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Malignant peripheral nerve sheath tumors (MPNSTs) constitute <2% of soft tissue neoplasms in children and display a wide histologic spectrum including low-grade and epithelioid variants, although most are high-grade spindle cell sarcomas. Here, we describe an unusual case of a large retroperitoneal epithelioid MPNST diagnosed in a 7-year-old girl without family history or clinical features of neurofibromatosis type 1. The patient was treated by repeated surgical interventions, polychemotherapy, autologous stem cell transplantation, and irradiation therapy. Over the years, she developed multiple disseminated abdominal recurrences but is currently alive with very slowly progressing disseminated intra-abdominal disease 18 years from initial diagnosis. Histologically, the tumor was composed of medium-sized polygonal and ovoid-to-spindled cells set within a copious myxoid matrix with a prominent reticular and microcystic pattern reminiscent of the recently described reticular/microcystic schwannoma. Immunohistochemistry revealed strong and diffuse expression of S100, CD56, CD57, collagen IV, and neuron-specific enolase, with negativity for perineurial cell markers (claudin-1, epithelial membrane antigen, and glucose transporter-1) and other lineage-specific mesenchymal and epithelial antigens. This unusual variant of low-grade MPNST must be differentiated from a variety of other entities, in particular benign perineurioma, myxoid neurofibroma, and benign reticular/microcystic schwannoma. Confinement of the recurrent disease to the abdominal cavity emphasizes the necessity of primary curative wide excision of this highly recurring but nonmetastasizing low-grade neoplasm.

Objectives: computed tomography (CT) is considered the method of choice in thoracic imaging for a variety of indications. Sedation is usually necessary to enable CT and to avoid deterioration of image quality because of patient movement in small children. We evaluated a new, subsecond high-pitch scan mode (HPM), which obviates the need of sedation and to hold the breath., Material and Methods: a total of 60 patients were included in this study. 30 patients (mean age, 14 ± 17 month; range, 0-55 month) were examined with a dual source CT system in an HPM. Scan parameters were as follows: pitch = 3.0, 128 × 0.6 mm slice acquisition, 0.28 seconds gantry rotation time, ref. mAs adapted to the body weight (50-100 mAs) at 80 kV. Images were reconstructed with a slice thickness of 0.75 mm. None of the children was sedated for the CT examination and no breathing instructions were given. Image quality was assessed focusing on motion artifacts and delineation of the vascular structures and lung parenchyma. Thirty patients (mean age, 15 ± 17 month; range, 0-55 month) were examined under sedation on 2 different CT systems (10-slice CT, n = 18; 64-slice CT, n = 13 patients) in conventional pitch mode (CPM). Dose values were calculated from the dose length product provided in the patient protocol/dose reports, Monte Carlo simulations were performed to assess dose distribution for CPM and HPM., Results: all scans were performed without complications. Image quality was superior with HPM, because of a significant reduction in motion artifacts, as compared to CPM with 10- and 64-slice CT. In the control group, artifacts were encountered at the level of the diaphragm (n = 30; 100%), the borders of the heart (n = 30; 100%), and the ribs (n = 20; 67%) and spine (n = 6; 20%), whereas motion artifacts were detected in the HPM-group only in 6 patients in the lung parenchyma next to the diaphragm or the heart (P < 0,001). Dose values were within the same range in the patient examinations (CPM, 1.9 ± 0.6 mSv; HPM, 1.9 ± 0.5 mSv; P = 0.95), although z-overscanning increased with the increase of detector width and pitch-value., Conclusion: high-pitch chest CT is a robust method to provide highest image quality making sedation or controlled ventilation for the examination of infants, small or uncooperative children unnecessary, whereas maintaining low radiation dose values.

Purpose: To investigate the tolerance of MR examinations in children and adolescents performed in a 1.5 Tesla MR scanner with an expanded bore diameter., Method and Materials: 163 patients, ages 4 to 25, underwent MR examinations in a 1.5 Tesla MR scanner with an open design (MAGNETOM Espree, Siemens, Erlangen, Germany), characterized by a compact length of 125 cm and an expanded 70 cm bore diameter. MR imaging of the brain was carried out in most cases (78.5 %), followed by examinations of the spinal canal (9.8 %), the extremities (9.2 %) and the neck (2.5 %). The patients were divided into four age groups and the success rate, motion artifacts and diagnostic quality of the MR examinations were assessed using a 3-grade scale., Results: In 119 of 163 patients (73.0 %), MR examination was possible without any motion artifacts. With respect to the different age groups, 41.7 % of the 4 - 7-year-old children, 67.6 % of the 8 - 10-year-old children, 84.1 % of the 11 - 16-year-old children and 95.8 % of the patients older than 17 showed tolerance grade I without motion artifacts and excellent diagnostic image quality. In 39 of 163 children (23.9 %), the MR images showed moderate motion artifacts but had sufficient diagnostic quality. With regard to the different age groups, 52.8 % of the 4 - 7-year-old children, 26.5 % of the 8 - 10-year-old children, 15.9 % of the 11 - 16-year-old children and none of the patients older than 17 showed tolerance grade II with moderate motion artifacts and sufficient diagnostic image quality. In only 4 of 124 children < 10 years old and 1 child > 10 years old, the MR examination was not feasible and had to be repeated under sedation., Conclusion: Pediatric MR imaging using a 1.5 Tesla MR scanner with an open design can be conducted in children and adolescents with excellent acceptance. The failure rate of 3.0 % of cases for pediatric MR imaging is comparable to that of a conventional low-field open MR scanner.

Purpose: The VIBE (volume interpolated breath-hold examination) sequence in combination with parallel acquisition technique (iPAT: integrated parallel acquisition technique) allows dynamic contrast-enhanced MRI of the liver with high temporal and spatial resolution. The aim of this study was to obtain first clinical experience with this technique for the detection and characterization of focal liver lesions., Materials and Methods: We examined 10 consecutive patients using a 1.5 T MR system (gradient field strength 30 mT/m) with a phased-array coil combination. Following sequences were acquired: T 2 -w TSE and T 1 -w FLASH, after administration of gadolinium, 6 VIBE sequences with iPAT (TR/TE/matrix/partition thickness/time of acquisition: 6.2 ms/ 3.2 ms/256 x 192/4 mm/13 s), as well as T 1 -weighted FLASH with fat saturation. Two observers evaluated the different sequences concerning the number of lesions and their dignity. Following lesions were found: hepatocellular carcinoma (5 patients), hemangioma (2), metastasis (1), cyst (1), adenoma (1)., Results: The VIBE sequences were superior for the detection of lesions with arterial hyperperfusion with a total of 33 focal lesions. 21 lesions were found with T 2 -w TSE and 20 with plain T 1 -weighted FLASH. Diagnostic accuracy increased with the VIBE sequence in comparison to the other sequences., Conclusion: VIBE with iPAT allows MR imaging of the liver with high spatial and temporal resolution providing dynamic contrast-enhanced information about the whole liver. This may lead to improved detection of liver lesions, especially hepatocellular carcinoma.

Introduction: Besides surgery, treatment of rectal cancer increasingly comprises (neo-)adjuvant strategies. To optimise the selection process for these therapy regimens especially in the field of cellular and molecular biology, new prognostic factors besides the established TNM system are being investigated., Methods: Retrospectively, two groups of patients (n = 2 x 85) with rectal carcinoma curatively treated by surgery alone were studied (median follow-up: 6.1 years). The patients were selected to be free of local disease, in order to exclude surgical influence. Patient groups were matched for age, gender, UICC stage and year of operation (1982-1991) and differed only in subsequent metachronous distant metastatic spread, the criterion to be studied. The factors to be investigated in uni- and multivariate analysis were angiogenesis, density of dendritic cells, grading, venous and lymphatic invasion., Results: Grading always proved to be the only significant prognostic factor (P < 0.0001). In univariate analysis, absent venous invasion also correlated significantly with increased disease-free survival (P = 0.0170)., Conclusions: Angiogenesis and density of dendritic cells in rectal cancer are not prognostic factors for metachronous distant metastasis and, therefore, cannot serve as selection parameters for adjuvant therapy.