0.038, per patient). The specificity for diagnosing ISCMs among spinal cord masses was 100%. The central dot sign was present in the axial plane only in two ISCMs and in the axial and sagittal planes in two ISCMs. The two ISCMs harboring the central dot sign also demonstrated both the previously described rim and flame signs, and two also demonstrated the rim sign alone., Conclusion: The central dot sign is not sensitive but highly specific for ISCMs compared to primary spinal cord masses. The rim and/or flame signs may or may not be concurrently present in ISCMs.

Intramedullary spinal cord metastases (ISCM) is a rare, but devastating complication of malignant disease. Prognosis is poor, with an overall median survival (OS) of 4 months from the time of diagnosis. Yet, ISCMs are being increasingly diagnosed, related to advances and increased use of imaging and therapies that prolong survival in patients with cancer. Prompt and accurate diagnosis of ISCM is necessary for effective treatment, and magnetic resonance imaging (MRI) is the preferred imaging technique. The optimal management of these patients is controversial because of the multitude of clinical circumstances and the lack of controlled studies on the efficacy of the different therapeutic approaches. Increased awareness of this rare entity may lead to an earlier diagnosis at a stage when neurological deficits are reversible, and therefore, more effective palliation may be achieved. Therefore, we carried out this retrospective research of 3 observations of ISCM, associated with a detailed review of the literature describing the diagnostic, therapeutic and evolutionary characteristics of this special rare entity., (Copyright © 2020 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Abstract: Glioblastoma multiforme is one of the most common malignant types of tumor arising from the central nervous system known for its devastating intracranial progress and dismal prognosis. Macroscopically evident and symptomatic spinal cord metastasis detected with FDG PET/CT imaging is a rare event. We present a rare case of symptomatic diffuse spinal metastases of glioblastoma multiforme in a 25-year-old woman, who has been previously treated surgically with gross tumor resection followed by adjuvant radiotherapy and chemotherapy with temozolomide., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Extrarenal Wilms tumor (ERWT) is an extremely rare neoplasm of childhood. It occurs predominantly in retroperitoneum and pelvic or inguinal region without involvement of the kidneys. Although the importance of FDG PET/CT in Wilms tumor is well known, its use in ERWT is limited. Herein, we present FDG PET/CT findings of a 3-year-old girl with a lumbar mass, which was later diagnosed with ERWT.

Objective: Pediatric skull base chordoma is a rare entity that is traditionally considered to display aggressive behavior with an increased risk of recurrence. There is an absence of literature examining outcomes in the pediatric population in general and using the endoscopic endonasal approach (EEA)., Methods: The authors retrospectively reviewed all patients with skull base chordomas presenting by the age of 18 years to the Children's Hospital of Pittsburgh or the University of Pittsburgh Medical Center from 2004 to 2019. Clinical outcomes, the number and location of recurrences, and progression-free survival time were determined., Results: Twenty patients met the study criteria. The most common presenting complaints were diplopia (n = 7), headache (n = 6), and swallowing difficulty (n = 4). Three cases were incidentally discovered. Twelve patients underwent single-stage EEA alone, 2 patients had two-stage EEA, and 6 patients had combined EEA with open far-lateral or extreme-lateral approaches. Fourteen patients underwent gross-total resection (GTR), and 6 patients had near-total resection. Larger tumors were more likely to require staging or a combined approach (86% vs 7%) and were less likely to receive GTR (33% vs 86%) but had comparable recurrence and mortality rates. Five patients developed CSF leaks requiring reoperation, 2 patients developed a permanent abducens nerve palsy, 1 patient suffered an internal carotid artery injury, 1 patient developed an epidural hematoma, and 1 patient developed a subdural empyema. Four (20%) patients had recurrence during follow-up (mean radiographic follow-up 59 months and mean time to local recurrence 19 months). Two patients with recurrence underwent further resection, and 1 patient elected to stop treatment. Both patients who underwent repeat resection experienced a second recurrence, one of whom elected to stop treatment. Both patients who died had an elevated Ki-67 (p = 0.039), one of whom developed de-differentiated histology. A third patient died of progressive spinal metastases without local recurrence and is one of 2 patients who developed postoperative spinal metastases. Both patients whose tumors became de-differentiated progressed from tumors with an initial Ki-67 of 15 or greater (p = 0.035) and received prior radiotherapy to the bulk tumor (p = 0.03)., Conclusions: The majority of pediatric skull base chordomas, when managed at a specialized center with a goal of GTR, may have a better outcome than traditionally believed. Elevated Ki-67 rates may predict poor outcome and progression to de-differentiation.

Background: Metastatic spinal cord compression (MSCC) from prostate cancer (PC) influences not only patients' prognosis but also their quality of life. However, little is known about the clinical outcome of surgery for MSCC from PC. We evaluated both the oncological and functional outcomes of decompression and reconstruction surgery for patients with symptomatic MSCC from PC., Methods: We assessed 19 patients who underwent decompression and reconstruction surgery for symptomatic MSCC from PC. Of these 19 patients, 8 had metastatic hormone-naïve PC (mHNPC) and 11 had metastatic castration-resistant PC (mCRPC)., Results: The median age of the patients with mHNPC and mCRPC was 72 and 65 years, respectively. The median prostate-specific antigen level at the time of diagnosis of MSCC in patients with mHNPC and mCRPC was 910 and 67 ng/mL, respectively. Although two of eight patients (25.0%) with mHNPC were ambulatory preoperatively, six patients (75.0%) were ambulatory postoperatively. Among 11 patients with mCRPC, only 3 (27.3%) were ambulatory preoperatively, while 6 (54.5%) were ambulatory postoperatively. The median postoperative overall survival among patients with mHNPC and mCRPC were not reached and 8 months, respectively., Conclusions: Decompression and reconstruction surgery for symptomatic MSCC from PC might contribute to a favorable functional outcome among men with mHNPC and mCRPC. However, its role in improving the oncological outcome remains unclear. The treatment strategy should be chosen by shared decision-making among patients, urologists, radiation oncologists, and orthopedic surgeons.

Objectives: Haematological toxicity and treatment breaks are common during cranio-spinal irradiation (CSI) due to irradiation of large volume of bone marrow. We conducted this study to see the effect of prophylactic granulocyte colony stimulating factor (GCSF) in reducing treatment breaks., Patients and Methods: The study was conducted over a period of 15 months from August 2017 to November 2018. Histopathologically proven Medulloblastoma patients received prophylactic GCSF during CSI. Acute hematological toxicities and treatment breaks were noted and effect of age and pretreatment blood counts were analyzed by SPSS (Statistical Package for Social Sciences) version 23., Results: A total of 28 patients were included in the study. During CSI, hematological toxicity leading to treatment breaks was observed in 11 (39.3 %) patients, of which grade 3 and 2 toxicities were seen in ten and one patients respectively. Younger age (<10 years) at diagnosis was significantly associated with the development of hematological toxicity (p = 0.028, Chi-Square). No correlation was found with pre-treatment blood counts., Conclusion: Prophylactic use of GCSF may be effective in preventing radiation induced hematological toxicity and treatment breaks., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Intramedullary metastases to the caudal neuraxis with exophytic extension to the extramedullary space are rare. We describe the unique case of a patient with locally recurrent breast cancer who developed an intramedullary-extramedullary metastasis to the conus medullaris and cauda equina 22 years after primary diagnosis, the longest interval between primary breast cancer and intramedullary spread to date. We also reviewed the published literature on focal breast metastases to the conus medullaris or cauda equina., Case Description: A 66-year-old woman with a history of node-positive estrogen receptor/progesterone receptor-positive, infiltrating ductal carcinoma diagnosed in 1997 and locally recurrent in 2007. Initial treatment included lumpectomy and targeted chemoradiation with mastectomy and hormonal therapy at recurrence. Twelve years later, she developed 6 weeks of bilateral buttock and leg pain without motor or sphincter compromise. Magnetic resonance imaging of the total spine revealed a 2 x 1.7 cm bilobed intradural, intramedullary-extramedullary, homogenously enhancing, T1-and T2-isointense lesion involving the conus medullaris and cauda equina. She underwent subtotal resection of a hormone receptor-positive breast metastasis. Her pain improved postoperatively and she was stable at 5 months., Conclusions: We provide evidence that patients who present with symptoms of spinal neurologic disease and a history of hormone receptor-positive breast cancer require high suspicion for metastatic pathology, despite significant time lapse from primary diagnosis. The tumor may involve both the intramedullary and extramedullary space, complicating resection. Symptom relief and quality of life should guide resection of metastatic lesions to the caudal neuraxis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Pancreatic neuroendocrine tumors (pNETs) are known to frequently metastasize to the liver and lymphatics; however, metastasis to the spine is exceedingly rare. We report the first case of an intradural, extramedullary pNET metastasis to the upper cervical spine., Case Description: A 75-year-old Hispanic male patient with history of stage IV pNET with metastasis to the liver and lymph nodes and new-onset lymphadenopathy seen on CT of the chest was found on positron emission tomography scan to have a lesion in the cervical spine. The patient was neurologically intact on physical examination, yet given the patient's medical history, magnetic resonance imaging of the cervical spine was performed, revealing a right-sided intradural, extramedullary mass at the C1-C2 level with associated mass effect on the spinal cord, likely representing a schwannoma. Due to the tumor size, mass effect, and the need for definitive tissue diagnosis, a partial C1-C2 laminectomy with intradural resection of the tumor was performed. The histology was consistent with the patient's known pNET., Conclusions: As treatment for pNETs has evolved, there has been a surge in unique presentations of systemic well-differentiated pNETs being reported. It is vital that patients diagnosed with pNET be monitored for metastases, and when discovered, treated promptly., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Medulloblastoma (MB) is the most frequent malignant brain tumour in children with a poor outcome. Divided into four molecular subgroups, MB of the Sonic hedgehog (SHH) subgroup accounts for approximately 25% of the cases and is driven by mutations within components of the SHH pathway, such as its receptors PTCH1 or SMO. A fraction of these cases additionally harbour PIK3CA mutations, the relevance of which is so far unknown. To unravel the role of Pik3ca mutations alone or in combination with a constitutively activated SHH signalling pathway, transgenic mice were used. These mice show mutated variants within Smo, Ptch1 or Pik3ca genes in cerebellar granule neuron precursors, which represent the cellular origin of SHH MB. Our results show that Pik3ca mutations alone are insufficient to cause developmental alterations or to initiate MB. However, they significantly accelerate the growth of Shh MB, induce tumour spread throughout the cerebrospinal fluid, and result in lower survival rates of mice with a double Pik3caH1047R/SmoM2 or Pik3caH1047R/Ptch1 mutation. Therefore, PIK3CA mutations in SHH MB may represent a therapeutic target for first and second line combination treatments., Competing Interests: Declaration of competing interest The authors disclose no conflicts of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

A woman in her late sixties was referred to the orthopaedic clinic with progressive lower limb weakness and gait disturbance. She was known to have breast cancer with pre-existing infiltrative disease in the left brachial plexus. Magnetic resonance imaging of the spine revealed an intramedullary spinal cord metastasis in the lower cervical cord at C6-C7. She underwent surgical excision but died within six weeks of surgery. This rare case of an intramedullary spinal cord metastasis highlights the extremely poor prognosis in this condition as well as the possibility of perineural invasion into the spinal cord from the brachial plexus lesion. A detailed discussion of the literature on intramedullary spinal cord metastases is also presented.

Ease of access to vast amounts of information presents significant opportunities and challenges for nurses in the community as they seek to base their practice on the best available evidence. Growing expectations around evidence-based practice have developed alongside developments in evidence synthesis, which adopts robust approaches to identifying, appraising and synthesising key evidence for clinical decision-making. The context in which evidence-based practice occurs is key, and this article discusses the skills and knowledge needed for community nurses to discern how evidence and information should influence their decisions to review and change approaches to clinical practice. Importantly, if nurses understand the status of evidence underpinning areas of practice, they can ensure that the preferences and needs of patients and families are met.

Objective: Myxopapillary ependymomas (MPEs) in children are rarely reported low-grade glial tumors; however, MPEs sometimes possess malignant characteristics such as spinal seeding/drop metastasis (SSM). We aimed to present 3 pediatric MPE cases that experienced SSM at 2 neurosurgical centers., Materials and Methods: We retrospectively reviewed the medical records of 38 primary spinal MPE cases who underwent surgery at 2 neurosurgical centers spanning 16 years, from 2004 to 2019. All pediatric cases (patient age <18 years) who were diagnosed with MPE and re-presented with SSM were selected as the core sample for this study. Relevant literature was briefly reviewed., Results: Three pediatric MPE cases (2 females and 1 male) experienced SSM. The mean age at first presentation was 12.0 ± 1.0 years. The mean preoperative course was 2.9 ± 1.2 months. The predominant location was the lumbar spine in 2 tumors (both originated from terminal filum [TF]). Two tumors were located intradural intramedullary. Gross-total resection was achieved in 2 patients. No patient had neurofibromatosis type 2. No adjuvant treatment was given after the first surgery. The mean period between the first diagnosis and diagnosis of SSM was 44.0 ± 31.5 months. The location of SSM in all patients was the sacral spine (1 patient experienced distant metastasis in her brain besides her sacral metastasis). The mean follow-up was 68.3 ± 53.7 months., Conclusions: We found a statistically significant relationship between SSM in pediatric MPEs and the intramedullary location, TF origin, and number of affected segments. Close clinical and radiological follow-up is essential for pediatric MPE patients., (© 2020 S. Karger AG, Basel.)

We report a case of 46-year-old male operated on for moderately differentiated lung adenocarcinoma. Postoperatively, he underwent six courses of chemotherapy and radiotherapy. He developed progressive severe inferior paraparesis accompanied by excruciating pain between the shoulders two years later. Magnetic resonance imaging revealed metastases in the bodies of T2 and T3 vertebrae with adjacent intradural extramedullary lesion compressing the spinal cord. The patient underwent surgical decompression and vertebral body cement augmentation that lead to pain relief and partial neurological recovery. The histological examination was consistent with metastases from low differentiated pulmonary adenocarcinoma. Surgical resection of intradural extramedullary metastasis improves patient quality of life by reducing pain intensity and neurological deficit., (This is an open access article distributed under the terms of the CC0 Public Domain Dedication.)

Background: The rosette-forming glioneuronal tumor (RGNT) is a rare World Health Organization grade I tumor, most often originating in the fourth ventricle and characterized by a predominant glial component, as well as a neurocytic component. Despite its benign grading, there are a few documented cases of RGNT displaying more aggressive behavior with malignant characteristics., Case Description: Herein, we describe a uniquely aggressive presentation of RGNT in a 49-year-old man initially presenting with obstructive hydrocephalus with lesions in the right frontal lobe, fourth ventricle, and left lateral ventricle. The patient was found to have 2 distinct intracranial entities: a right frontal meningioma and disseminated intraventricular RGNT. He underwent an anatomically limited resection of the fourth ventricular lesion with fourth ventricle-to-subarachnoid space shunting. His disease progressed thereafter, with diffuse intracranial dissemination and drop metastases in the lumbar spine. He was treated with craniospinal radiation and remains on palliative care 5 months after completion of radiotherapy., Conclusions: This case supplements and complements the handful of published cases available documenting the evolution of a particularly aggressive case of disseminated RGNT., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Antiepileptic drug (AED) induced dyskinesia is an unusual manifestation in the medical field. In the previous case reports describing first generation-AED related involuntary movements, the authors suggested that a plausible cause is pharmacokinetic interactions between two or more AEDs. To date, development of dyskinesia after levetiracetam (LEV) has not been reported., Case Presentation: A 28-year-old woman with a history of brain metastasis from spinal cord glioblastoma presented with several generalized tonic-clonic seizures without restored consciousness. LEV was administered intravenously. Thereafter no more clinical or electroencephalographic seizures were noted on video-EEG monitoring, while chorea movement was observed in her face and bilateral upper limbs., Discussion and Conclusions: To our knowledge, there is no case report of dyskinesia after administration of LEV. Considering the temporal relationship and absence of ictal video-EEG findings, we suggest that development of choreoathetosis was closely associated with the undesirable effects of LEV. We propose that dopaminergic system dysregulation and genetic susceptibility might underlie this unusual phenomenon after LEV treatment.

Objective: To integrate 3-dimensional (3D) intraoperative ultrasound (iUS) data in spinal navigation., Methods: In 11 patients with intradural spinal tumors, 3D-iUS was performed before and after tumor resection. Intraoperative computed tomography (iCT) was used for automatic patient registration for spinal navigation; fiducial-based registration was performed in 1 case. The outlines of the vertebra were defined in preoperative image data by automatic mapping; risk and target structures were segmented manually; all these data were rigidly and if necessary non-rigidly registered with iCT. For 3D-iUS acquisition, tracked convex-shaped transducers (contact surface: 29 x 10 mm; scanning frequency: 10-3.8 MHz or 13-5 MHz) were used., Results: Navigated 3D-iUS was successfully implemented in all cases; 3D-iUS datasets were acquired and could be used as 3D image data for further navigation after iUS scanning. The 3D objects defined in preoperative image data, outlining the vertebra, target and risk structures, could be visualized in the 3D-iUS data. Navigated 3D-iUS allowed to reliably evaluate the extent of resection in all cases and updating of navigation, ensuring high navigational accuracy. The target registration error applying iCT-based automatic registration was 0.78 ± 0.23 mm. The effective dose for iCT was 0.11 ± 0.077 mSv for cervical and 1.75 ± 0.72 mSv for thoracic scans., Conclusions: Using 3D-iUS can be successfully integrated in spinal navigation. Automatic registration applying low-dose iCT and non-linear image registration offers displaying preoperative images in the same orientation as the 3D-iUS scan, as well as visualizing segmented structures in the navigated 3D-iUS data. This greatly facilitates image interpretation. Navigated 3D-iUS provides a possibility for navigation updating and immediate online quality control., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Several anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitors have been developed for the treatment of EML4-ALK-rearranged non-small-cell lung cancer, with the newer generation agents brigatinib, alectinib and lorlatinib showing pronounced central nervous system activities. Intracranial efficacy is an important feature for these agents, as metastatic lesions frequently occur in the central nervous system in the ALK-positive setting. Here, we report on an updated case of a patient who received her diagnosis in 2005 and has had disease progression with new lesions on six occasions over the last 8 years. During the first two progressions, only local recurrence was observed. After that, the lungs stayed clear and the patient progressed exclusively in the brain and spinal cord. Initial treatments consisted of chemotherapy and radiotherapy. In 2012, ALK-directed targeted therapy became available, and crizotinib was administered. The treatment was switched to brigatinib 3 years later because of spinal cord lesions. Brigatinib induced partial remission and was followed by lorlatinib and, later on, alectinib, when new metastases arose in the spinal cord and brain. Each of these drugs promoted complete remission of the recent lesions. In November 2018, imaging showed multiple cerebral metastases. As radiotherapy was not an option because of previous irradiation, and as chemotherapy cannot be expected to be active in the brain, the patient underwent brigatinib rechallenge, which led to partial remission. All of the central nervous system relapses were symptomatic, with symptoms resolved rapidly during treatment. This case of a patient with EML4-ALK-rearranged non-small-cell lung cancer shows that sequential treatment with next-generation ALK tyrosine kinase inhibitors, including rechallenge, can induce profound remission even in heavily pretreated patients, especially if the central nervous system is the site of progression.

Background: metastatic spinal cord compression (MSCC) is an oncology emergency. Prevalence is increasing. Treatment and care are complex and those diagnosed may be faced with life-changing challenges., Aims: to review the impact and management of MSCC in patients with cancer, in order to analyse nursing considerations for supporting patients., Methods: a literature review and thematic analysis of five primary research papers, published between 2009 and 2014., Findings: two themes of prognosis/survival time and independence versus dependence were discovered., Conclusions: the onset of MSCC may result in paralysis and associated loss of independence, impacting on a patient's quality of life. Understanding individuals' prognosis and treatment/care preferences is fundamental for the sensitive, individualised support of patients with MSCC. The findings reinforce the nurses' role in health education, in order to raise awareness of MSCC and promote early diagnosis so that patients maintain function and independence as long as possible. The findings support the need for nurses to be equipped with appropriate communication skills to initiate and engage in sensitive, difficult and proactive conversations with patients and their families, supporting the delivery of humanised care.

Intramedullary spinal cord metastases are rarely encountered in patients suffering from extra - central nervous system primary cancer, with only 2 described cases reported in the literature deriving from esophageal cancer. Intramedullary spinal cord metastases may occur at any level of the spinal cord but cervical location is the most frequent. We report the first case of intramedullary metastasis affecting the thoracic spinal cord from esophageal squamous cell carcinoma in a 35-year-old patient.

Background: Intradural extramedullary spinal metastasis is a relatively rare condition. Furthermore, there are few reports with the initial presentation being a neurological symptom from an intradural metastasis. We report a case of a patient with metastasis to the cauda equina from breast cancer found due to neurological symptoms as the initial presentation., Case Presentation: A 76-year-old Japanese woman who was previously healthy presented to our hospital with bilateral severe buttock and lower extremity pain without any history of injury. A solitary intradural cauda equina mass was found by magnetic resonance imaging at the L2/3 level, and we suspected a schwannoma initially. The patient hoped to undergo surgery due to the severe pain. However, the chest computed tomographic scan obtained to assess the patient's general status showed the suspected breast cancer of the left side and a lung metastasis. Hence, we considered the possibility of cauda equina tumor metastatic from the breast cancer. We performed an L1-3 laminectomy and tumor extirpation. The pathology revealed adenocarcinoma. After surgery, she had relief from pain, and her status remained satisfactory until she died 9 months after surgery., Conclusions: It is difficult to clarify whether the cauda equina tumor is benign or malignant based only on Magnetic resonance imaging findings. Clinicians should consider the possibility of metastasis when planning the surgery for intradural cauda equina tumor extirpation.

Intramedullary spinal cord metastases (ISCMs) of non-small cell lung cancer (NSCLC) constitute a serious if infrequent complication, characterised by rapid progression of neurological deficits, with poor prognosis. We describe a 52-year-old man with ISCMs secondary to lung adenocarcinoma who acquired the T790M mutation of the epidermal growth factor receptor (EGFR) after previous use of a first-generation EGFR tyrosine kinase inhibitor (TKI); he was successfully treated with osimertinib. This is the first report of the use of osimertinib in ISCMs: due to its high central nervous system activity, osimertinib could be useful for treating ISCMs secondary to NSCLC in patients who exhibit the T790M mutation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement., Case Description: We present a 33-year-old woman previously submitted to five brain surgeries to treat a posterior fossa intradural chordoma since December 2012. Currently, she presented almost with right and left hemiplegia (grade 2 bilaterally) and also left oculomotor, adbucent and facial nerve paresis. We performed neuroaxis magnetic resonance MR which disclosed stability of posterior fossa tumor which was previously irradiated. However, there were new intradural lesions at the level of C3, T11/T12 and L4/L5/S1 vertebrae., Discussion: With the advent of contemponaeous surgery, radiotherapy options and even available chemotherapy to treat Chordomas (Imatinib), patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis. Our case illustrates a late (6 years) follow-up presentation of an initial posterior fossa intradural chordoma. It suggests that whole neuraxis involvement may be the final presentation of all patients harbouring chordomas and surviving after adequate initial treatment., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Introduction: Intradural extramedullary (IDEM) metastatic disease is infrequently encountered by spine surgeons and consequently poorly understood. Discovery often corresponds with the onset of neurologic symptoms and no consensus exists regarding the importance of complete resection or anticipated postoperative outcome. We aim to elucidate treatment methodologies that exist in the literature., Case Presentation: We present a unique case of a 57-year-old male with a known history of esophageal adenocarcinoma, including brain and visceral metastases, who presented with cauda equina syndrome. An IDEM metastatic esophageal adenocarcinoma lesion was identified on advanced imaging and biopsy. This was treated operatively without return of neurologic function., Discussion: We reviewed and summarized the existing literature. Trends are highlighted to further guide surgeons treating this unusual metastatic phenomenon., Conclusion: Intradural metastasis is a harbinger of advanced disease with a poor prognosis regardless of the etiology of the primary lesion. There are a number of proposed mechanisms for metastatic spread with little available literature for surgeon guidance. Most authors are advocates of a palliative, decompressive approach., Competing Interests: Conflict of interestAll authors declare that they have no conflict of interest., (© International Spinal Cord Society 2019.)

We report the case of a metastatic lung carcinoma that had metastasized to the thoracic extradural space and penetrated the dura, resulting in a combined extra and intradural spinal tumour. To our knowledge this is the first such report of a metastatic lung adenocarcinoma demonstrating direct dural invasion.

Introduction: Intramedullary spinal cord metastases (ISCMs) are rare lesions affecting the spinal cord in patients with disseminated malignancy. Today, due to increased survival, these lesions are more frequently diagnosed. Literature on survival and neurologic outcomes is sparse. Herein, we describe a single institutional case series on ISCMs reported to date in the English literature., Methods: We retrospectively analyzed the medical records of patients diagnosed with intramedullary metastatic lesions at our institution between 1997 and 2016. We analyzed different approaches to management and factors influencing survival and neurologic outcomes., Results: A total of 70 patients (86 lesions) were analyzed. Most lesions were found in thoracic spinal cord (50%) followed by cervical (34%) and conus medullaris (14%). Mean age at diagnosis was 55.6 ± 10.6 years with 60% (n = 42) being females. Median survival was 104.5 days (range 1-888 days). Twenty-three patients (33%) received conservative management, 39 (56%) received palliative radiotherapy, whereas 8 (11%) underwent surgery with one patient receiving only a biopsy. Age, sex, presence of concomitant brain and other systemic metastasis didn't influence survival. Patients with solitary metastases had longer survival compared to multiple lesions (3.6 vs. 2.2 months, p = 0.01). In patients with solitary lesions without brain metastasis, surgical resection was associated with significantly longer survival (6 months vs. 3 months, p = 0.02)., Conclusion: The overall survival in patients with intramedullary metastasis remains poor. Surgical management may contribute to improved survival and neurologic outcomes in selected patients. Intramedullary metastasis may have a greater role on overall survival compared to systemic metastatic burden.