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Academic Radiology Mar 2024Medulloblastoma (MB) and Ependymoma (EM) in children, share similarities in age group, tumor location, and clinical presentation. Distinguishing between them through...
RATIONALE AND OBJECTIVES
Medulloblastoma (MB) and Ependymoma (EM) in children, share similarities in age group, tumor location, and clinical presentation. Distinguishing between them through clinical diagnosis is challenging. This study aims to explore the effectiveness of using radiomics and machine learning on multiparametric magnetic resonance imaging (MRI) to differentiate between MB and EM and validate its diagnostic ability with an external set.
MATERIALS AND METHODS
Axial T2 weighted image (T2WI) and contrast-enhanced T1weighted image (CE-T1WI) MRI sequences of 135 patients from two centers were collected as train/test sets. Volume of interest (VOI) was manually delineated by an experienced neuroradiologist, supervised by a senior. Feature selection analysis and the least absolute shrinkage and selection operator (LASSO) algorithm identified valuable features, and Shapley additive explanations (SHAP) evaluated their significance. Five machine-learning classifiers-extreme gradient boosting (XGBoost), Bernoulli naive Bayes (Bernoulli NB), Logistic Regression (LR), support vector machine (SVM), linear support vector machine (Linear SVC) classifiers were built based on T2WI (T2 model), CE-T1WI (T1 model), and T1 + T2WI (T1 + T2 model). A human expert diagnosis was developed and corrected by senior radiologists. External validation was performed at Sun Yat-Sen University Cancer Center.
RESULTS
31 valuable features were extracted from T2WI and CE-T1WI. XGBoost demonstrated the highest performance with an area under the curve (AUC) of 0.92 on the test set and maintained an AUC of 0.80 during external validation. For the T1 model, XGBoost achieved the highest AUC of 0.85 on the test set and the highest accuracy of 0.71 on the external validation set. In the T2 model, XGBoost achieved the highest AUC of 0.86 on the test set and the highest accuracy of 0.82 on the external validation set. The human expert diagnosis had an AUC of 0.66 on the test set and 0.69 on the external validation set. The integrated T1 + T2 model achieved an AUC of 0.92 on the test set, 0.80 on the external validation set, achieved the best performance. Overall, XGBoost consistently outperformed in different classification models.
CONCLUSION
The combination of radiomics and machine learning on multiparametric MRI effectively distinguishes between MB and EM in childhood, surpassing human expert diagnosis in training and testing sets.
PubMed: 38508934
DOI: 10.1016/j.acra.2024.02.040 -
Journal of Medical Radiation Sciences Apr 2024Proton beam therapy (PBT) is increasingly used to treat cancers, especially in the paediatric and adolescent and young adult (AYA) population. As PBT becomes more...
Proton beam therapy (PBT) is increasingly used to treat cancers, especially in the paediatric and adolescent and young adult (AYA) population. As PBT becomes more accessible, determining when PBT should be used instead of photon irradiation can be difficult. There is a need to balance patient, tumour and treatment factors when making this decision. Comparing the dosimetry between these two modalities plays an important role in this process. PBT can reduce low to intermediate doses to organs at risk (OAR), but photon irradiation has its dosimetric advantages. We present two cases with brain tumours, one paediatric and one AYA, in which treatment plan comparison between photons and protons showed dosimetric advantages of photon irradiation. The first case was an 18-month-old child diagnosed with posterior fossa ependymoma requiring adjuvant radiotherapy. Photon irradiation using volumetric modulated arc therapy (VMAT) had lower doses to the hippocampi but higher doses to the pituitary gland. The second case was a 21-year-old with an optic pathway glioma. There was better sparing of the critical optic structures and pituitary gland using fractionated stereotactic radiation therapy over PBT. The dosimetric advantages of photon irradiation over PBT have been demonstrated in these cases. This highlights the role of proton-to-photon comparative treatment planning to better understand which patients might benefit from photon irradiation versus PBT.
Topics: Adolescent; Humans; Child; Infant; Young Adult; Adult; Proton Therapy; Radiotherapy Dosage; Radiotherapy Planning, Computer-Assisted; Radiotherapy, Intensity-Modulated; Radiosurgery
PubMed: 38504608
DOI: 10.1002/jmrs.773 -
BMC Cancer Mar 2024Preoperative diagnosis of filum terminale ependymomas (FTEs) versus schwannomas is difficult but essential for surgical planning and prognostic assessment. With the...
PURPOSE
Preoperative diagnosis of filum terminale ependymomas (FTEs) versus schwannomas is difficult but essential for surgical planning and prognostic assessment. With the advancement of deep-learning approaches based on convolutional neural networks (CNNs), the aim of this study was to determine whether CNN-based interpretation of magnetic resonance (MR) images of these two tumours could be achieved.
METHODS
Contrast-enhanced MRI data from 50 patients with primary FTE and 50 schwannomas in the lumbosacral spinal canal were retrospectively collected and used as training and internal validation datasets. The diagnostic accuracy of MRI was determined by consistency with postoperative histopathological examination. T1-weighted (T1-WI), T2-weighted (T2-WI) and contrast-enhanced T1-weighted (CE-T1) MR images of the sagittal plane containing the tumour mass were selected for analysis. For each sequence, patient MRI data were randomly allocated to 5 groups that further underwent fivefold cross-validation to evaluate the diagnostic efficacy of the CNN models. An additional 34 pairs of cases were used as an external test dataset to validate the CNN classifiers.
RESULTS
After comparing multiple backbone CNN models, we developed a diagnostic system using Inception-v3. In the external test dataset, the per-examination combined sensitivities were 0.78 (0.71-0.84, 95% CI) based on T1-weighted images, 0.79 (0.72-0.84, 95% CI) for T2-weighted images, 0.88 (0.83-0.92, 95% CI) for CE-T1 images, and 0.88 (0.83-0.92, 95% CI) for all weighted images. The combined specificities were 0.72 based on T1-WI (0.66-0.78, 95% CI), 0.84 (0.78-0.89, 95% CI) based on T2-WI, 0.74 (0.67-0.80, 95% CI) for CE-T1, and 0.81 (0.76-0.86, 95% CI) for all weighted images. After all three MRI modalities were merged, the receiver operating characteristic (ROC) curve was calculated, and the area under the curve (AUC) was 0.93, with an accuracy of 0.87.
CONCLUSIONS
CNN based MRI analysis has the potential to accurately differentiate ependymomas from schwannomas in the lumbar segment.
Topics: Humans; Retrospective Studies; Cauda Equina; Magnetic Resonance Imaging; Neural Networks, Computer; Neurilemmoma; Ependymoma
PubMed: 38504164
DOI: 10.1186/s12885-024-12023-0 -
Frontiers in Neurology 2024Ependymomas mostly locate in the infratentorial region and often occur in children. Anaplastic ependymomas account for 45-47% of supratentorial and 15-17% of...
BACKGROUND
Ependymomas mostly locate in the infratentorial region and often occur in children. Anaplastic ependymomas account for 45-47% of supratentorial and 15-17% of infratentorial ependymomas, also known as malignant ependymomas. Adult supratentorial extraventricular anaplastic ependymoma (SEAE) is rare in clinical practice, and only a few cases have been reported so far, and there is no clinical study with large sample size. We report a case of adult supratentorial extraventricular anaplastic ependymoma in the occipital lobe with cerebrospinal fluid dissemination metastases.
CASE DESCRIPTION
A 58-year-old female patient presented with unexplained pain in multiple parts of the body for the past half a year, mainly manifested as pain in the head, abdomen and chest. On August, 2022, Head MRI of the patient showed abnormal signal shadow in the left occipital lobe, which was considered a malignant lesion. The patient underwent tumor resection under general anesthesia on September 3, 2022. Postoperative pathological examination showed anaplastic ependymoma. The postoperative follow-up head MRI showed multiple cerebrospinal fluid dissemination metastases in the brain.
CONCLUSION
Adult SEAE is a rare tumor with high malignancy and have a tendency to disseminate into the CSF, resulting in drop metastases. Immunohistochemistry is very important for the diagnosis of SEAE. It is recommended to administer adjuvant chemotherapy or radiation therapy appropriately after surgery, based on the tumor being completely resected as much as possible.
PubMed: 38481945
DOI: 10.3389/fneur.2024.1351674 -
Journal of Neurosciences in Rural... 2024Tanycytic ependymomas mostly occur in the spinal cord and it is the rarest histological subtype of ependymoma. A 29-year-old male was referred from the infertility...
Tanycytic ependymomas mostly occur in the spinal cord and it is the rarest histological subtype of ependymoma. A 29-year-old male was referred from the infertility clinic after serum prolactin levels were found to be elevated. Magnetic resonance imaging (MRI) brain showed an irregular necrotic lesion in the periventricular region of the left parietal lobe which had an intraventricular component and associated perilesional edema. In addition, a sellar mass with suprasellar extension was also found on the MRI. He was started on cabergoline therapy for macroprolactinoma and underwent a left parietal craniotomy, and microsurgical excision of the tumor using intraoperative neurosonographic guidance. Histologically, the tumor showed spindle cytologic features and poorly developed inconspicuous pseudorosettes, with areas of rounded nuclear profiles and perinuclear cytoplasmic clearing. Tumor cells were positive for vimentin, glial fibrillary acidic protein and S100, and negative for epithelial membrane antigen. Ki67 was <7%. He was diagnosed with tanycytic ependymoma and a coexistent prolactinoma. He received 10 cycles of image-guided radiotherapy. Post-operative imaging showed minimal residual tumor the size of which remained stable at 1-year follow-up scan. The pituitary macroadenoma regressed with cabergoline therapy and he clinically improved. This presentation of synchronous macroprolactinoma and tanycytic ependymoma has not been reported in the literature previously. An exhaustive literature review showed only 18 previously reported cases of supratentorial tanycytic ependymoma.
PubMed: 38476418
DOI: 10.25259/JNRP_217_2023 -
Journal of Medical Case Reports Mar 2024Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor...
BACKGROUND
Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management.
CASE PRESENTATION
We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted.
CONCLUSIONS
Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.
Topics: Humans; Male; Middle Aged; Prognosis; Sarcoma, Ewing; Spinal Cord Neoplasms; Spinal Neoplasms
PubMed: 38459600
DOI: 10.1186/s13256-024-04384-8 -
Journal of Pediatric Hematology/oncology Apr 2024Surveillance magnetic resonance imaging (MRI) is routinely used to detect recurrence in pediatric central nervous system (CNS) tumors. The frequency of neuroimaging...
Surveillance magnetic resonance imaging (MRI) is routinely used to detect recurrence in pediatric central nervous system (CNS) tumors. The frequency of neuroimaging surveillance varies without a standardized approach. A single-institutional retrospective cohort study evaluated the frequency of recurrences. This study included 476 patients with the majority diagnosed with low-grade glioma (LGG) (n=138, 29%), high-grade glioma (HGG) (n=77, 16%), ependymoma (n=70, 15%), or medulloblastoma (n=61, 13%). LGG, HGG, and ependymoma patients more commonly had multiply recurrent disease ( P =0.08), with ependymoma patients demonstrating ≥2 relapses in 47% of cases. Recurrent disease was identified by imaging more often than clinical symptoms (65% vs. 32%; P =<0.01). Patients diagnosed with meningioma demonstrated the longest mean time to first relapse (74.7 mo) whereas those with atypical teratoid rhabdoid tumor and choroid plexus carcinoma tended to have the shortest time to relapse (8.9 and 9 mo, respectively). Overall, 22 patients sustained first relapse >10 years from initial diagnosis. With a higher tendency toward detection of tumor recurrence/progression on MRI surveillance in comparison to clinical progression, surveillance imaging is necessary in routine follow up of pediatric CNS tumor survivors. With some relapses >10 years from initial diagnosis, imaging beyond this time point may be useful in particular tumor types. While the study is limited in outcome analysis, earlier detection of recurrence would lead to earlier initiation of treatment and implementation of salvage treatment regimens which can impact survival and quality of life.
Topics: Child; Humans; Retrospective Studies; Quality of Life; Neoplasm Recurrence, Local; Brain Neoplasms; Glioma; Central Nervous System Neoplasms; Ependymoma; Magnetic Resonance Imaging; Cerebellar Neoplasms; Recurrence
PubMed: 38447113
DOI: 10.1097/MPH.0000000000002850 -
Journal of Neuro-oncology May 2024Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion...
PURPOSE
Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion extent of resection (EOR) has been studied, existing analyses are limited by small samples and archaic data resulting in an inhomogeneous population. We investigated the relationship between EOR and survival outcomes, chiefly overall survival (OS) and progression-free survival (PFS), in a large contemporary cohort of spinal ependymoma patients.
METHODS
Adult patients diagnosed with a spinal ependymoma from 2006 to 2021 were identified from an institutional registry. Patients undergoing primary surgical resection at our institution, ≥ 1 routine follow-up MRI, and pathologic diagnosis of ependymoma were included. Records were reviewed for demographic information, EOR, lesion characteristics, and pre-/post-operative neurologic symptoms. EOR was divided into 2 classifications: gross total resection (GTR) and subtotal resection (STR). Log-rank test was used to compare OS and PFS between patient groups.
RESULTS
Sixty-nine patients satisfied inclusion criteria, with 79.7% benefitting from GTR. The population was 56.2% male with average age of 45.7 years, and median follow-up duration of 58 months. Cox multivariate model demonstrated significant improvement in PFS when a GTR was attained (p <.001). Independently ambulatory patients prior to surgery had superior PFS (p <.001) and OS (p =.05). In univariate analyses, patients with a syrinx had improved PFS (p =.03) and were more likely to benefit from GTR (p =.01). Alternatively, OS was not affected by EOR (p =.78).
CONCLUSIONS
In this large, contemporary series of adult spinal ependymoma patients, we demonstrated improvements in PFS when GTR was achieved.
Topics: Humans; Male; Ependymoma; Female; Middle Aged; Adult; Spinal Cord Neoplasms; Neurosurgical Procedures; Progression-Free Survival; Follow-Up Studies; Retrospective Studies; Survival Rate; Young Adult; Aged; Prognosis; Adolescent
PubMed: 38438766
DOI: 10.1007/s11060-024-04623-4 -
Advances in Radiation Oncology Feb 2024Volumetric modulated arc therapy (VMAT) is a relatively new treatment technique in sub-Saharan Africa. Although craniospinal irradiation (CSI) in the pediatric...
PURPOSE
Volumetric modulated arc therapy (VMAT) is a relatively new treatment technique in sub-Saharan Africa. Although craniospinal irradiation (CSI) in the pediatric population has been practiced in Nigeria for many years, the use of VMAT to deliver this treatment is previously undocumented. We reviewed the first set of patients to undergo CSI at a cancer center in Nigeria, detailing the treatment technique, the progress experienced, dose statistics achieved, treatment toxicities, and cancer outcomes to date.
METHODS AND MATERIALS
This was a prospective case series of 5 children with histologically diagnosed cancers requiring CSI whose parents consented to the study. They were recruited at evaluation and followed through the process of their therapy. Toxicity was monitored at weekly review appointments using the Common Terminology Criteria for Adverse Events version 5.0. Follow-up of the children will continue in the long-term effects clinic.
RESULTS
Five patients with a median age of 6 were recruited. Diagnoses were intracranial germ cell tumor (n = 2), medulloblastoma (n = 1), pineoblastoma (n = 1), and ependymoma (n = 1). For all patients, a dose of 36.0 Gy in 1.8 Gy daily fractions was prescribed to the entire neuraxis. A subsequent boost of 18 Gy (n = 4) to 19.8 Gy (n = 1) in 10 daily fractions to the primary tumor bed (n = 2) and posterior fossa (n = 2) was delivered. Four patients had chemotherapy before, during, or after radiation therapy. No patient experienced grade 3 or greater toxicity.
CONCLUSIONS
Our results indicate great progress has been made in the delivery of CSI in Nigeria, demonstrating tolerable acute side effects using VMAT. This series suggests the feasibility of implementing VMAT technology in low- or middle-income countries. Additional follow-up will be needed to determine whether survival rates and chronic toxicity rates are similar to those reported in the literature.
PubMed: 38405304
DOI: 10.1016/j.adro.2023.101325 -
NPJ Precision Oncology Feb 2024Pre-surgery differential diagnosis is valuable for personalized treatment planning in intramedullary spinal cord tumors. This study assessed the performance of...
Pre-surgery differential diagnosis is valuable for personalized treatment planning in intramedullary spinal cord tumors. This study assessed the performance of sequencing cell-free DNA (cfDNA) in cerebrospinal fluid (CSF) for differential diagnosis of these tumors. Prospectively enrolling 45 patients with intramedullary spinal cord lesions, including diffuse midline glioma (DMG), H3K27-altered (14/45), glioblastoma (1/45), H3-wildtype-astrocytoma (10/45), ependymoma (11/45), and other lesions (9/45), CSF samples were collected via lumbar puncture (41/45), intraoperative extraction (3/45), and Ommaya reservoir (1/45). Then, these samples underwent targeted sequencing along with paired tissue DNA. DMG, H3K27-altered patients exhibited a higher ctDNA positivity (85.7%, 12/14) compared to patients with H3-wildtype-astrocytoma (0/8, P = 0.0003), ependymoma (2/10, P = 0.003), and glioneuronal tumor (0/3, P = 0.009). The histological-grade-IV (P = 0.0027), Ki-67 index ≥10% (P = 0.014), and tumor reaching spinal cord surface (P = 0.012) are also associated with higher ctDNA positivity. Interestingly, for patients with TERT promoter mutant tumors, TERT mutation was detectable in the CSF cfDNA of one DMG case, but not other five cases with histological-grade-II tumors. Shared copy number variants were exclusively observed in DMG, H3K27-altered, and showed a strong correlation (Correlation = 0.95) between CSF and tissue. Finally, H3K27M mutations in CSF exhibited high diagnostic efficiency for DMG, H3K27-altered (Sensitivity = 85.7%, Specificity = 100.0%, AUC = 0.929). Notably, H3K27M was detectable in CSF from patients with recurrent tumors, making it easily applicable for postoperative monitoring. In conclusion, the molecular profile from ctDNA released into CSF of malignant tumors was more frequently detected compared to relatively benign ones. Sequencing of ctDNA in CSF exhibited high efficiency for the differential diagnosis of DMG, H3K27-altered.
PubMed: 38388726
DOI: 10.1038/s41698-024-00541-w