Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.
Adult ; Biopsy ; Brain ; Brain Neoplasms ; Cerebrospinal Fluid ; Child ; Choroid Plexus Neoplasms ; Choroid Plexus ; Choroid ; Craniotomy ; Diagnosis ; Drug Therapy ; Edema ; Female ; Fourth Ventricle ; Glioma ; Humans ; Intracranial Pressure ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pathology ; Recurrence ; Spinal Cord

Bilateral thalamic gliomas (BTGs) are rare brain tumors. In general, the prognosis is poor because of the involvement of bilateral thalami and limitations of surgical excision. Consequently, patients with symptoms of personality changes and memory impairment must be differentiated from others. Magnetic resonance imaging (MRI) is essential for the diagnosis of BTGs and reveals a hypo-intense lesion on T1-weighted images and a hyper-intense lesion on T2 images. We report a case of a 17-year-old female patient suffering from progressive cognitive dysfunction and personality changes and subsequent rehabilitation treatment. Brain MRI showed an enlarged bilateral thalamus, with hyperintensity on T2-weighted images and iso-intensity on T1-weighted images. A biopsy was performed, and the pathology revealed a high-grade glioma. The patient was referred for radiotherapy and chemotherapy. She also underwent rehabilitation treatment for 5 weeks and showed improvement in standing balance, endurance, and speech fluency. The patient's Modified Barthel Index scores also improved. Cancer rehabilitation is important in brain tumor patients because they have a higher incidence of neurological sequelae than others. Rehabilitation of patients with a malignant brain tumor is also important for improving health-related quality of life by maintaining the general condition and preventing complications during and after cancer treatment.
Adolescent ; Biopsy ; Brain ; Brain Neoplasms ; Diagnosis ; Drug Therapy ; Female ; Glioma ; Humans ; Incidence ; Magnetic Resonance Imaging ; Memory ; Memory Disorders ; Neurobehavioral Manifestations ; Pathology ; Prognosis ; Quality of Life ; Radiotherapy ; Rehabilitation ; Thalamus

BACKGROUND: Pilocytic astrocytoma (PA) is a brain tumor that is relatively more common in children and young adults. METHODS: We retrospectively reviewed the medical records of patients with PA treated at a single center between 1988 and 2018. RESULTS: We included 31 subjects with PA. The median age at diagnosis was 13.4 years, and the median follow-up duration was 9.9 years. The total PA group had a 10-year disease-specific survival (DSS) rate of 92.6% [95% confidence interval (CI), 82.6–100] and 10-year progression-free survival (PFS) rate of 52.8% (95% CI, 32.0–73.6). In patients aged <20 years, tumors were more likely to be located in sites in which gross total tumor resection (GTR) was impossible. No statistically significant difference in 10-year DSS was found between the GTR (100%) and non-GTR (89.7%; 95% CI, 76.2–100; p=0.374) groups. However, a statistically significant difference in 10-year PFS was found between the GTR (100%) and non-GTR groups (30.7%; 95% CI, 8.6–52.8; p=0.012). In the non-GTR group, no statistically significant difference in 10-year DSS was found between the patients who received immediate additional chemotherapy and/or radiotherapy (Add-Tx group, 92.9%; 95% CI, 79.4–100) and the non-Add-Tx group (83.3%; 95% CI, 53.5–100; p=0.577). No statistically significant difference in 10-year PFS was found between the Add-Tx group (28.9%; 95% CI, 1.7–56.1) and non-Add-Tx group (33.3%; 95% CI, 0–70.9; p=0.706). CONCLUSION: The PFS of the patients with PA in our study depended only on the degree of surgical excision associated with tumor location. This study is limited by its small number of patients and retrospective nature. A multicenter and prospective study is necessary to confirm these findings.
Adolescent ; Astrocytoma ; Brain Neoplasms ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Follow-Up Studies ; Glioma ; Humans ; Medical Records ; Prognosis ; Prospective Studies ; Radiotherapy ; Retrospective Studies ; Survivors ; Young Adult

The abscopal effect is a term that has been used to describe the phenomenon in which localized radiation therapy treatment of a tumor lesion triggers a spontaneous regression of metastatic lesion(s) at a non-irradiated distant site(s). Radiation therapy induced abscopal effects are believed to be mediated by activation and stimulation of the immune system. However, due to the brain’s distinctive immune microenvironment, extracranial abscopal responses following cranial radiation therapy have rarely been reported. In this report, we describe the case of 42-year-old female patient with metastatic melanoma who experienced an abscopal response following her cranial radiation therapy for her brain metastasis. The patient initially presented with a stage III melanoma of the right upper skin of her back. Approximately 5 years after her diagnosis, the patient developed a large metastatic lesion in her upper right pectoral region of her chest wall and axilla. Since the patient’s tumor was positive for BRAF and MEK, targeted therapy with dabrafenib and trametinib was initiated. However, the patient experienced central nervous system (CNS) symptoms such as headache and disequilibrium and developed brain metastases prior to the start of targeted therapy. The patient received radiation therapy to a dose of 30 Gy delivered in 15 fractions to her brain lesions while the patient was on dabrafenib and trametinib therapy. The patient’s CNS metastases improved significantly within weeks of her therapy. The patient’s non-irradiated large extracranial chest mass and axilla mass also shrank substantially demonstrating the abscopal effect during her CNS radiation therapy. Following radiation therapy of her residual chest lesions, the patient was disease free clinically and her CNS lesions had regressed. However, when the radiation therapy ended and the patient continued her targeted therapy alone, recurrence outside of her previously treated fields was noted. The disease recurrence could be due to the possibility of developing BRAF resistance clones to the BRAF targeted therapy. The patient died eventually due to wide spread systemic disease recurrence despite targeted therapy.
Adult ; Axilla ; Brain ; Central Nervous System ; Clone Cells ; Diagnosis ; Female ; Headache ; Humans ; Immune System ; Immunization ; Melanoma ; Molecular Targeted Therapy ; Neoplasm Metastasis ; Radiation, Ionizing ; Recurrence ; Skin ; Skin Neoplasms ; Thoracic Wall ; Thorax

BACKGROUND: Leptomeningeal metastasis (LM) is an uncommon, but devastating complication of advanced cancer and has no standard treatment. Herein, we analyzed the clinical characteristics and outcomes of patients with solid tumors who were diagnosed with LM. METHODS: Between January 2007 and December 2017, we retrospectively analyzed the medical records of patients with solid tumors who were diagnosed with LM. RESULTS: A total of 58 patients were enrolled in this study. The median age of patients was 51 years (range, 27–72 years), and 62.1% had a poor Eastern Cooperative Oncology Group (ECOG) performance status (PS) (>2). The common types of primary tumor were breast cancer (39.7%), gastric cancer (25.9%), and non-small cell lung cancer (20.7%). Forty-two patients (72.4%) were diagnosed with LM by MRI of the brain and/or spine and cerebrospinal fluid (CSF) analysis, 14 were diagnosed by CSF analysis alone, and 2 were diagnosed by MRI alone. Treatments for LM were performed in 53 patients (91.4%), and best supportive care was provided for 5 patients (8.6%). Intrathecal chemotherapy, radiotherapy, and systemic chemotherapy were administered in 43 (74.1%), 17 (29.3%), and 24 (41.4%) patients, respectively. The median overall survival of the entire cohort was 2.4 months (95% confidence interval, 1.0–3.7). In the analysis of prognostic factors for survival, a good ECOG PS (≤2), administration of systemic chemotherapy after LM diagnosis, and a prior history of brain radiation were associated with prolonged survival. CONCLUSION: Although the prognosis of LM in patients with solid tumors is poor, systemic chemotherapy might improve survival in selected patients with a good PS.
Brain ; Breast Neoplasms ; Carcinoma, Non-Small-Cell Lung ; Cerebrospinal Fluid ; Cohort Studies ; Diagnosis ; Drug Therapy ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Meningeal Carcinomatosis ; Neoplasm Metastasis* ; Prognosis ; Radiotherapy ; Retrospective Studies* ; Spine ; Stomach Neoplasms

This study aimed at identifying the frequency and final diagnoses of stroke mimics (SMs) among suspected stroke cases, and seeking differences in clinical & imaging features between SMs and true strokes. It was retrospective with data taken from an ongoing stroke registry for patients admitted to a Stroke Unit between 1 May and 31 October 2011. Baseline characteristics, clinical & imaging features, complications, and outcomes of SMs were compared to those with true strokes by appropriate statistical tests. The total number of cases admitted was 537. Only 232 (43.2%) of them presented during the critical time thrombolytic intervention. SMs comprised 15.9% of all suspected stroke cases. The commonest mimics were psychiatric disorders (43.2%), migraines (16.2%), seizures (8.1%), brain tumors (5.4%), and systemic infections (5.4%). SMs were younger, more common in females, with less evidence of preexisting hypertension (43.2% vs. 56.9% for true strokes). Hemianopia (p = 0.001), sensory inattention, neglect or both (p < 0.001) were more associated with true strokes, while cerebellar, brainstem signs or both (p = 0.045) excluding dysarthria were linked only to SMs. Acute ischemic signs in imaging scans were found linked to true strokes. Some clinical and imaging characteristics were found to differentiate to some extent between SMs and true strokes.
Brain Neoplasms ; Brain Stem ; Diagnosis ; Diagnosis, Differential ; Dysarthria ; Female ; Hemianopsia ; Humans ; Hypertension ; Migraine Disorders ; Retrospective Studies ; Seizures ; Stroke* ; Thrombolytic Therapy

Pancreatic cancer is well known as a relentlessly progressive and fatal disease. Although distant metastasis is common at the time of diagnosis, brain metastasis originating from pancreatic cancer is rare and its clinical manifestation remains poorly described. Additionally, it is generally known that the prognosis for patients with pancreatic cancer and brain metastasis is very poor. Surgical resection of brain metastasis may play a limited role or may allow long-term survival in patients for whom the primary pancreatic cancer is well controlled. We present a case of brain metastasis in patient with pancreatic cancer after pylorus-preserving pancreaticoduodenectomy and complete response to chemotherapy for hepatic metastases. Brain metastasis was resected successfully, but survival period was relatively short, even though the patient received curative treatment for pancreatic cancer. This case demonstrated that resection of brain metastatic lesion from pancreatic cancer being controlled may be helpful, but the timing of resection is also important.
Brain* ; Diagnosis ; Drug Therapy ; Humans ; Neoplasm Metastasis* ; Pancreas ; Pancreatic Neoplasms* ; Pancreaticoduodenectomy ; Prognosis

Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18-year-old woman in the anterior mediastinum.
Adolescent ; Brain ; Diagnosis ; Drug Therapy ; Female ; Hemangioendothelioma ; Hemangioendothelioma, Epithelioid* ; Humans ; Immunohistochemistry ; Incidence ; Liver ; Lung ; Mediastinum* ; Vascular Neoplasms

The prognosis of brain tumors in children has improved for last a few decades. However, the prognosis remains dismal in patients with recurrent brain tumors. The outcome for infants and young children in whom the use of radiotherapy (RT) is very limited because of unacceptable long-term adverse effect of RT remains poor. The prognosis is also not satisfactory when a large residual tumor remains after surgery or when leptomeningeal seeding is present at diagnosis. In this context, a strategy using high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) has been explored to improve the prognosis of recurrent or high-risk brain tumors. This strategy is based on the hypothesis that chemotherapy dose escalation might result in improvement in survival rates. Recently, the efficacy of tandem HDCT/auto-SCT has been evaluated in further improving the outcome. This strategy is based on the hypothesis that further dose escalation might result in further improvement in survival rates. At present, the number of studies employing tandem HDCT/auto-SCT for brain tumors is limited. However, results of these pilot studies suggest that tandem HDCT/auto-SCT may further improve the outcome. In this review, we will summarize our single center experience with tandem HDCT/auto-SCT for recurrent or high-risk brain tumors.
Brain Neoplasms ; Brain ; Child ; Diagnosis ; Drug Therapy ; Humans ; Infant ; Neoplasm, Residual ; Pilot Projects ; Prognosis ; Radiotherapy ; Stem Cell Transplantation ; Stem Cells ; Survival Rate

PURPOSE: This study was conducted to investigate the neurocognitive functioning of children with intracranial germ cell tumor (IGCT) prior to receiving proton beam therapy (PBT), and to identify differential characteristics of their neurocognitive functioning depending on tumor location. As a secondary object of this study, neurocognitive functions were followed up at 1-2 years after PBT to examine early post-treatment changes. MATERIALS AND METHODS: Between 2008 and 2014, 34 childrenwith IGCT treatedwho received PBT atNational Cancer Center, Korea were enrolled in this study. Standardized neurocognitive tests of intelligence, memory, and executive functioning were performed with baseline psychological assessments using the Child Behavior Checklist (CBCL). Follow-up assessments after PBT were conducted in 20 patients (T2). The results were analyzed based on the locations of tumors, which included the suprasellar, pineal gland, basal ganglia, and bifocal regions. RESULTS: The neurocognitive function of IGCT patients was significantly lower than that of the normal population in performance intelligence quotient (p=0.041), processing speed (p=0.007), memory (p < 0.001), and executive functioning (p=0.010). Patients with basal ganglia tumors had significantly lower scores for most domains of neurocognitive functioning and higher scores for CBCL than both the normal population and patients with IGCT in other locations. There was no significant change in neurocognitive function between T1 and T2 for all types of IGCT patients in first 1-2 years after PBT. CONCLUSION: Tumor location significantly affects the neuropsychological functioning in patients with IGCT. Neuropsychological functioning should be closely monitored from the time of diagnosis in IGCT patients.
Basal Ganglia ; Brain Neoplasms ; Checklist ; Child Behavior ; Child* ; Cognition ; Diagnosis ; Follow-Up Studies ; Germ Cells* ; Humans ; Intelligence ; Korea ; Memory ; Neoplasms, Germ Cell and Embryonal* ; Pineal Gland ; Proton Therapy