Simultaneous Visualization of the Thoracic Duct and Blood Vessels Using MRI: A Comparison Between Balanced Turbo-field-echo and Spin-echo.

OBJECTIVE: Magnetic resonance thoracic ductography (MRTD), concomitant with blood vessel imaging, provides useful anatomical information. The purpose of this study was to assess the visibility of the thoracic duct and blood vessels simultaneously by MRTD using balanced turbo-field-echo (bTFE) and turbo spin-echo (TSE). METHODS: MRTDs concomitant with blood vessel imaging on bTFE and TSE were obtained for 10 healthy volunteers with a 1.5T-magnetic resonance unit. Visibility of the thoracic duct, blood vessels in the thoracic region; motion artifacts; and overall image quality were scored by two radiologists using three-to-five-point scales; those were compared between bTFE and TSE. RESULTS: The thoracic duct was generally well-visualized on MRTD sequences. The upper part of the thoracic duct was better visualized on TSE than on bTFE (p < 0.05). The blood vessels were well visualized on bTFE and TSE; the bilateral subclavian arteries and the right subclavian veins were better visualized on TSE than on bTFE (all p < 0.05). Motion artifacts and overall image quality were better on TSE than on bTFE (p = 0.0039 and 0.0020, respectively). CONCLUSION: MRTD concomitant with blood vessel imaging on TSE has better visibility of the thoracic duct and blood vessels than bTFE.

Computed diffusion-weighted imaging with a low-apparent diffusion coefficient-pixel cut-off technique for breast cancer detection.

OBJECTIVE: This study aimed to compare the image quality and diagnostic performance of computed diffusion-weighted imaging (DWI) with low-apparent diffusion coefficient (ADC)-pixel cut-off technique (cDWI cut-off) and actual measured DWI (mDWI). METHODS: Eighty-seven consecutive patients with malignant breast lesions and 72 with negative breast lesions who underwent breast MRI were retrospectively evaluated. Computed DWI with high b-values of 800, 1200, and 1500 s/mm2 and ADC cut-off thresholds of none, 0, 0.3, and 0.6 (×10-3 mm2/s) were generated from DWI with two b-values (0 and 800 s/mm2). To identify the optimal conditions, two radiologists evaluated the fat suppression and lesion reduction failure using a cut-off technique. The contrast between breast cancer and glandular tissue was evaluated using region of interest analysis. Three other board-certified radiologists independently assessed the optimised cDWI cut-off and mDWI data sets. Diagnostic performance was evaluated using receiver operating characteristic (ROC) analysis. RESULTS: When an ADC cut-off threshold of 0.3 or 0.6 (× 10-3 mm2/s) was applied, fat suppression improved significantly (p < .05). The contrast of the cDWI cut-off with a b-value of 1200 or 1500 s/mm2 was better than the mDWI (p < .01). The ROC area under the curve for breast cancer detection was 0.837 for the mDWI and 0.909 for the cDWI cut-off (p < .01). CONCLUSION: The cDWI cut-off provided better diagnostic performance than mDWI for breast cancer detection. ADVANCES IN KNOWLEDGE: Using the low-ADC-pixel cut-off technique, computed DWI can improve diagnostic performance by increasing contrast and eliminating un-suppressed fat signals.

Temperature measurement of intracranial cerebrospinal fluid using diffusion tensor imaging after revascularization surgery in Moyamoya disease.

OBJECTIVE: Brain temperature monitoring using a catheter thermometer has been reported to be a useful technique to predict prognosis in neurosurgery. To investigate the possibility of measuring intracranial cerebrospinal fluid temperature for postoperative monitoring in patients with Moyamoya disease (MMD) after bypass surgery. MATERIALS AND METHODS: This study evaluated fifteen patients with MMD who were indicated for bypass surgery. Diffusion tensor imaging for brain thermometry were performed on a 1.5-T MR scanner. Intracranial cerebrospinal fluid temperature with/without considering the fractional anisotropy component, body temperature, C-reactive protein levels, white blood cell count, and cerebral blood flow measured by 123I-IMP single-photon emission computed tomography were obtained before surgery and 1-3 days after surgery. Pixel values considered to be signal outliers in fractional anisotropy processing were defined as cerebrospinal fluid noise index and calculated. Wilcoxon signed-rank test and effect size were performed to compare the changes before and after revascularization. Spearman's rho correlation coefficient was used to analyze the correlations between each parameter. Statistical significance was defined as p < 0.05. RESULTS: All parameter values became significantly higher compared to those measured before revascularization (p < 0.01 in all cases). The effect sizes were largest for the cerebrospinal fluid temperature with fractional anisotropy processing and for C-reactive protein levels (Rank-biserial correlation = 1.0). The cerebrospinal fluid noise index and cerebrospinal fluid temperatures with fractional anisotropy processing (r = 0.84, p < 0.0001) or without fractional anisotropy processing (r = 0.95, p < 0.0001) showed highly significant positive correlations. Although no significant correlation was observed, cerebrospinal fluid temperatures with fractional anisotropy had small or moderately positive correlations with cerebral blood flow, body temperature, C-reactive protein levels, and white blood cell count (r = 0.37, 0.42, 0.41, and 0.44, respectively; p > 0.05). CONCLUSION: Our findings suggest the possibility of postoperative monitoring for MMD patients by measuring intracranial cerebrospinal fluid temperature with fractional anisotropy processing. Intracranial cerebrospinal fluid temperature might be considered as combined response since cerebrospinal fluid, body temperature, and inflammation are equally correlated.

Quantitative Values from Synthetic MRI Correlate with Breast Cancer Subtypes.

The purpose of this study is to correlate quantitative T1, T2, and proton density (PD) values with breast cancer subtypes. Twenty-eight breast cancer patients underwent MRI of the breast including synthetic MRI. T1, T2, and PD values were correlated with Ki-67 and were compared between ER-positive and ER-negative cancers, and between Luminal A and Luminal B cancers. The effectiveness of T1, T2, and PD in differentiating the ER-negative from the ER-positive group and Luminal A from Luminal B cancers was evaluated using receiver operating characteristic analysis. Mean T2 relaxation of ER-negative cancers was significantly higher than that of ER-positive cancers (p < 0.05). The T1, T2, and PD values exhibited a strong positive correlation with Ki-67 (Pearson's r = 0.75, 0.69, and 0.60 respectively; p < 0.001). Among ER-positive cancers, T1, T2, and PD values of Luminal A cancers were significantly lower than those of Luminal B cancers (p < 0.05). The area under the curve (AUC) of T2 for discriminating ER-negative from ER-positive cancers was 0.87 (95% CI: 0.69−0.97). The AUC of T1 for discriminating Luminal A from Luminal B cancers was 0.83 (95% CI: 0.61−0.95). In conclusion, quantitative values derived from synthetic MRI show potential for subtyping of invasive breast cancers.

Detecting lymph node metastasis of esophageal cancer on dual-energy computed tomography.

BACKGROUND: Using conventional computed tomography (CT), the accurate diagnosis of lymph node (LN) metastasis of esophageal cancer is difficult. PURPOSE: To examine dual-energy CT parameters to predict LN metastasis preoperatively in patients with esophageal cancer. MATERIAL AND METHODS: Twenty-six consecutive patients who underwent dual-energy CT before an esophageal cancer surgery (19 patients with LN metastases) were analyzed. The included LNs had a short-axis diameter of ≥4 mm and were confirmed to be resected on postoperative CT. Their short-axis diameter, CT value, iodine concentration (IC), and fat fraction were measured on early- and late-phase contrast-enhanced dual-energy CT images and compared between pathologically confirmed metastatic and non-metastatic LNs. RESULTS: In total, 51 LNs (34 metastatic and 17 non-metastatic) were included. In the early phase, IC and fat fraction were significantly lower in the metastatic than in the non-metastatic LNs (IC = 1.6 mg/mL vs. 2.2 mg/mL; fat fraction = 20.3% vs. 32.5%; both P < 0.05). Furthermore, in the late phase, IC and fat fraction were significantly lower in the metastatic than in the non-metastatic LNs (IC = 2.0 mg/mL vs. 3.0 mg/mL; fat fraction = 20.4% vs. 33.0%; both P < 0.05). Fat fraction exhibited accuracies of 82.4% and 78.4% on early- and late-phase images, respectively. Conversely, short-axis diameter and CT value on both early- and late-phase images were not significantly different between the metastatic and non-metastatic LNs (P > 0.05). CONCLUSION: Using dual-energy CT images, IC and fat fraction are useful for diagnosing LN metastasis in patients with esophageal cancer.

Usefulness of prone-position computed tomography as preoperative simulation prior to thoracoscopic esophagectomy for thoracic esophageal cancer.

PURPOSE: The study aimed to evaluate the usefulness of prone-position computed tomography (CT) for predicting relevant thoracic procedure outcomes in minimally invasive esophagectomy (MIE) for thoracic esophageal cancer. MATERIALS AND METHODS: A total of 59 patients underwent esophagectomy between May 2019 and December 2020 in Tokai University Hospital. Preoperative CT imaging was conducted with the patient in both the supine and prone positions, and the magnitude of change in the intramediastinal space was calculated. In the 56 patients (94.9%) who had undergone MIE, the effects of such a difference on the surgical outcomes were analyzed. RESULTS: A significant correlation of the magnitude of change in VE (distance between ventral aspect of the vertebral body and the midpoint of the esophagus) with the surgical outcome was revealed in the 17 patients (30.4%) in whom the magnitude of change in VE was over the 75th percentile. That is, in this subgroup, the magnitude of change in VE showed a negative correlation with the thoracic operation time (rs = - 0.57, p = 0.01) and blood loss during the thoracic procedure (rs = - 0.46, p = 0.01). Multivariate analysis identified a magnitude of change in VE ≥ 9 mm (OR = 0.14, p = 0.03) as an independent risk factor for postoperative pneumonia. CONCLUSIONS: This study indicates that preoperative prone-position CT imaging is useful for predicting the level of ease or difficulty of securing an adequate operative field, surgical outcomes, and the risk of postoperative pneumonia in MIE.

Clinical impacts of magnetic resonance thoracic ductography on preventing postoperative chylothorax after thoracoscopic esophagectomy for esophageal cancer.

PURPOSE: The study aimed to determine whether magnetic resonance thoracic ductography (MRTD) is useful for preventing injury to the thoracic duct (TD) during thoracoscopic esophagectomy and for reducing the incidence of postoperative chylothorax. MATERIALS AND METHOD: A total of 389 patients underwent thoracoscopic esophagectomy between September 2009 and February 2019 in Tokai University Hospital. Of them, we evaluated 228 patients who underwent preoperative MRTD (MRTD group) using Adachi's classification and our novel classification (Tokai classification). Then, the clinicopathological factors of the MRTD group (n = 228) were compared with those of the non-MRTD group (n = 161), and comparative analyses were conducted after propensity score matching (PSM). RESULTS: The TD could be visualized by MRTD in 228 patients. The MRTD findings were divided into 9 classifications including normal findings and abnormal TD findings (Adachi classification vs Tokai classification; 5.3% vs 16.2%). After PSM, both groups consisted of 128 patients. The rate of postoperative chylothorax after thoracoscopic esophagectomy was significantly lower in the MRTD group (0.8%) than in the non-MRTD group (6.3%) (p = 0.036). In the multivariate analysis for risk factors for chylothorax, the independent prognostic factors were preoperative therapy and the presence of MRTD. CONCLUSIONS: This study revealed that MRTD was useful for preventing of chylothorax after thoracoscopic esophagectomy for esophageal cancer.

Visibility of bronchial arteries using virtual and advanced virtual monoenergetic imaging.

BACKGROUND: The utility of virtual monoenergetic imaging (VMI) for fine arteries has not been well clarified. PURPOSE: To assess bronchial artery visualization using VMI and noise-optimized advanced VMI (VMI+). MATERIAL AND METHODS: Eighty-seven patients with esophageal cancer underwent computed tomography (CT) using a third-generation dual-source system before surgery. Tube voltages were set to 90 kVp and 150 kVp, respectively. Images were reconstructed using VMI and VMI+ with energy levels of 40-120 keV (in 10-keV increments); composite images equivalent to CT images at 105 kVp were also generated. The CT attenuation value and contrast-to-noise ratio (CNR) of bronchial arteries using VMI and VMI+ were compared with those obtained using composite imaging. Two radiologists subjectively analyzed bronchial artery visualization with reference to the composite image. RESULTS: CT attenuation values for bronchial arteries using VMI at 40-60 keV and VMI+ at 40 keV and 50 keV were significantly higher than those obtained using composite imaging (P < 0.05). CNR using VMI at 40-60 keV was significantly higher than that obtained using composite imaging (P < 0.05), whereas no differences were noted for values obtained using composite imaging between VMI+ at 40 keV and 50 keV. In the subjective analysis, VMI at 40 keV and 50 keV yielded significantly better visibility of bronchial arteries than VMI+ (P < 0.05). CONCLUSION: VMI and VMI+ at low voltages (40-50 keV) may be useful for bronchial artery visualization. VMI+ may be less effective for fine vessels as bronchial artery visualization.

The Visibility of the Terminal Thoracic Duct Into the Venous System Using MR Thoracic Ductography with Balanced Turbo Field Echo Sequence.

RATIONALE AND OBJECTIVES: Magnetic resonance thoracic ductography (MRTD) with balanced turbo field echo (bTFE) can visualize both the thoracic duct and its surrounding vessels. This study aimed to investigate the visibility of the terminal thoracic duct into the venous system in the subclavian region using MRTD with bTFE. MATERIALS AND METHODS: MRTD was performed with bTFE as a preoperative workup comprising respiratory gating on a 1.5-T magnetic resonance system for patients with esophageal cancer. The portion and the number of terminal thoracic ducts into the venous system and preterminal branching in the left subclavian region were assessed using MRTD in 132 patients. The confidence level of the visibility using MRTD was also evaluated. RESULTS: The most frequent terminal portion of the thoracic duct was the jugulovenous angle (92 patients, 69.7%), followed by the subclavian vein (27 patients, 20.5%) and the internal jugular vein (8 patients, 6.1%). Four patients also exhibited double entry of the thoracic duct into the venous system. The preterminal branching was single in 96 patients (72.7%) and multiple in 36 patients (27.3%). The confidence level of the visibility of the thoracic duct using MRTD was absolutely certain in 112 patients (84.8%) and was somewhat certain in 20 patients (15.2%). CONCLUSIONS: MRTD with bTFE is a robust imaging modality to visualize the terminal portion of the thoracic duct into the venous system in the subclavian region.

MR imaging findings in some rare neurological complications of paediatric cancer.

Neurological complications of paediatric cancers are a substantial problem. Complications can be primary from central nervous system (CNS) spread or secondary from indirect or remote effects of cancer, as well as cancer treatments such as chemotherapy and radiation therapy. In this review, we present the clinical and imaging findings of rare but important neurological complications in paediatric patients with cancer. Neurological complications are classified into three phases: pre-treatment, treatment and post-remission. Paraneoplastic neurological syndromes, hyperviscosity syndrome, haemophagocytic lymphohistiocytosis and infection are found in the pre-treatment phase, while Trousseau's syndrome, posterior reversible encephalopathy syndrome and methotrexate neurotoxicity are found in the treatment phase; though some complications overlap between the pre-treatment and treatment phases. Hippocampal sclerosis, radiation induced tumour, radiation induced focal haemosiderin deposition and radiation-induced white matter injury are found in the post-remission phase. With increasingly long survival after treatment, CNS complications have become more common. It is critical for radiologists to recognise neurological complications related to paediatric cancer or treatment. Magnetic resonance imaging (MRI) plays a significant role in the recognition and proper management of the neurological complications of paediatric cancer. TEACHING POINTS: • Neurological complications of paediatric cancer include various entities. • Neurological complications are classified into three phases: pre-treatment, treatment and post-remission. • Radiologists should be familiar with clinical and imaging findings of neurological complications. • MRI features may be characteristic and lead to early diagnosis and proper treatments.

Clinical Significance of New Magnetic Resonance Thoracic Ductography Before Thoracoscopic Esophagectomy for Esophageal Cancer.

BACKGROUND: Preoperative simulation of the thoracic duct using magnetic resonance thoracic ductography (MRTD) would enable a safe lymph node dissection near the thoracic duct and the prevention of chylothorax after an esophagectomy. The aim of this study was to determine whether MRTD is useful for preventing injury to the thoracic duct during surgery and for reducing the incidence of chylothorax after surgery. METHODS: We evaluated 130 patients who underwent preoperative MRTD followed by a thoracoscopic esophagectomy for the treatment of thoracic esophageal cancer between August 2014 and April 2017 (MRTD group). These patients were then compared with 160 patients with esophageal cancer who underwent a thoracoscopic esophagectomy without preoperative MRTD (non-MRTD group). RESULTS: Four patients in the non-MRTD group developed Type IIIB chylothorax (International Consensus on Standardization), while none of the patients in the MRTD group developed Type III chylothorax. Some type of abnormal finding was found during MRTD in 24 patients (18.5%). Among them, 13 patients (10.0%) exhibited abnormal divergence, which was the most frequent finding, followed by 5 patients (3.8%) with window formation and 2 patients (1.5%) with stitch formation. CONCLUSIONS: The present study revealed the frequencies of abnormal findings of the thoracic duct and of patients with false-negative MRTD findings. Injury to the thoracic duct can be avoided through the use of appropriate care during procedures performed in patients with abnormal findings on preoperative MRTD.

Neuroimaging findings in Joubert syndrome with C5orf42 gene mutations: A milder form of molar tooth sign and vermian hypoplasia.

PURPOSE: Little is known regarding neuroimaging-genotype correlations in Joubert syndrome (JBTS). To elucidate one of these correlations, we investigated the neuroimaging findings of JBTS patients with C5orf42 mutations. MATERIALS AND METHODS: Neuroimaging findings in five JBTS patients with C5orf42 mutations were retrospectively assessed with regard to the infratentorial and supratentorial structures on T1-magnetization prepared rapid gradient echo (MPRAGE), T2-weighted images, and color-coded fractional anisotropy (FA) maps; the findings were compared to those in four JBTS patients with mutations in other genes (including three with AHI1 and one with TMEM67 mutations). RESULTS: In C5orf42-mutant patients, the infratentorial magnetic resonance (MR) images showed normal or minimally thickened and minimally elongated superior cerebellar peduncles (SCP), normal or minimally deepened interpeduncular fossa (IF), and mild vermian hypoplasia (VH). However, in other patients, all had severe abnormalities in the SCP and IF, and moderate to marked VH. Supratentorial abnormalities were found in one individual in other JBTS. In JBTS with all mutations, color-coded FA maps showed the absence of decussation of the SCP (DSCP). CONCLUSION: The morphological neuroimaging findings in C5orf42-mutant JBTS were distinctly mild and made diagnosis difficult. However, the absence of DSCP on color-coded FA maps may facilitate the diagnosis of JBTS.

Interhemispheric Lipoma, Callosal Anomaly, and Malformations of Cortical Development: A Case Series.

Intracranial lipomas are rare congenital malformations. The most common type of intracranial lipoma is the interhemispheric lipoma, which is frequently associated with callosal anomalies such as hypogenesis or agenesis of the corpus callosum. In contrast, interhemispheric lipomas are less often accompanied with malformations of cortical development (MCD). We report magnetic resonance imaging findings of three infants with an interhemispheric lipoma, associated with a callosal anomaly, and MCD: two infants with nodular interhemispheric lipoma, agenesis of the corpus callosum, and polymicrogyria, and one infant with interhemispheric curvilinear lipoma, hypoplasia of the corpus callosum, and heterotopias. An association was suggested regarding the occurrence of these malformations.

Age-related changes of susceptibility-weighted imaging in subependymal nodules of neonates and children with tuberous sclerosis complex.

OBJECTIVES: To assess age-related changes in susceptibility-weighted imaging (SWI) and phase images of subependymal nodules (SENs) in neonates and children with tuberous sclerosis complex (TSC). METHODS: Images of 17 children (age range, 0-14years; mean, 5.5years) with TSC were retrospectively assessed. Changes in signals of SENs on SWI and filtered phase images were scored by two experienced radiologists using a three-point scale. The relation was assessed between patients' age and the average scores of SENs. The phase shift of SENs on the filtered phase image was also measured, and the correlation between age and phase shift was evaluated. Calcification in SENs on CT was compared to the finding on SWI and filtered phase image in four children. RESULTS: No change in signal was found in neonates in SENs on both SWI and filtered phase image. There was a positive correlation between the average scores of SENs and patients' age on both SWI and filtered phase image (Spearman ρ=0.64, P=0.006; ρ=0.71, P=0.001, respectively). There was a negative correlation between the patients' age and mean phase shift (Spearman ρ=-0.86, P<0.001). Comparable or slight lower detectability of susceptibility changes was revealed in SENs on SWI and filtered phase image, compared to calcification on CT. CONCLUSIONS: There are age-related changes in susceptibility and phase in SENs, which may reflect the appearance of calcification in children with TSC. No magnetically susceptible changes were detected in neonates, suggesting a lack of calcification.

Intracranial Hemorrhage and Tortuosity of Veins Detected on Susceptibility-weighted Imaging of a Child with a Type IV Collagen α1 Mutation and Schizencephaly.

Type IV collagen α1 (COL4A1) forms a sheet-like network beneath the endothelium and surrounding smooth muscle cells. Associations of mutations in COL4A1 with porencephaly, schizencephaly, and intracranial hemorrhages are known. We report susceptibility-weighted imaging (SWI) findings showing hemorrhages in the peripheral portion of the region of schizencephaly, intraparenchymal hemorrhages, and tortuosity of the intracranial veins in a child with a COL4A1 mutation. SWI findings may be helpful for understanding the possible relationship between schizencephaly and COL4A1 mutations.

Treatment outcomes and late toxicities in patients with embryonal central nervous system tumors.

BACKGROUND: Standard treatment strategies for embryonal central nervous system (CNS) tumors have not yet been established. We treated these tumors using an original chemoradiation therapy protocol; the clinical outcomes and toxicities were retrospectively evaluated. METHODS: Twenty-four patients were enrolled including sixteen with medulloblastoma, four with supratentorial primitive neuroectodermal tumor (sPNET), three with atypical teratoid/rhabdoid tumor, and one with pineoblastoma. Immediately after diagnosis, all patients underwent surgery initially. They were then categorized as high- or average-risk groups independent of tumor type/pathogenesis. The average-risk group included patients who were aged ≥3 years at diagnosis, had non-metastatic disease at diagnosis (M0), and had undergone gross total resection. Other patients were categorized as the high-risk group; this group received more intensive treatment than the average-risk group, including high-dose chemotherapy with autologous stem-cell transplantation. All patients received craniospinal irradiation (CSI). The CSI dose was 23.4 Gy for M0 patients aged ≥5 years, 18 Gy for M0 patients aged <5 years, and 30-36 Gy for all patients with M + disease. The total dose to the primary tumor bed was 54 Gy. RESULTS: The median follow-up time was 73.5 (range, 19-118) months. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 71.1 and 88.9%, respectively in the average-risk group (n = 9) and 66.7 and 71.1%, respectively in the high-risk group (n = 15). The PFS and OS rates were not significantly different between the average- and high-risk groups. In patients with medulloblastoma only, these rates were also not significantly different between the average- and high-risk groups. Three of four patients with sPNET were disease free. The height standard deviation score (SDS) was significantly decreased at the last assessment relative to that at diagnosis (P < 0.0001). The latest median height SDS was -1.6 (range, 0.9 to -4.8), and the latest median full-scale intelligence quotient (FSIQ) score was 86 (range, 59-128). The CSI doses and age at the start of radiation therapy did not influence clinical outcomes, height SDSs, and FSIQ scores. CONCLUSIONS: Our original protocol for patients with embryonal CNS tumors was feasible and yielded favorable clinical outcomes.

Syndromes associated with vascular tumors and malformations: a pictorial review.

Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system has been strongly recommended in recent years because of the need for separate therapeutic measures for patients with vascular tumors and malformations. In the ISSVA classification system, vascular tumors, which are neoplastic, are distinguished from vascular malformations, which are caused by vascular structural anomalies and are not neoplastic, on the basis of the presence or absence of neoplastic proliferation of vascular endothelial cells. It is important that radiologists be familiar with the development, diagnosis, and treatment of vascular tumors and malformations, especially the imaging features of low- and high-flow vascular malformations. Some vascular tumors and malformations develop in isolation, whereas others develop within the phenotype of a syndrome. Syndromes that are associated with vascular tumors include PHACE syndrome. Syndromes that are associated with vascular malformations include Sturge-Weber, Klippel-Trénaunay, Proteus, blue rubber bleb nevus, Maffucci, and Gorham-Stout syndromes, all of which demonstrate low flow, and Rendu-Osler-Weber, Cobb, Wyburn-Mason, and Parkes Weber syndromes, all of which demonstrate high flow. Because imaging findings may help identify such syndromes as systemic, it is important that radiologists familiarize themselves with these conditions.

Primary cerebellar histiocytic sarcoma in a 17-month-old girl.

The authors report on a case of histiocytic sarcoma (HS) in a pediatric patient presenting with a solitary tumor in the cerebellum, with the aim of providing insight into primary HS in the CNS, which is especially rare. A 17-month-old Japanese girl presented with a 2-week history of progressive gait disturbance. Brain MRI revealed a 4.7 × 4.3 × 4.3-cm well-demarcated solitary mass in the right hemisphere of the cerebellum, initially suggestive of medulloblastoma, ependymoma, or anaplastic astrocytoma. On intraoperative inspection the cerebellar tumor showed intensive dural attachment and was subtotally removed. Histological and immunohistochemical findings were consistent with HS. The patient subsequently received chemotherapy, and her preoperative neurological symptoms improved. Primary HS in the CNS usually demonstrates an aggressive clinical course and is currently considered to have a poor prognosis. The possibility of this rare tumor should be included in the differential diagnosis of localized cerebellar tumors in the pediatric age group.

Treatment outcomes, growth height, and neuroendocrine functions in patients with intracranial germ cell tumors treated with chemoradiation therapy.

PURPOSE: We carried out a retrospective review of patients receiving chemoradiation therapy (CRT) for intracranial germ cell tumor (GCT) using a lower dose than those previously reported. To identify an optimal GCT treatment strategy, we evaluated treatment outcomes, growth height, and neuroendocrine functions. METHODS AND MATERIALS: Twenty-two patients with GCT, including 4 patients with nongerminomatous GCT (NGGCT) were treated with CRT. The median age at initial diagnosis was 11.5 years (range, 6-19 years). Seventeen patients initially received whole brain irradiation (median dose, 19.8 Gy), and 5 patients, including 4 with NGGCT, received craniospinal irradiation (median dose, 30.6 Gy). The median radiation doses delivered to the primary site were 36 Gy for pure germinoma and 45 Gy for NGGCT. Seventeen patients had tumors adjacent to the hypothalamic-pituitary axis (HPA), and 5 had tumors away from the HPA. RESULTS: The median follow-up time was 72 months (range, 18-203 months). The rates of both disease-free survival and overall survival were 100%. The standard deviation scores (SDSs) of final heights recorded at the last assessment tended to be lower than those at initial diagnosis. Even in all 5 patients with tumors located away from the HPA, final height SDSs decreased (p = 0.018). In 16 patients with tumors adjacent to the HPA, 8 showed metabolic changes suggestive of hypothalamic obesity and/or growth hormone deficiency, and 13 had other pituitary hormone deficiencies. In contrast, 4 of 5 patients with tumors away from the HPA did not show any neuroendocrine dysfunctions except for a tendency to short stature. CONCLUSIONS: CRT for GCT using limited radiation doses resulted in excellent treatment outcomes. Even after limited radiation doses, insufficient growth height was often observed that was independent of tumor location. Our study suggests that close follow-up of neuroendocrine functions, including growth hormone, is essential for all patients with GCT.