Effect of steroid therapy in a 16-year-old girl observed for lymphocytic hypophysitis.

Not required for Clinical Vignette.

Trilateral retinoblastoma: an institutional experience and review of the literature.

BACKGROUND: Retinoblastoma is the most common pediatric intraocular neoplasm. The association of uni- or bilateral retinoblastoma with synchronic or metachronic ectopic midline intracranial tumor [trilateral retinoblastoma (TRB)] is uncommon. MATERIALS AND METHODS: MR examinations of 202 children with retinoblastoma treated at our institute were retrospectively reviewed. MR images and clinical data of children with TRB were evaluated for the patient's age at diagnosis of the intracranial tumor and intraocular lesions, tumor size, signal characteristics, and further course in follow-up MR examinations. RESULTS: There were three patients with TRB in our group of patients. All three children had had a negative family history. Two of them had a primary midline intracranial tumor and intraocular lesions at the time of the first diagnosis. In the third case, the first diagnosis was intracranial midline primitive neuroectodermal tumor. Diagnosis of lesions in both eyes was confirmed in ophthalmologic examination 1 month later. In one case, the intracranial tumor was in the pineal region and, in the other two cases, in the sellar and suprasellar regions. There was no evidence of leptomeningeal spread of the tumors in any patient. CONCLUSIONS: Patients with uni- and bilateral intraocular tumors should receive brain screening by MR imaging. We also recommend that patients under the age of 4 years with midline tumors should be carefully diagnosed for ocular neoplasms.

Usefulness of magnetic resonance imaging with SWI sequence (susceptibility-weighted imaging) in diagnosing cerebral hemosiderosis - case report.

BACKGROUND: The purpose of this study was to determine whether the SWI sequence may improve the MRI evaluation of hemosiderin deposits in the brain. CASE REPORT: We report a case of a 7-year-old girl after a total resection of a large tumor mass (ependymoma G II) located in the left hemisphere. Late complication of surgery was hemosiderosis of the brain diagnosed with SWI sequence. CONCLUSIONS: SWI sequence is very sensitive in diagnosing hemosiderin deposits. It allowed us to recognize cerebral hemosiderosis in this case. We suggest to include this sequence in routine MRI examinations of the brain, especially in cases of potential and suspected intracranial bleeding.

Congenital intramedullary spinal cord tumours: a report of two cases.

We present two different cases of congenital intramedullary tumours, one of a patient in whom treatment was started without pathological confirmation of a malignant tumour and the other of a primitive neuroectodermal tumour. Magnetic resonance imaging is the most useful tool in the diagnosis of malignant intramedullary tumours and differentiation from other types of spinal cord lesions.

Infratentorial tumors in children - value of ADC in prediction of grade of neoplasms.

BACKGROUND: The purpose of this study was to evaluate ADC values in the preoperative grading of primary infratentorial brain tumors in children. MATERIAL /METHODS: We retrospectively reviewed 50 MR examinations of patients with infratentorial tumors. All children were operated on and tumors were histopathologically proved as low-grade - 25 (24 pilocytic astrocytomas, 1 ependymoma) and high-grade lesions - 25 (19 medulloblastomas, 6 anaplastic ependymomas). In all patients with contrast-enhanced tumors, ROIs were placed in the enhanced region. In patients with non-enhancing tumors, ROIs were placed in the solid part of the lesion. Cystic, hemorrhagic and necrotic areas of tumors were excluded. Statistical analysis was performed by using a Student's t-test. RESULTS: Statistically significant differences were found in the comparisons of mean ADC of pilocytic astrocytomas (1.54×10(-3)mm(2)/s ±0.2) with medulloblastomas (0.75×10(-3)mm(2)/s ±0.075) and pilocytic astrocytomas (1.54×10(-3)mm(2)/s ±0.2) with anaplastic ependymomas (0.99×10(-3)mm(2)/s ±0.25). Statistical analysis including ependymomas should be discussed, because of small number of these tumors and a non-homogenous group of lesions. CONCLUSIONS: DWI imaging and ADC map provide useful information for preoperative grading of infratentorial tumors in children.

Cyst-like cortical tubers in patients with tuberous sclerosis complex: MR imaging with the FLAIR sequence.

BACKGROUND: Tuberous sclerosis complex (TSC) is an inherited disorder characterized by the presence of cortical tubers, the majority of which are solid and show high signal on FLAIR images. Low-signal tubers are less frequent. OBJECTIVE: To evaluate cystic cortical tubers in patients with TSC on the basis of their appearance on FLAIR images. MATERIALS AND METHODS: MR examinations of 73 patients were retrospectively reviewed and 17 children (aged 25 days to 12.3 years) with a typical cyst-like tubers were selected for further analysis. The age of the patients, the number of lesions, and the shape and MR intensity were analyzed. RESULTS: Cyst-like cortical tubers were detected in 82% of children below 7 years of age and in 18% of those older then 7 years (P=0.00086). Most of the cystic lesions were of the shrunken type (72%), the expansile type being less frequently seen (28%). CONCLUSIONS: FLAIR images confirm the cystic character of some of the cortical tubers in TSC patients although the pathogenesis of cystic change is still unknown.

[Results of the treatment of paediatric non-rhabdomyosarcoma. One centre experience]. / Wyniki leczenia miesaków tkanek miekkich nie-rhabdomyosarcoma w jednym osrodku.

UNLABELLED: Non-rhabdomyosarcoma soft tissue sarcomas (NR STS) are a rare group of neoplasms of mesenchymal origin. The incidence of these tumours in children is low and due to it's heterogeneity and different response to chemotherapy and radiotherapy, unified treatment methods have not yet been established. THE AIM of our study was to analyze methods and treatment results of patients with NR STS treated in our centre. MATERIALS AND METHODS: Between 1996 and 2004, 64 patients with NR-STS, aged 2.5-21.5 yrs, were treated in our institution. Treatment protocol included primary tumour resection or biopsy, induction (neoadjuvant) chemotherapy, local treatment: surgery and/or radiotherapy and adjuvant chemotherapy. Results of treatment were analyzed in relation to stage, tumour diameter, extent of surgery and response to chemotherapy. RESULTS: Out of 64 patients, 48 are alive (75%), with a median observation time 4 yrs 3 m. Sixteen patients died: 1 of treatment complications, the rest from basic disease. Four years overall (OS) and event free survival (EFS) are 75% and 64% respectively. Early stage, tumour size less than 5 cm in diameter, radical surgery, complete and very good response to induction chemotherapy had a significant influence on survival. CONCLUSIONS: Our results indicate that besides stage and tumour size, radical surgery played key role in the treatment of NRMSSTS and that radical resections were possible to perform after induction chemotherapy in 33% of patients with primarily unresectable tumours. High number of patients with stage IV disease at diagnosis, occurrence of distant relapses and good response to chemotherapy indicate the necessity for the use of chemotherapy in patients with NR STS.

[The value of the proton magnetic resonance spectroscopy (HMRS) of white matter injury in children treated with chemo- and/or radiotherapy. Preliminary report]. / Wartosc spektroskopii protonowej rezonansu magnetycznego (HMRS) w diagnostyce zmian w istocie bialej u dzieci leczonych radio- i/lub chemoterapia. Doniesienie wstepne.

UNLABELLED: THE AIM of this study was to estimate the value of HMRS in the diagnosis of brain lesions observed in children treated with chemo and radiotherapy and to assess the possibility to differentiate these lesions from neoplasm or recurrent disease. MATERIALS AND METHOD: We examined 6 children, aged from 7 to 15 yrs, 3 with brain tumours, 1 with esthesioneuroblastoma treated with chemo and radiotherapy and 2 patients with chemotherapy only, for other neoplasms (leukemia, Wilms tumor). Indications to perform imaging studies were routine for brain tumour patients (no clinical symptoms) and suspicion of disease recurrence in others. MR with 1.5 T scanner and additionally single voxel HMRS in PRESS sequence were performed. RESULTS: In all cases MRI showed extensive hyperintensive changes in brain tissue with significant mass effect. In 3 cases HMRS was within normal limits, in other 3 cases moderately elevated peak of choline and peak of lactate and lipids were found. In follow up examinations all lesions regressed and all patients are in good clinical condition. COMMENTS: It could be difficult to differentiate white matter injury from malignancies on MRI. HMRS in case of doubtful findings on MRI in children treated with chemo and radiotherapy, especially in those without symptoms of their disease, is a helpful method.

[Can proton magnetic resonance spectroscopy be of any value as a prognostic factor in medulloblastoma?]. / Czy protonowa spektroskopia magnetyczna moze miec wartosc prognostyczna w medulloblastoma? Doniesienie wstepne.

UNLABELLED: Proton MR spectroscopy (MRS) is a noninvasive chemical analysis of metabolites in brain tissue. Metabolite ratios are useful because age dependant normal values are known and differ significantly from the values obtained in various diseases of brain tissues. AIM: The aim of our study was to evaluate whether MRS correlates with magnetic resonance imaging (MRI), course of disease, pathology of resected tumours after preoperative chemotherapy and can be of prognostic value in patients with medulloblastoma (MB). MATERIALS AND METHODS: Eight patients with histologically proven MB were studied. All patients underwent MRS and MRI at diagnosis and after neoadjuvant chemotherapy consisting of VCR,VP,CTX,CBCA. Preoperative chemotherapy was followed by surgery, radiotherapy and maintenance chemotherapy. Assessment whether MRI correlates with MRS after preoperative chemotherapy was performed. Additionally MRS was correlated with the course of disease and pathology results. RESULTS: Out of 8 patients 5 had partial remission of their tumours on MRI. Two of them had favorable reaction on MRS defined as elevation of NAA and Cr decrease in Cho,Lac/Lip. Both are alive and disease free. In 2 patients no changes, compared with MRS at diagnosis were found. Both died of the disease. One had an indistinct positive change and died. Stabilization on MRI was observed in 1 patient, who also had a good reaction on MRS. He is alive, disease free. Two patients had disease progression on MRI. Both had poor reaction on MRS, one died, one is alive with disease. It was noticed that good response on MRS correlated with chemotherapy induced tumour changes observed in pathology specimens from resected tumours. Small number of patients does not allow to draw conclusions but our results signal that MRS might be of prognostic value in brain tumours.