Primary plasma cell leukemia (pPCL) is a rare, yet aggressive form of de novo plasma cell tumor, distinct from secondary PCL (sPCL) which represents a leukemic transformation of pre‐existing multiple myeloma (MM). Herein, we performed a comprehensive molecular analysis of a prospective series of pPCLs by means of FISH, single nucleotide polymorphism (SNP) array and gene expression profiling (GEP). IGH@ translocations were identified in 87% of pPCL cases, with prevalence of t(11;14) (40%) and t(14;16) (30.5%), whereas the most frequent numerical alterations involved 1p (38%), 1q (48%), 6q (29%), 8p (42%), 13q (74%), 14q (71%), 16q (53%), and 17p (35%). We identified a minimal biallelic deletion (1.5 Mb) in 8p21.2 encompassing the PPP2R2A gene, belonging to a family of putative tumor suppressors and found to be significantly down‐regulated in deleted cases. Mutations of TP53 were identified in four cases, all but one associated with a monoallelic deletion of the gene, whereas activating mutations of the BRAF oncogene occurred in one case and were absent in N‐ and K‐RAS. To evaluate the influence of allelic imbalances in transcriptional expression we performed an integrated genomic analysis with GEP data, showing a significant dosage effect of genes involved in transcription, translation, methyltransferase activity, apoptosis as well as Wnt and NF‐kB signaling pathways. Overall, we provide a compendium of genomic alterations in a prospective series of pPCLs which may contribute to improve our understanding of the pathogenesis of this aggressive form of plasma cell dyscrasia and the mechanisms of tumor progression in MM. Am. J. Hematol. 2013. © 2012 Wiley Periodicals, Inc.

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Single-session SIRT appeared to be as safe and had a similar impact on HRQoL as multiple sessions of TACE, suggesting that SIRT might be an alternative option for patients eligible for TACE.

Retroperitoneal fibrosis (RPF) is a well described clinical entity that is being diagnosed with increasing frequency. RPF is characterized by replacement of the normal tissue of the retroperitoneum with fibrosis and/or chronic inflammation. However, aetiology, clinical presentation and radiological appearance in many cases are protean. Up to 15% of patients have additional fibrotic processes outside the retroperitoneum. In the abdomen, RPF may spread contiguously to involve multiple structures in both the retroperitoneum and the peritoneal cavity or multiple non-contiguous sites may be involved. We retrospectively reviewed 30 patients (19 male, 11 female; age range 28-79 years) with biopsy proven RPF. Although we found RPF most commonly as an isolated fibrotic plaque in the lower lumbar region (18 patients), 12 patients (40%) presented with RPF in atypical locations (4 peripancreatic, 1 periduodenal, 7 pelvic). The RPF was non-malignant in 24 patients (21 idiopathic, 2 perianeurysmal, 1 ergot-derivative treatment) and malignant in six cases. We present a pictorial review of the varied appearances of RPF, concentrating on atypical sites. The radiological differential diagnosis and its appearance with various imaging modalities are discussed. Current concepts with respect to management, prognosis and treatment are summarized.

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