[en] To evaluate the diagnostic value of MRI, we compared MR images of primary scalp mass with pathological findings. Eight cases in seven patients who had presented primary scalp mass during the previous three years were evaluated by MRI and confirmed with surgical excision and pathologic evaluation. 0.38T(Resonex 4000, USA) MRI was used and all MR images were analysed retrospectively. Eight pathologically confirmed cases consisted of three cases of epidermal cysts, one of steatocystoma simplex, three of neurofibromatosis and one of lymphangioma. All epidermal cysts were located in the occipital area and showed a higher signal intensity than CSF in T1WI, and proton density and variable signal intensity in T2WI. The Steatocystoma showed a signal iniensity similar to fat in T1WI and proton density and a lower signal intensity than fat in T2WI. Neurofibromatosis accounted for three cases in two neurofibromatosis patients;one was located in the right parieto-occipital area and accompanied by hematoma and skull defect; two cases were located in the occipital and posterior cervical area of the same patient. The masses showed a higher signal intensity than muscle in T1WI and hyper or isointensity in T2WI with well enhancement. The lymphangioma showed a signal intensity similar to CSF in all pulse sequences and contained a fat component. MRI is a useful modality for evaluating primary scalp mass

[en] Fibrocystic disease of the breast is a frequent cause of abnormal mammogram and ultrasonogram in patients with breast pain. The differentiation of fibrocystic disease form fibroadenoma or carcinoma is often difficult. The present study was performed to evaluate the mammographic and sonographic features of fibrocystic disease of the breast Mammograms and sonogram s of 45 patients with pathologically proven fibrocystic disease of breast were analyzed and correlated retrospectively with the pathologic specimens. Clinically the patients had palpable breast lumps or focal skin thickening. The patients were aged 19 to 64 years(average, 37 years) Histologic examination of 29 patients with abnormal findings in mammograms and/or sonograms revealed stromal fibrosis in 28(97%), cyst formation in 24(83%), epithelial hyperplasia in 29(90%), apocrine metaplasia in 7 (24%), chronic inflammation in 15 (52%), fibroadenomatous change in 2 (7%), large pseudocyst formation in 2 (7%), calcification in 1 (3%), and/or chondroid maseptol in 1 (3%). Of 43 patients with mammograms, 23 showed no detectable lesion, even in retrospect, because of dense mammograms there were well marginated mass in 7, poorly marginated mass in 4, multiple small nodules in 2, mixed density radio opaque mass in 3, radiolucent mass in one, and asymmetrical dense breast in 3. One case had calcification within the mass, and another one had very high density nodules that showed chondroid metaplasia. In 14 patients with sonograms, theechogenicity was variable ; anechoic in 2, hypoechoic in 8, isoechoic in 3, or mixed echoic in one. The shape of the lesion was round in 2, ovoid in 8, tubular in 2, and irregular in 2. The margin was well marginated in 11 and poorly marginated in 3. Fibrocystic breast disease, which is a frequent cause of breast lumps, has variable findings in mammograms and sonograms. Therefore, fibrocystic disease should be included in the differential diagnosis of breast mass when the typical mammographic and/or sonographic evidence of fibroadenoma or carcinoma is not present

[en] Primary angiitis of the central nervous system (PACNS) is an extremely rare vasculitis of unknown etiology. The purpose of this study was to describe the initial and follow-up magnetic resonance (MR) imaging features of the tumor-mimicking PACNS. We retrospectively reviewed a total of 21 initial and follow-up brain MR images obtained in four patients with biopsy-proven PACNS mimicking brain tumor on MR images during the periods from 1 to 8.1 years. In the initial study, diffusion-weighted imaging (DWI; n=4), MR angiogram (n=4), conventional catheter angiogram (n=3), perfusion MR (n=1), and computed tomography (n=1) and proton MR spectroscopy (MRS; n=2) were included. The lesions of the brain were qualitatively assessed in terms of location, number, size, shape, signal intensity, absence or presence of hemorrhage, enhancement pattern, and changes on the follow-up studies. Initially, the lesion manifested as single suprasellar (n=1) and frontal hemispheric (n=1) mass and as multiple-enhancing lesions in the unilateral supratentorial hemisphere (n=2). A patient showed steno-occlusive lesions in the internal carotid and middle cerebral arteries. DWI, perfusion imaging, and MRS revealed inconsistent findings among the patients. On the follow-up studies, a patient had two relapses but there was either significant decrease in size and extent or disappearance of the lesions with immunosuppressive therapy in all patients. Tumor-mimicking PACNS shows variable features on initial MR images but shows good responses to appropriate immunosuppressive therapy on follow-up MR images. (orig.)

[en] 

Background

High-dose chemotherapy (HDC) and autologous stem-cell transplantation (auto-SCT) are used to improve the survival of children with high-risk brain tumors who have a poor outcome with the standard treatment. This study aims to evaluate the outcome of HDC/auto-SCT with topotecan–thiotepa–carboplatin and melphalan–etoposide–carboplatin (TTC/MEC) regimens in pediatric brain tumors.

Methods

We retrospectively analyzed the data of 33 children (median age 6 years) who underwent HDC/auto-SCT (18 tandem and 15 single) with uniform conditioning regimens.

Results

Eleven patients aged < 3 years at diagnosis were eligible for HDC/auto-SCT to avoid or defer radiotherapy. In addition, nine patients with high-risk medulloblastoma (presence of metastasis and/or postoperative residual tumor ≥ 1.5 cm²), eight with other high-risk brain tumor (six CNS primitive neuroectodermal tumor, one CNS atypical teratoid/rhabdoid tumor, and one pineoblastoma), and five with relapsed brain tumors were enrolled. There were three toxic deaths, and two of which were due to pulmonary complications. The main reason for not performing tandem auto-SCT was due to toxicities and patient refusal. The event-free survival (EFS) and overall survival (OS) rates of all patients were 59.4% and 80.0% at a median follow-up with 49.1 months from the first HDC/auto-SCT, respectively. The EFS/OS rates of patients aged < 3 years at diagnosis, high-risk medulloblastoma, other high-risk brain tumor, and relapsed tumors were 50.0/81.8%, 87.5/85.7%, 66.7/88.9%, and 20.0/60.0%, respectively.

Conclusions

Although tandem HDC/auto-SCT with TTC/MEC regimens showed promising survival rates, treatment modifications are warranted to reduce toxicities. The survival rates with relapsed brain tumors were unsatisfactory despite HDC/auto-SCT, and further study is needed.

[en] The inhibitor of differentiation (ID) genes have been implicated as promoters of tumor progression and metastasis in many human cancers. The current study investigated the expression and functional roles of ID genes in seeding and prognosis of medulloblastoma. ID gene expression was screened in human medulloblastoma tissues. Knockdown of ID3 gene was performed in medulloblastoma cells in vitro. The expression of metastasis-related genes after ID3 knockdown was assessed. The effect of ID3 knockdown on tumor seeding was observed in an animal model in vivo. The survival of medulloblastoma patients was plotted according to the ID3 expression levels. Significantly higher ID3 expression was observed in medulloblastoma with cerebrospinal fluid seeding than tumors without seeding. Knockdown of ID3 decreased proliferation, increased apoptosis, and suppressed the migration of D283 medulloblastoma cells in vitro. In a seeding model of medulloblastoma, ID3 knockdown in vivo with shRNA inhibited the growth of primary tumors, prevented the development of leptomeningeal seeding, and prolonged animal survival. High ID3 expression was associated with shorter survival of medulloblastoma patients, especially in Group 4 medulloblastomas. High ID3 expression is associated with medullolbastoma seeding and is a poor prognostic factor, especially in patients with Group 4 tumors. ID3 may represent the metastatic/ aggressive phenotype of a subgroup of medulloblastoma

[en] The purpose of this study was to investigate the role of ¹⁸F-fluorodeoxyglucose (FDG) PET in detecting high-grade meningioma and predicting the recurrence in patients with meningioma after surgical resection. Fifty-nine patients (27 men and 32 women) with intracranial meningioma who underwent preoperative FDG PET and subsequent surgical resection were enrolled. All patients underwent clinical follow-up for tumor recurrence with a mean duration of 34±20 months. The tumor to gray matter ratio (TGR) of FDG uptake was calculated and a receiver-operating characteristic (ROC) curve of the TGR was drawn to determine the cutoff value of the TGR for detection of high-grade meningioma. Further, univariate analysis with the log-rank test was performed to assess the predictive factors of meningioma recurrence. The TGR in high-grade meningioma (WHO grade II and III) was significantly higher than that in low-grade ones (WHO grade I) (p=0.002) and significantly correlated with the MIB-1 labeling index (r=0.338, p=0.009) and mitotic count of the tumor (r=0.284, p=0.03). The ROC analysis revealed that the TGR of 1.0 was the best cutoff value for detecting high-grade meningioma with a sensitivity of 43%, specificity of 95%, and accuracy of 81%. Of 59 patients, 5 (9%) had a recurrent event. In the log-rank test, the TGR, MIB-1 labeling index, presence of brain invasion, and WHO grade were significantly associated with tumor recurrence. The cumulative recurrence-free survival rate of patients with a TGR of 1.0 or less was significantly higher than that of patients with a TGR of more than 1.0 (p=0.0003) FDG uptake in meningioma was the significant predictive factor of tumor recurrence and significantly correlated with the proliferative potential of the tumor. (orig.)

[en] To assess the association between MR imaging features and major genomic profiles in glioblastoma. Qualitative and quantitative imaging features such as volumetrics and histogram analysis from normalised CBV (nCBV) and ADC (nADC) were evaluated based on both T2WI and CET1WI. The imaging parameters of different genetic profile groups were compared and regression analyses were used for identifying imaging-molecular associations. Progression-free survival (PFS) was analysed by a Kaplan-Meier test and Cox proportional hazards model. An IDH mutation was observed in 18/176 patients, and ATRX loss was positive in 17/158 of the IDH-wt cases. The IDH-mut group showed a larger volume on T2WI and a higher volume ratio between T2WI and CET1WI than the IDH-wt group (p < 0.05). In the IDH-mut group, higher mean nADC values were observed compared with the IDH-wt tumours (p < 0.05). Among the IDH-wt tumours, IDH-wt, ATRX-loss tumours revealed higher 5th percentile nADC values than the IDH-wt, ATRX-noloss tumours (p = 0.03). PFS was the longest in the IDH-mut group, followed by the IDH-wt, ATRX-loss groups and the IDH-wt, ATRX-noloss groups, consecutively (p < 0.05). We found significant associations of PFS with the genetic profiles and imaging parameters. Major genetic profiles of glioblastoma showed a significant association with MR imaging features, along with some genetic profiles, which are independent prognostic parameters for GBM. (orig.)

[en] A majority of high-grade gliomas relapse despite combined surgery, radiotherapy and chemotherapy. There is no consensus on standard treatment for recurrent high-grade gliomas, or defined efficacy of adjuvant re-RT after re-Op. This retrospective study evaluated the benefit and safety of re-RT after re-Op (re-Op/RT).

[en] To prospectively explore the value of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in predicting the progression of enhancing lesions persisting after standard treatment in patients with surgically resected glioblastoma (GBM). Forty-seven GBM patients, who underwent near-total tumorectomy followed by concurrent chemoradiation therapy (CCRT) with temozolomide (TMZ) between May 2014 and February 2016, were enrolled. Twenty-four patients were finally analyzed for measurable enhancing lesions persisting after standard treatment. DCE-MRI parameters were calculated at enhancing lesions. Mann-Whitney U tests and multivariable stepwise logistic regression were used to compare parameters between progression (n = 16) and non-progression (n = 8) groups. Mean K"t"r"a"n"s and v_e were significantly lower in progression than in non-progression (P = 0.037 and P = 0.037, respectively). The 5th percentile of the cumulative K"t"r"a"n"s histogram was also significantly lower in the progression than in non-progression group (P = 0.017). Mean v_e was the only independent predictor of progression (P = 0.007), with a sensitivity of 100%, specificity of 63%, and an overall accuracy of 88% at a cut-off value of 0.873. DCE-MRI may help predict the progression of enhancing lesions persisting after the completion of standard treatment in patients with surgically resected GBM, with mean v_e serving as an independent predictor of progression. (orig.)

[en] The aim of this study is to investigate perfusion characteristics of glioblastoma with an oligodendroglioma component (GBMO) compared with conventional glioblastoma (GBM) using dynamic susceptibility contrast (DSC) perfusion magnetic resonance (MR) imaging and microvessel density (MVD). The study was approved by the institutional review board. Newly diagnosed high-grade glioma patients were enrolled (n = 72; 20 GBMs, 14 GBMOs, 19 anaplastic astrocytomas (AAs), 13 anaplastic oligodendrogliomas (AOs), and six anaplastic oligoastrocytomas (AOAs)). All participants underwent preoperative MR imaging including DSC perfusion MR imaging. Normalized cerebral blood volume (nCBV) values were analyzed using a histogram approach. Histogram parameters were subsequently compared across each tumor subtype and grade. MVD was quantified by immunohistochemistry staining and correlated with perfusion parameters. Progression-free survival (PFS) was assessed according to the tumor subtype. GBMO displayed significantly reduced nCBV values compared with GBM, whereas grade III tumors with oligodendroglial components (AO and AOA) exhibited significantly increased nCBV values compared with AA (p < 0.001). MVD analyses revealed the same pattern as nCBV results. In addition, a positive correlation between MVD and nCBV values was noted (r = 0.633, p < 0.001). Patients with oligodendroglial tumors exhibited significantly increased PFS compared with patients with pure astrocytomas in each grade. In contrast to grade III tumors, the presence of oligodendroglial components in grade IV tumors resulted in paradoxically reduced perfusion metrics and MVD. In addition, patients with GBMO exhibited a better clinical outcome compared with patients with GBM. (orig.)