Congenital pancreatoblastoma: report of an atypical case and review of the literature.

Pancreatoblastoma is a rare malignant tumor of the pancreas mostly diagnosed in childhood. The clinical presentation and outcome of infantile and congenital pancreatoblastoma have not been clearly elucidated. This report describes our recent institutional experience with an unusual case of congenital pancreatoblastoma. Review of the scientific literature identifies approximately 200 cases of pancreatoblastoma. We describe the 9 infantile (aged 3 mo and younger) and 4 congenital cases previously reported and summarize their clinical presentation and outcome. We also define the close association of infantile/congenital pancreatoblastoma and Beckwith-Wiedemann syndrome (50%) versus all affected age groups (4.5%).

Magnetic resonance and diffusion tensor imaging in pediatric white matter diseases.

The central nervous system undergoes profound and predictable developmental changes during the first few years of life that provide the structural and functional elements necessary for normal neurological development. The establishment and maturation of white matter pathways is a critical component of the developing nervous system. Diffusion tensor imaging (DTI) offers a noninvasive and quantitative means for the evaluation of white matter changes. DTI has contributed to the evaluation of a number of childhood leukoencephalopathies; it has also been used to follow brain maturation in abnormal states, such as premature birth or early brain injury. Furthermore, it has helped characterize the relation between white matter integrity and cognitive abilities. In the future, DTI is expected to play an increasingly large role in defining developmental abnormalities at an early age and in assessing therapies for pediatric disorders such as leukodystrophies.

The effect of prior surgery on blood oxygen level-dependent functional MR imaging in the preoperative assessment of brain tumors.

BACKGROUND AND PURPOSE: Blood oxygen level-dependent functional MR imaging (BOLD fMRI) is a clinically useful technique for preoperative mapping of eloquent cortices in patients with brain tumors. The purpose of this study was to determine the effect on BOLD fMRI accuracy of susceptibility artifacts caused by prior surgery by comparing volumes of activation in the primary motor cortex (PMC) of patients with and without prior brain surgery. METHODS: The volumes of fMRI activation of the PMC were measured for the tumor and nontumor sides in patients with (n = 13) and without (n = 30) prior neurosurgery. Statistical comparisons of the volumes were performed by using paired t tests and linear regression analysis. The location and degree of susceptibility artifact were subjectively assessed. RESULTS: No significant difference was found between the mean tumor and nontumor volumes of fMRI activations in patients without prior surgery (P = .51). In patients who had prior surgery, the volume of activation was significantly smaller on the side of the prior operation when compared with the contralateral side (P = .001). The volume of activation on the side of the tumor was also significantly smaller in the patients with prior surgery compared with those without prior surgery (P < .001). Nevertheless, the PMC was identified in all cases, and its location was confirmed intraoperatively. CONCLUSION: Prior surgery is associated with a decrease in the volume of fMRI activation in patients with prior surgery; however, by examining the T2 images, an astute radiologist can recognize this phenomenon, draw the appropriate conclusions, and correctly identify the PMC.

Functional MRI in the brain tumor patient.

Functional magnetic resonance imaging (fMRI) has been adopted almost universally by disciplines that endeavor to understand how the brain works. As basic scientists tune the technique, clinicians are increasingly able to apply brain mapping with fMRI to their clinical practice. We present here a guide to using fMRI in a clinical setting. We discuss the basic considerations of functional brain mapping in patients with brain tumors including: patient screening and training, paradigm design, data analysis and interpretation of the fMRI scans.

Scapular stress fracture initially masquerading as tumor or infection.

We present a case of a scapular stress fracture in a 17-year-old athletic male that was initially thought to be a neoplasm. Radiographs showed a lesion at the neck of the glenoid with aggressive features. A subsequent MRI and bone scan supported a diagnosis of either tumor or infection. However, before a scheduled bone biopsy, a screening chest CT for pulmonary metastases revealed features of a healing stress fracture. The patient ceased intense athletic activity and four weeks later experienced a continued decrease in shoulder symptoms. A followup shoulder CT confirmed a healing fracture. This case illustrates how a stress fracture at an uncommon location may mimic a neoplasm. CT provides complementary information to radiography and MRI and helps establish a definitive diagnosis.