Identifying MRI-based radiomics features capable to assess response to systemic treatment in multiple myeloma (MM) patients. Retrospective analysis of whole-body MR-image data in 67 consecutive stage III MM patients (40 men; mean age, 60.4 years). Bone marrow involvement was evaluated using a standardized MR-imaging protocol consisting of T1w-, short-tau inversion recovery- (STIR-) and diffusion-weighted-imaging (DWI) sequences. Ninety-two radiomics features were evaluated, both in focally and diffusely involved bone marrow. Volumes of interest (VOI) were used. Response to treatment was classified according to International Myeloma Working Group (IMWG) criteria in complete response (CR), very-good and/or partial response (VGPR + PR), and non-response (stable disease (SD) and progressive disease (PD)). According to the IMWG-criteria, response categories were CR (n = 35), VGPR + PR (n = 19), and non-responders (n = 13). On apparent diffusion coefficient (ADC)-maps, gray-level small size matrix small area emphasis (Gray Level Size Zone (GLSZM) small area emphasis (SAE)) significantly correlated with CR (p < 0.001), whereas GLSZM non-uniformity normalized (NUN) significantly (p < 0.008) with VGPR/PR in focal medullary lesions (FL), whereas in diffuse involvement, 1st order root mean squared significantly (p < 0.001) correlated with CR, whereas for VGPR/PR Log (gray-level run-length matrix (GLRLM) Short Run High Gray Level Emphasis) proved significant (p < 0.003). On T1w, GLRLM NUN significantly (p < 0.002) correlated with CR in FL, whereas gray-level co-occurrence matric (GLCM) informational measure of correlation (Imc1) significantly (p < 0.04) correlated with VGPR/PR. For diffuse myeloma involvement, neighboring gray-tone difference matrix (NGTDM) contrast and 1st order skewness were significantly associated with CR and VGPR/PR (p < 0.001 for both). On STIR-images, CR correlated with gray-level co-occurrence matrix (GLCM) Informational Measure of Correlation (IMC) 1 (p < 0.001) in FL and 1st order mean absolute deviation in diffusely involved bone marrow (p < 0.001). VGPR/PR correlated at best in FL with GSZLM size zone NUN (p < 0.019) and in all other involved medullary areas with GLSZM large area low gray level emphasis (p < 0.001). GLSZM large area low gray level emphasis also significantly correlated with the degree of bone marrow infiltration assessed histologically (p = 0.006). GLCM IMC 1 proved significant throughout T1w/STIR sequences, whereas GLSZM NUN in STIR and ADC. MRI-based texture features proved significant to assess clinical and hematological response (CR, VPGR, and PR) in multiple myeloma patients undergoing systemic treatment.

Background Retroperitoneal fibrosis is a rare disease with an incidence of 0–1/100 000 inhabitants per year and is associated with chronic inflammatory fibrosis of the retroperitoneum and the abdominal aorta. This article sheds light on the role of radiological imaging in retroperitoneal fibrosis, names various differential diagnoses and provides an overview of drug and surgical treatment options. Methods A literature search for the keywords “retroperitoneal fibrosis” and “Ormond’s disease” was carried out in the PubMed database between January 1, 1995 and December 31, 2019 (n = 1806). Mainly original papers were selected, but also reviews, in English and German language, with a focus on publications in the last 10 years, without excluding older publications that the authors believe are relevant to the topic discussed in the review (n = 40). Results and Conclusion Ormond’s disease is a rare but important differential diagnosis for nonspecific back and flank pain. Imaging diagnostics using CT or MRI show a retroperitoneal mass, which must be differentiated from lymphoma, sarcoma, multiple myeloma and Erdheim-Chester disease. Patients have an excellent prognosis under adequate therapy. FDG-PET/CT or FDG-PET/MRT should be considered as potential modalities, as hybrid imaging can evaluate both the morphological changes and the inflammation. Key Points:  Citation Format

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BackgroundPontocerebellar hypoplasia type 2 (PCH2) is caused by a defect in the TSEN54-gene and leads to severe and early disruption of brain development, especially of cerebellum and pons. The aim of this work was to quantify the infra- and supratentorial brain growth during postnatal brain development in children with PCH2.MethodsMRI data of 24 children with PCH2 (age 0.02–17 years., 13 females) were analysed volumetrically and compared to images of 24 typically developing age- and gender-matched children. All children with PCH2 had the homozygous p.A307S mutation in the TSEN54-gene. In 5 patients follow-up MRI investigations were available.Images of the children with PCH2 were available either on film (n = 12) or in digital format (n = 21). Images on film were digitalized. Brain structures were manually masked and further adjusted semi-automatically using intensity thresholding to exclude CSF. Volumes of cerebellum, brain stem, and pons were measured, as well as supratentorial brain and frontal lobe volume. For validation of the method part of the digital images were processed as images on film. In addition, intra- and inter-rater variabilities were tested.ResultsChildren with PCH2 showed reduced volumes of all measured brain structures compared to healthy controls. Severely hypoplastic cerebellum, pons and brain stem only slightly increased in size postnatally. Supratentorial brain volume also showed reduced growth compared to the healthy controls. Differences between patients and controls could already be seen at birth but became more significant during childhood. Validation of the method showed high precision and reproducibility.ConclusionsIn a genetically very homogenous group of children with PCH2 severely hypoplastic infratentorial structures, the hallmark of the disease, showed only slight increase in volume postnatally. Supratentorial brain structures, which are considered normal at birth, also showed smaller volumes neonatally and a lower growth rate compared to controls, leading to severe microcephaly. Volume loss, however, could not be observed during the first years of life. This argues for a severe disruption of the cerebellar-cerebral networks during pre- and postnatal development caused by a primary cerebellar dysfunction, rather than postnatal neurodegeneration. The developmental progress in these children, although little, further supports this.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0481-4) contains supplementary material, which is available to authorized users.

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Objective The aim of this study was to evaluate the performance of the automated computed tomography (CT) postprocessing software unfolded rib images for improved detection of both benign and malignant rib lesions during routine diagnostic workup of oncological patients. Materials and Methods One thousand eight in-patients and out-patients (63.66 ± 14.25 years; range, 18.67–95.67 years; 405 females and 603 males), undergoing chest CT between July 2018 to January 2019 at our institution, were retrospectively evaluated. Patients underwent chest CT alone or as part of a whole-body CT staging/restaging. The CT protocol consisted of the following: 120 kV; 100 mAs; matrix, 512 × 512; collimation, 0.6 mm; reconstructed section thickness of 3 mm and 1 mm using a soft tissue spatial resolution kernel (I30f) and a sharp kernel (B70f). Both transversal image data sets were used for “conventional” diagnosis including coronal reformates with 3-mm slice thickness. One-millimeter slice thickness image data sets of all patients were additionally directed from the scanner to a computational server where they were automatically postprocessed to 3-dimensional unfolded ribs. The “unfolding” of the rib using the centerline as an axis allows a synchronous display and rotation of all ribs by mouse scrolling. These postprocessed image data sets were evaluated in a separate reading session (approximately 4 weeks later). The readers had no information about the underlying medical history or clinical presentation. They were asked to record the lesion number, site of involvement along the rib (proximal, body, distal), number of the involved ribs, and the character of the lesion in terms of lytic versus sclerotic versus mixed lytic/sclerotic. The standard of reference was 18F-FDG PET, 68Ga-DOMITATE PET/CT, bone scan, or imaging follow-up (>6 months). Results From a total of 1008 evaluated patients, 763 (73.02%) were hemato-oncologic patients. A total of 104 rib lesions were found by transversal CT image reading, whereas the unfolded rib image reading detected 305 lesions. Eighty-nine were classified malignant, and 202 were classified benign. Detection of malignant rib lesions proved significant both for less than 1 cm (P < 0.02) and more than 1 cm in diameter (P < 0.007). The sensitivity, specificity, positive predictive value, and negative predictive value for detection of malignant rib lesions were 97.7%, 98.5%, 96.6%, and 99% for unfolding ribs, and 76.4%, 100%, 92.7%, and 90.5% for conventional (transversal) image reading, respectively. Detection of sclerotic rib lesions and lesions greater than 1 cm in diameter were significantly better (P < 0.01) for the unfolding rib algorithm. Conclusions The “unfolded rib” reformates are significantly superior for rib lesion detection compared with conventional transversal CT scan reading and should therefore be used in all patients, particularly those with an oncologic background.

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