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1.
Medicine (Baltimore) ; 103(18): e37910, 2024 May 03.
Article in English | MEDLINE | ID: mdl-38701282

ABSTRACT

To illustrate the clinical characteristics and prognostic factors of adult patients pathologically confirmed with brainstem gliomas (BSGs). Clinical data of 40 adult patients pathologically diagnosed with BSGs admitted to Beijing Shijitan Hospital from 2009 to 2022 were recorded and retrospectively analyzed. The primary parameters included relevant symptoms, duration of symptoms, Karnofsky performance status (KPS), tumor location, type of surgical resection, diagnosis, treatment, and survival. Univariate and multivariate analyses were evaluated by Cox regression models. The gliomas were located in the midbrain of 9 patients, in the pons of 14 cases, in the medulla of 5 cases, in the midbrain and pons of 6 cases and invading the medulla and pons of 6 cases, respectively. The proportion of patients with low-grade BSGs was 42.5%. Relevant symptoms consisted of visual disturbance, facial paralysis, dizziness, extremity weakness, ataxia, paresthesia, headache, bucking, dysphagia, dysacousia, nausea, dysphasia, dysosmia, hypomnesia and nystagmus. 23 (57.5%) patients accepted stereotactic biopsy, 17 (42.5%) patients underwent surgical resection. 39 patients received radiotherapy and 34 cases were treated with temozolomide. The median overall survival (OS) of all patients was 26.2 months and 21.5 months for the median progression-free survival (PFS). Both duration of symptoms (P = .007) and tumor grading (P = .002) were the influencing factors for OS, and tumor grading was significantly associated with PFS (P = .001). Duration of symptoms for more than 2 months and low-grade are favorable prognostic factors for adult patients with BSGs.


Subject(s)
Brain Stem Neoplasms , Glioma , Humans , Male , Female , Retrospective Studies , Adult , Brain Stem Neoplasms/therapy , Brain Stem Neoplasms/pathology , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/mortality , Middle Aged , Glioma/pathology , Glioma/therapy , Glioma/mortality , Glioma/diagnosis , Prognosis , Young Adult , Karnofsky Performance Status , Aged
2.
Med J Aust ; 220(10): 533-538, 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38699949

ABSTRACT

INTRODUCTION: The main mission of the Australian and New Zealand Children's Haematology and Oncology Group (ANZCHOG) is to develop and facilitate local access to the world's leading evidence-based clinical trials for all paediatric cancers, including brain tumours, as soon as practically possible. Diffuse intrinsic pontine gliomas (DIPGs) - a subset of a larger group of tumours now termed diffuse midline glioma, H3K27-altered (DMG) - are paediatric brain cancers with less than 10% survival at two years. In the absence of any proven curative therapies, significant recent advancements have been made in pre-clinical and clinical research, leading many to seek integration of novel therapies early into standard practice. Despite these innovative therapeutic approaches, DIPG remains an incurable disease for which novel surgical, imaging, diagnostic, radiation and systemic therapy approaches are needed. MAIN RECOMMENDATIONS: All patients with DIPG should be discussed in multidisciplinary neuro-oncology meetings (including pathologists, neuroradiologists, radiation oncologists, neurosurgeons, medical oncologists) at diagnosis and at relapse or progression. Radiation therapy to the involved field remains the local and international standard of care treatment. Proton therapy does not yield a superior survival outcome compared with photon therapy and patients should undergo radiation therapy with the available modality (photon or proton) at their treatment centre. Patients may receive concurrent chemotherapy or radiation-sensitising agents as part of a clinical trial. Biopsy should be offered to facilitate consideration of experimental therapies and eligibility for clinical trial participation. After radiation therapy, each patient should be managed individually with either observation or considered for enrolment on a clinical trial, if eligible, after full discussion with the family. Re-irradiation can be considered for progressive disease. CHANGES IN MANAGEMENT AS A RESULT OF THE GUIDELINE: Every child diagnosed with DIPG should be offered enrolment on a clinical trial where available. Access to investigational drugs without biological rationale outside the clinical trial setting is not supported. In case of potentially actionable target identification with molecular profiling and absence of a suitable clinical trial, rational targeted therapies can be considered through compassionate access programs.


Subject(s)
Brain Stem Neoplasms , Diffuse Intrinsic Pontine Glioma , Humans , New Zealand , Brain Stem Neoplasms/therapy , Brain Stem Neoplasms/diagnosis , Australia , Child , Diffuse Intrinsic Pontine Glioma/therapy , Diffuse Intrinsic Pontine Glioma/diagnosis
3.
Crit Rev Oncol Hematol ; 196: 104261, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38395241

ABSTRACT

Adult brainstem gliomas (BSGs) are a group of rare central nervous system tumors with varying prognoses and controversial standard treatment strategies. To provide an overview of current trends, a systematic review using the PRISMA guidelines, Class of evidence (CE) and strength of recommendation (SR), was conducted. The review identified 27 studies. Surgery was found to have a positive impact on survival, particularly for focal lesions with CE II SR C. Stereotactic image-guided biopsy was recommended when resective surgery was not feasible with CE II and SR B. The role of systemic treatments remains unclear. Eight studies provided molecular biology data. This review gathers crucial literature on diagnosis and management of adult BSGs. It provides evidence-based guidance with updated recommendations for diagnosing and treating, taking into account recent molecular and genetic advancements. The importance of brain biopsy is emphasized to optimize treatment using emerging genetic-molecular findings and explore potential targeted therapies.


Subject(s)
Brain Neoplasms , Brain Stem Neoplasms , Glioma , Adult , Humans , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/therapy , Brain Stem Neoplasms/pathology , Glioma/diagnosis , Glioma/therapy , Glioma/pathology , Prognosis , Biopsy , Brain Neoplasms/pathology
5.
Rev Neurol (Paris) ; 179(5): 475-480, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37061388

ABSTRACT

Diffuse intrinsic pontine glioma (DIPG) is the most frequent brainstem glioma and the most lethal brain tumor in childhood. Despite transient benefit with radiotherapy, the prognosis of children with this disease remains dismal with severe neurological morbidity and median survival less than 12months. Oncolytic immunovirotherapy is emerging as a potential therapeutic approach in neuro-oncology. The oncolytic adenovirus Delta-24-RGD has shown efficacy in adult patients with recurrent GBM. Our group has demonstrated that Delta-24-RGD has oncolytic activity and triggers immune response in preclinical models of DIPG, and has a synergistic effect with radiotherapy in animal models of this disease. In this scenario, we conducted a first-in-human phase 1 clinical trial to evaluate the safety and efficacy of intratumoral injection of Delta-24-RGD in pediatric patients with newly diagnosed DIPG prior to standard radiotherapy. The study confirmed the feasibility of this treatment with an acceptable safety profile and encouraging efficacy results. Correlative analyses showed a biological activity from Delta-24-RGD in DIPG. Further advanced trials are needed to validate these results. Meanwhile, plenty of opportunities to increase the potential contribution of oncolytic viruses in the management of devastating tumors with no current effective treatment such as DIPG need to be explored and exploited.


Subject(s)
Brain Stem Neoplasms , Glioma , Oncolytic Virotherapy , Adult , Animals , Humans , Child , Oncolytic Virotherapy/methods , Glioma/therapy , Glioma/pathology , Brain Stem Neoplasms/therapy , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Oligopeptides/therapeutic use
6.
J Pediatr Hematol Oncol ; 45(3): e410-e414, 2023 04 01.
Article in English | MEDLINE | ID: mdl-36730386

ABSTRACT

We present 4 children (diagnosed between 1 and 8 y, 3 females and 1 male) with molecularly distinct tectal gliomas (2 KRAS mutant, 1 EGFR mutant, 1 SRGAP3-RAF-1 fusion) that contributes to the growing literature of this uncommonly biopsied tumor. The patient with EGFR R222C mutation had a more severe course, earlier diagnosis, subsequent leptomeningeal metastatic disease, required more aggressive therapies, and died 9 years after diagnosis. Patients with KRAS mutations and SRGAP3-RAF-1 fusion had a more indolent course. Our series expands the molecular phenotype of tectal glioma with the potential for leptomeningeal dissemination. Future studies on establishing genotypic/phenotypic correlation from those who undergo biopsy are needed.


Subject(s)
Brain Neoplasms , Brain Stem Neoplasms , Glioma , Female , Male , Humans , Glioma/genetics , Glioma/pathology , Brain Stem Neoplasms/genetics , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Proto-Oncogene Proteins p21(ras)/genetics , ErbB Receptors/genetics , Mutation , Brain Neoplasms/genetics
7.
Neoplasia ; 37: 100876, 2023 03.
Article in English | MEDLINE | ID: mdl-36709715

ABSTRACT

Diffuse Intrinsic Pontine Glioma (DIPG), now known as Diffuse Midline Glioma (DMG) is a devastating pediatric brain tumor with limited treatment options and a very poor prognosis. Despite more than 250 clinical trials aimed to treat children diagnosed with DMG, no curative therapies currently exist for this patient population. A major obstacle has been the intact blood brain barrier (BBB) which prevents most therapeutics from crossing into the tumor bed. Focused Ultrasound (FUS) is an emerging, noninvasive medical technology which has been shown in both preclinical and clinical research to disrupt the blood brain barrier safely and temporarily. FUS blood brain barrier opening has been studied in combination with chemotherapies in preclinical DMG models, and this technology is now being investigated in clinical trials for the treatment of pediatric brain tumors. Focused ultrasound has additional mechanisms of action, including sonodynamic therapy and radiation sensitization, that hold promise as future DMG therapies as well. This paper, largely based off the proceedings from a workshop held by the Focused Ultrasound Foundation in October of 2021, summarizes the current state of the field of focused ultrasound for DIPG/DMG, including preclinical, technical, and clinical summaries in addition to recommended next steps for continued advancement of the game changing technology of Focused Ultrasound.


Subject(s)
Brain Stem Neoplasms , Child , Humans , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Brain Stem Neoplasms/therapy , Consensus , Blood-Brain Barrier , Prognosis
8.
Cancer Res Treat ; 55(1): 41-49, 2023 Jan.
Article in English | MEDLINE | ID: mdl-35255651

ABSTRACT

PURPOSE: This multicenter retrospective study aimed to investigate clinical, radiologic, and treatment-related factors affecting survival in patients with newly diagnosed diffuse intrinsic pontine glioma (DIPG) treated with radiotherapy. MATERIALS AND METHODS: Patients aged <30 years who underwent radiotherapy as an initial treatment for DIPG between 2000 and 2018 were included; patients who did not undergo magnetic resonance imaging at diagnosis and those with pathologically diagnosed grade I glioma were excluded. We examined medical records of 162 patients collected from 10 participating centers in Korea. The patients' clinical, radiological, molecular, and histopathologic characteristics, and treatment responses were evaluated to identify the prognosticators for DIPG and estimate survival outcomes. RESULTS: The median follow-up period was 10.8 months (interquartile range, 7.5 to 18.1). The 1- and 2-year overall survival (OS) rates were 53.5% and 19.0%, respectively, with a median OS of 13.1 months. Long-term survival rate (≥ 2 years) was 16.7%, and median OS was 43.6 months. Age (< 10 years), poor performance status, treatment before 2010, and post-radiotherapy necrosis were independently associated with poor OS in multivariate analysis. In patients with increased post-radiotherapy necrosis, the median OS estimates were 13.3 months and 11.4 months with and without bevacizumab, respectively (p=0.138). CONCLUSION: Therapeutic strategy for DIPG has remained unchanged over time, and the associated prognosis remains poor. Our findings suggest that appropriate efforts are needed to reduce the occurrence of post-radiotherapy necrosis. Further well-designed clinical trials are recommended to improve the poor prognosis observed in DIPG patients.


Subject(s)
Brain Stem Neoplasms , Diffuse Intrinsic Pontine Glioma , Glioma , Child , Humans , Prognosis , Retrospective Studies , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/radiotherapy , Brain Stem Neoplasms/pathology , Glioma/pathology
9.
Tumori ; 109(3): 269-275, 2023 Jun.
Article in English | MEDLINE | ID: mdl-35708347

ABSTRACT

INTRODUCTION: The H3K27M-mutant diffuse midline glioma (DMG) was first included in the World Health Organization (WHO) Classification of central nervous system (CNS) tumors in 2016, and confirmed in its fifth edition. The biological behavior and dismal prognosis of this tumor resemble diffuse intrinsic pontine gliomas (DIPG). Homogeneously-treated series are rarely reported. METHODS: From 2016 onwards, we treated patients with DMG with radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus re-irradiation at relapse, as already done for DIPG. RESULTS: We treated nine patients, seven females, with a median age at diagnosis of 13 years. Tumor sites were: thalamic in five cases, pontocerebellar in two, pineal in one, and paratrigonal with nodular/leptomeningeal dissemination in one. Three patients were biopsied, and six had partial tumor resections. Central pathological review was always performed. The median time to local progression was 12.7 months, and the median overall survival was 17.8 months. Six patients died of tumor progression, one of cerebral bleeding at progression. Two were alive, one in continuous remission, the other after relapsing, at 38.6 and 46.3 months after diagnosis. Progression-free survival was 33.3% at one year. Overall survival was 88.9%, 33.3% and 22.2% at 1, 2 and 3 years, respectively. CONCLUSIONS: This is a small series of homogeneously-treated DMG patients. The results obtained are comparable with those of DIPG patients. Given the phenotypically- and molecularly-defined setting of DMG and severe outcome in this orphan population, they should be treated and included in registries and protocols of DIPG.


Subject(s)
Brain Stem Neoplasms , Glioma , Female , Humans , Adolescent , Glioma/diagnosis , Glioma/genetics , Glioma/therapy , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/genetics , Brain Stem Neoplasms/therapy , Neoplasm Recurrence, Local/genetics , Prognosis , Vinorelbine
10.
Child Neuropsychol ; 29(6): 934-958, 2023 08.
Article in English | MEDLINE | ID: mdl-36369715

ABSTRACT

Children with Diffuse Intrinsic Pontine Gliomas (DIPG), a malignant brainstem tumor, experience poor prognosis. Because of the disease's rarity and highly aggressive course, there is a dearth of research on cognitive and psychosocial outcomes in this underserved, vulnerable population. However, evaluating effects of the disease and treatment on the cognitive and daily functioning of these patients is important to better understand their specific needs and improve their quality of life. The current longitudinal study administered prospective neuropsychological assessments to children diagnosed with CNS malignancies, including the largest sample of children with DIPG to date (n = 21, mean age = 7.86 years, range = 3-16) in neurocognitive, behavioral, social-emotional, and adaptive functioning at baseline, two weeks post-radiation, and six months later. The results describe population-based, cross-sectional characteristics and within-patient longitudinal changes. Prior to radiation, children with DIPG exhibited significant weaknesses compared to normative samples in both parent-report and performance-based measures of attention, and tests of processing speed and verbal learning/memory. Younger children demonstrated poorer inhibitory control on performance tests and worse parent-reported behavioral regulation, depression, and social withdrawal compared to older children. Six-months post-radiation, older children exhibited poorer socialization than younger children. Longitudinally, children with DIPG exhibited short-term improvements immediately post-radiation in performance-based attention tests and parent-reported behavior, including attention, hyperactivity, behavioral regulation, and executive function. However, these improvements did not persist and significant decline was documented on tests of attention by six months. Clinical implications for professionals working with children with DIPG and recommendations for cognitive remediation and quality of life interventions are provided.


Subject(s)
Brain Stem Neoplasms , Diffuse Intrinsic Pontine Glioma , Glioma , Humans , Child , Adolescent , Infant, Newborn , Glioma/complications , Glioma/radiotherapy , Glioma/diagnosis , Prospective Studies , Brain Stem Neoplasms/radiotherapy , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Quality of Life , Longitudinal Studies , Cross-Sectional Studies
11.
Acta Neurochir (Wien) ; 165(4): 1099-1103, 2023 04.
Article in English | MEDLINE | ID: mdl-36481874

ABSTRACT

BACKGROUND: Brainstem diffuse midline gliomas represent infiltrative and rare pediatric tumors with a dismal prognosis. Surgical biopsy is emerging as a valid technique to define diagnosis and molecular markers for future targeted therapies. METHOD: We describe the key steps of an endoscopic trans-ventricular biopsy of a brainstem diffuse midline glioma and associated ventriculomegaly. The relevant surgical anatomy along with an illustrative video is described. CONCLUSION: The endoscopic third ventriculostomy combined with a punch biopsy of a brainstem diffuse midline glioma associated with ventriculomegaly represent a feasible and low-risk procedure to simultaneously treat incipient hydrocephalus and molecular diagnosis for future treatment and research.


Subject(s)
Brain Stem Neoplasms , Glioma , Hydrocephalus , Neuroendoscopy , Child , Humans , Glioma/diagnosis , Glioma/surgery , Glioma/complications , Neuroendoscopy/methods , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/surgery , Hydrocephalus/surgery , Hydrocephalus/complications , Biopsy
12.
Neoplasia ; 35: 100851, 2023 01.
Article in English | MEDLINE | ID: mdl-36410226

ABSTRACT

Diffuse Midline Glioma (DMG) which includes Diffuse Intrinsic Pontine Glioma (DIPG) is an infiltrative tumor of the midline structures of the central nervous system that demonstrates an aggressive pattern of growth and has no known curative treatment. As these tumors progress, children experience ongoing neurological decline including inability to ambulate, swallow and communicate effectively. We propose that optimal care for patients with DMG should involve a specialized team experienced in caring for the multifaceted needs of these patients and their families. Herein we review the roles and evidence to support early involvement of a specialized interdisciplinary team and outline our views on best practices for these challenging tumors.


Subject(s)
Brain Stem Neoplasms , Glioma , Humans , Child , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/therapy , Brain Stem Neoplasms/pathology , Glioma/diagnosis , Glioma/genetics , Glioma/therapy
13.
Pediatr Dermatol ; 40(2): 345-348, 2023 Mar.
Article in English | MEDLINE | ID: mdl-36411544

ABSTRACT

A 13-year-old girl with a history of diffuse intrinsic pontine glioma (DIPG) suffered from progressively worsening facial ulcerations secondary to paresthesia-induced self-excoriation. She was diagnosed with trigeminal trophic syndrome (TTS) induced by DIPG and struggled to heal her lesions in the background of this excoriation disorder. A multidisciplinary approach that included mood disorder management with sertraline and amitriptyline helped diminish paresthesia, improve her quality of life, and promote healing of the ulcers despite the progression of her DIPG. This case highlights the multifactorial complexity of TTS in pediatric patients and the need for successful management strategies.


Subject(s)
Brain Stem Neoplasms , Diffuse Intrinsic Pontine Glioma , Skin Ulcer , Soft Tissue Injuries , Female , Humans , Child , Adolescent , Paresthesia/diagnosis , Quality of Life , Skin Ulcer/drug therapy , Skin Ulcer/etiology , Wound Healing , Brain Stem Neoplasms/complications , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/therapy
14.
J Clin Neurosci ; 106: 8-13, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36228504

ABSTRACT

BACKGROUND: Biopsy of intrinsic brainstem tumours presumed to be diffuse midline gliomas (previously known as DIPG) is controversial. Surgery has risks of injury to the eloquent brainstem and may not have direct benefit to the patient. Technological improvements in operative adjuncts have allowed the role of biopsy for paediatric brainstem lesions to be revisited with new insights. This study aims to evaluate our institutional experience in brainstem biopsy. METHODS: This is an ethics-approved retrospective study based in KK Women's and Children's Hospital. Patients diagnosed with intrinsic brainstem tumours and managed by the Neurosurgical Service were included. Variables of interest included patient demographics, neuroimaging features, type of surgery, histological and molecular diagnosis, treatment, and outcomes. RESULTS: From 2006 to 2021, a total of 27 brainstem intrinsic tumours were referred to the Neurosurgical Service. Eleven (40.7 %) patients underwent stereotactic biopsy and 10 (37 %) had open biopsies. Histologically, 10 (37 %) were confirmed to be high grade gliomas, eight (29.6 %) were low grade gliomas and 3 (11.1 %) were malignant embryonal tumours. No negative diagnostic results or permanent postoperative complications were encountered. Five patients went on to have their tumours interrogated via next-generation sequencing to look for targetable mutations. The remaining 6 (22.2 %) patients did not undergo biopsy, whereby 1 of them is still alive after 6 years. CONCLUSION: Biopsy of paediatric brainstem intrinsic tumours is a safe procedure that concurrs with accurate tissue diagnosis. This option can be offered to affected patients, especially to identify relevant markers for targeted therapy.


Subject(s)
Brain Stem Neoplasms , Glioma , Child , Humans , Female , Retrospective Studies , Singapore , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/genetics , Brain Stem Neoplasms/surgery , Biopsy/methods , Glioma/diagnosis , Glioma/genetics , Glioma/surgery , Hospitals
15.
J Neurooncol ; 160(1): 159-170, 2022 Oct.
Article in English | MEDLINE | ID: mdl-36083426

ABSTRACT

PURPOSE: This study aimed to assess the benefit-risk ratio by determining diagnostic yield and safety of brainstem biopsies in adult patients. The secondary objectives were (i) to compare brainstem biopsy safety and postbiopsy patients' outcomes and survival with those of patients biopsied for a brain or cerebellar lesion, and (ii) to assess the impact of brainstem biopsy on final diagnosis and further therapeutic management. METHODS: Among 1784 stereotactic biopsies performed in adult patients at a tertiary center between April 2009 and October 2020, we retrospectively examined 50 consecutive brainstem biopsies. We compared variables regarding diagnostic yield, safety and post-biopsy outcomes between brainstem biopsy patients and brain/cerebellum biopsy patients. RESULTS: Brainstem biopsy led to a diagnosis in 86% of patients (94.6% in patients with suspected tumor). Lesion contrast enhancement on imaging was the sole predictor of obtaining a diagnosis. Rates of symptomatic complications and mortality were significantly higher in brainstem biopsy patients compared to brain/cerebellum biopsy patients (20% vs 0%; p < 0.001 and 6% vs 0%; p = 0.01, respectively). Transfrontal trajectory and prebiopsy swallowing disorders were predictors of brainstem biopsy-related symptomatic complications. Brainstem biopsy findings led to diagnostic change in 22% of patients. CONCLUSIONS: Stereotactic biopsy in adult patients with brainstem lesion has a high diagnostic yield. Although stereotactic brainstem biopsy is associated with more functional and fatal complications than biopsies targeting the brain/cerebellum, its safety profile appears acceptable. Thus, the benefit-risk ratio of stereotactic biopsy in patients with brainstem lesion is favorable but should nevertheless be carefully weighted on a case-by-case basis.


Subject(s)
Biopsy , Brain Stem Neoplasms , Stereotaxic Techniques , Adult , Humans , Biopsy/adverse effects , Biopsy/methods , Brain Neoplasms/diagnosis , Brain Neoplasms/pathology , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Retrospective Studies , Stereotaxic Techniques/adverse effects , Central Nervous System Diseases/diagnosis , Central Nervous System Diseases/etiology , Central Nervous System Diseases/pathology , Risk Assessment , Treatment Outcome
16.
Curr Oncol ; 29(7): 4558-4565, 2022 06 28.
Article in English | MEDLINE | ID: mdl-35877220

ABSTRACT

Stereotactic frame-based brain tumor biopsy (SFB) is a potent diagnostic tool considering its minimal invasiveness, though its diagnostic power and safety for brainstem lesions remain to be discussed. Here, we aimed to examine the usefulness of SFB for brainstem tumors. Twenty-two patients with brainstem tumors underwent 23 SFBs at our institution during 2002-2021. We retrospectively analyzed patient characteristics, tumor pathology, surgical procedures, and outcomes, including surgery-related complications and the diagnostic value. Seven (32%) tumors were located from the midbrain to the pons, eleven (50%) in the pons only, and four (18%) from the pons to the medulla oblongata. The target lesions were in the middle cerebellar peduncles in sixteen procedures (70%), the cerebellum in four (17%), the inferior cerebellar peduncles in two (9%), and the superior cerebellar peduncles in one (4%). A definitive diagnosis was made in 21 patients (95%) at the first SFB. The diagnoses were glioma in seventeen (77%) cases, primary central nervous system lymphoma in four (18%), and a metastatic brain tumor in one (5%). The postoperative complications (cranial nerve palsy in three [13%] cases, ataxia in one [4%]) were all transient. SFB for brainstem tumors yields a high diagnostic rate with a low risk of morbidity.


Subject(s)
Brain Stem Neoplasms , Stereotaxic Techniques , Biopsy , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Brain Stem Neoplasms/surgery , Humans , Retrospective Studies
17.
Neuro Oncol ; 24(12): 2190-2199, 2022 12 01.
Article in English | MEDLINE | ID: mdl-35552452

ABSTRACT

BACKGROUND: Children ≤36 months with diffuse intrinsic pontine glioma (DIPG) have increased long-term survival (LTS, overall survival (OS) ≥24 months). Understanding distinguishing characteristics in this population is critical to improving outcomes. METHODS: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included. Presentation, clinical course, imaging, pathology and molecular findings were analyzed. RESULTS: Among 1183 patients in IDIPGR, 40 were eligible (median age: 29 months). Median OS was 15 months. Twelve patients (30%) were LTS, 3 (7.5%) very long-term survivors ≥5 years. Among 8 untreated patients, median OS was 2 months. Patients enrolled in the registry but excluded from our study by central radiology review or tissue diagnosis had median OS of 7 months. All but 1 LTS received radiation. Among 32 treated patients, 1-, 2-, 3-, and 5-year OS rates were 68.8%, 31.2%, 15.6% and 12.5%, respectively. LTS had longer duration of presenting symptoms (P = .018). No imaging features were predictive of outcome. Tissue and genomic data were available in 18 (45%) and 10 patients, respectively. Among 9 with known H3K27M status, 6 had a mutation. CONCLUSIONS: Children ≤36 months demonstrated significantly more LTS, with an improved median OS of 15 months; 92% of LTS received radiation. Median OS in untreated children was 2 months, compared to 17 months for treated children. LTS had longer duration of symptoms. Excluded patients demonstrated a lower OS, contradicting the hypothesis that children ≤36 months with DIPG show improved outcomes due to misdiagnosis.


Subject(s)
Astrocytoma , Brain Stem Neoplasms , Glioma , Child, Preschool , Humans , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/genetics , Brain Stem Neoplasms/therapy , Glioma/genetics , Glioma/therapy , Glioma/pathology , Registries
18.
Future Oncol ; 18(16): 1943-1950, 2022 May.
Article in English | MEDLINE | ID: mdl-35193393

ABSTRACT

Background: This study aimed to investigate diffuse intrinsic pontine glioma-specific symptoms in the last 12 weeks before death and to describe current palliative care. Materials & methods: A retrospective study included 80 pediatric diffuse intrinsic pontine glioma patients diagnosed between January 2018 and December 2019. Results: The most frequently encountered symptoms were headache, gait disturbance, vomiting, dysphagia, sensory loss, seizures and constipation. Steroids were used in 96% of patients with a high success rate, as well as analgesics (67.5%), antiemetics (59%), neuropathic medication (42.5%) and anticonvulsants (37.5%). Re-irradiation improved symptoms in 50% of patients. Conclusion: Steroids were efficient in managing many symptoms, with tolerated side effects. The symptomatic treatment succeeded in relieving end-of-life symptoms. Re-irradiation should be considered a good palliative tool in addition to regular symptomatic treatment.


This study aimed to describe symptoms in 80 patients with aggressive brain tumors in the brain stem, as well as proper management of the symptoms at the end of life. The symptoms encountered were headache, imbalance during walking, vomiting and fits. They were managed successfully with steroids, painkillers, nausea and vomiting medications and anti-fit medication. Giving radiotherapy for a second time improved symptoms in 50% of patients, and steroids can be used efficiently to improve the misery of these patients. These data can help to put a strategy for managing these devastating symptoms in the end-of-life period.


Subject(s)
Astrocytoma , Brain Stem Neoplasms , Diffuse Intrinsic Pontine Glioma , Glioma , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/therapy , Child , Death , Glioma/drug therapy , Glioma/therapy , Humans , Palliative Care , Retrospective Studies , Steroids/therapeutic use
19.
Cancer Control ; 28: 10732748211059858, 2021.
Article in English | MEDLINE | ID: mdl-34875878

ABSTRACT

OBJECTIVES: To evaluate the diagnostic yield and safety of brainstem stereotactic biopsy for brainstem lesions. METHODS: We performed a meta-analysis of English articles retrieved from the PubMed, Web of Science, Cochrane Library, and APA psycInfo databases up to May 12, 2021. A binary fixed-effect model, the inverse variance method, or a binary random-effect model, the Dersimonian Laird method, were utilized for pooling the data. This meta-analysis was registered with INPLASY, INPLASY202190034. FINDINGS: A total of 41 eligible studies with 2792 participants were included. The weighted average diagnostic yield was 97.0% (95% confidential interval [CI], 96.0-97.9%). The weighted average proportions of temporary complications, permanent deficits, and deaths were 6.2% (95% CI, 4.5-7.9%), .5% (95% CI, .2-.8%), and .3% (95% CI, .1-.5%), respectively. The subgroup analysis indicated a nearly identical weighted average diagnostic yield between MRI-guided stereotactic biopsy and CT-guided stereotactic biopsy (95.9% vs 95.8%) but slightly increased proportions of temporary complications (7.9% vs 6.0%), permanent deficits (1.9% vs .2%), and deaths (1.1% vs .4%) in the former compared to the latter. Moreover, a greater weighted average diagnostic yield (99.2% vs 97.6%) and lower proportions of temporary complications (5.1% vs 6.8%) and deaths (.7% vs 1.5%) were shown in the pediatric patient population than in the adult patient population. CONCLUSIONS: Brainstem stereotactic biopsy demonstrates striking accuracy plus satisfying safety in the diagnosis of brainstem lesions. The diagnostic yield, morbidity, and mortality mildly vary based on the diversity of assistant techniques and subject populations.


Subject(s)
Biopsy/statistics & numerical data , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/mortality , Early Detection of Cancer/statistics & numerical data , Stereotaxic Techniques/statistics & numerical data , Adult , Biopsy/methods , Brain Stem/pathology , Child , Early Detection of Cancer/methods , Female , Humans , Male
20.
Cancer Med ; 10(21): 7514-7524, 2021 11.
Article in English | MEDLINE | ID: mdl-34510820

ABSTRACT

BACKGROUND: The brainstem has the critical role of regulating cardiac and respiratory function and it also provides motor and sensory function to the face via the cranial nerves. Despite the observation of a brainstem lesion in a radiological examination, it is difficult to obtain tissues for a pathological diagnosis because of the location and small volume of the brainstem. Thus, we aimed to share our 6-year experience with stereotactic biopsies from brainstem lesions and confirm the value and safety of stereotactic biopsy on this highly eloquent area in this study. METHODS: We retrospectively reviewed the medical records of 42 adult patients who underwent stereotactic biopsy on brainstem lesions from 2015 to 2020. The radiological findings, surgical records, pathological diagnosis, and postoperative complications of all patients were analyzed. RESULTS: Histopathological diagnoses were made in 40 (95.2%) patients. Astrocytic tumors were diagnosed in 29 (69.0%) patients, diffuse large B cell lymphoma in 5 (11.9%) patients, demyelinating disease in 4 (9.5%) patients, germinoma in 1 (2.4%) patient, and radiation necrosis in 1 (2.4%) patient. In the 40 patients with successful stereotactic biopsy, 10 (25.0%) patients had inconsistent preoperative radiological diagnosis and postoperative pathological diagnosis. In addition, there was a difference between the treatments prescribed by the radiological and pathological diagnoses in 8 out of 10 patients whose diagnoses changed after biopsy. There was no operative mortality among the 42 patients. CONCLUSIONS: A pathological diagnosis can be made safely and efficiently in brainstem lesions using stereotactic biopsy. This pathological diagnosis will enable patients to receive appropriate treatment.


Subject(s)
Biopsy/methods , Brain Stem Neoplasms/diagnosis , Brain Stem Neoplasms/pathology , Brain Stem/pathology , Stereotaxic Techniques , Adult , Biopsy/adverse effects , Brain Stem/diagnostic imaging , Brain Stem/surgery , Brain Stem Neoplasms/diagnostic imaging , Brain Stem Neoplasms/surgery , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Neuroimaging , Retrospective Studies , Stereotaxic Techniques/adverse effects
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