Refine
Has Fulltext
- yes (70)
Is part of the Bibliography
- yes (70)
Year of publication
Document Type
- Journal article (70)
Language
- English (70)
Keywords
- multiple myeloma (5)
- apoptosis (4)
- gene expression (4)
- Hodgkin lymphoma (3)
- Medizin (3)
- Activation (2)
- CD30 (2)
- CXCR4 (2)
- NFATc1 (2)
- T cells (2)
- T-cell lymphoma (2)
- amplicon sequencing (2)
- anaplastic large cell lymphoma (2)
- cancer (2)
- cell staining (2)
- cell-cycle arrest (2)
- flow cytometry (2)
- gene regulation (2)
- lymphoma (2)
- machine learning (2)
- metastasis (2)
- survival (2)
- thymus (2)
- 3D lung tumor tissue models (1)
- AKT-signaling (1)
- ALCL (1)
- ALK-1 (1)
- AOM/DSS (1)
- ATG7 (1)
- Antibodies (1)
- B cell malignancies (1)
- B cell receptor (BCR) (1)
- B cells (1)
- B-MYB (1)
- B-cell lymphoma (1)
- BIRC7 (1)
- Bioluminescence imaging (1)
- Biomarker (1)
- Bone marrow cells (1)
- Bone marrow transplantation (1)
- Breast-cancer (1)
- Bruton Tyrosine Kinase (1)
- Burkitt lymphoma (1)
- Burkitt lymphoma (BL) (1)
- B‐cell lymphoma (1)
- CD/metabolism (1)
- CD274 (1)
- CD319 (1)
- CD40 ligand (1)
- CRC (1)
- CRISPR/Cas9 (1)
- CS1 (1)
- CX5461 (1)
- CXCR4/SDF-1 (1)
- Cancer (1)
- Cancer genetics (1)
- Cancer treatment (1)
- Chains (1)
- Clonality (1)
- Cushings syndrome (1)
- DHAP (1)
- DNA hypermethylation (1)
- DNA methylation (1)
- Defined burkitts lymphoma (1)
- Delta Repertoire (1)
- Design (1)
- EBER in situ hybridization (1)
- EBV (1)
- EMT (1)
- EZH1 (1)
- EZH2 (1)
- Epitope (1)
- Epstein-Barr virus (1)
- Expression (1)
- FDG PET/CT (1)
- FFPE (1)
- Fak regulation (1)
- Frequency (1)
- GIST (1)
- Gene-expression (1)
- Genome wide analysis (1)
- GvHD (1)
- H3K27me3 (1)
- HD (1)
- Hans algorithm (1)
- High-resolution (1)
- Histone deacetylase inhibition (1)
- IL-17 (1)
- IRF4 (1)
- Ibrutinib (1)
- Induced apoptosis (1)
- Intestinal Intraepithelial Lymphocy (1)
- JAK inhibitor (1)
- KIT (1)
- KRAS (1)
- KRAS biomarker signatures (1)
- Kidney cancer (1)
- LESA (1)
- Ligand (1)
- Lipom (1)
- Lung-cancer (1)
- LyP (1)
- Lymph2Cx assay (1)
- Lymphoma (1)
- Lymphomas (1)
- MEK/ERK-signaling (1)
- MIZ1 (1)
- MTB (1)
- MTCH2 (1)
- MTX (1)
- MYC (1)
- Malignancies (1)
- Mantle cell lymphoma (1)
- Mechanisms (1)
- Medical research (1)
- Microarray (1)
- Migration (1)
- Molecular pathogenesis (1)
- Multiple (1)
- Myb-MuvB (1)
- Myeloma cells (1)
- Myelomas (1)
- NF-Kappa-B (1)
- NFAT (1)
- NGS (1)
- NLPHL (1)
- Nuclear expression (1)
- Organoids (1)
- PCI-32765 (1)
- PET (1)
- PLAG1 rearrangement (1)
- PTCL (1)
- Pathogenesis (1)
- Pathway (1)
- Phase- (1)
- Positron emission tomography (1)
- Profiling (1)
- Prognosis (1)
- Proliferation (1)
- Promoter (1)
- R-CHOP (1)
- RCC (1)
- RNA probe (1)
- RNAPOL1 (1)
- Renal cell carcinoma (1)
- Rituximab plus (1)
- Riutximab (1)
- SGN-35 (1)
- Sox9 (1)
- Spleen (1)
- Sprue (1)
- Stromal cells (1)
- Survival (1)
- T cell receptors (1)
- T(H)17 cells (1)
- T-cell non-Hodgkin's lymphomas (1)
- T-cell transfer (1)
- T-follicular regulatory cell (1)
- TCR (1)
- TCR signaling cascade (1)
- Targeted Therapies (1)
- Targets (1)
- Th17 (1)
- Transcription-factor (1)
- Translational research (1)
- Tregs (1)
- Tumor Microenvironment (1)
- Tumour markers (1)
- USP9X (1)
- Usage (1)
- XIAP (1)
- YAP (1)
- [\(^{68}\)Ga] pentixafor (1)
- abnormalities (1)
- actin (1)
- activated B cell-like diffuse large B-cell lymphoma (ABC-DLBCL) (1)
- acute graft-versus host disease (1)
- acute myeloid leukaemia (1)
- adenomas (1)
- adrenal tumor (1)
- adrenocortical cancer (1)
- alloreactive T cells (1)
- alternative splicing (1)
- aluminum granuloma (1)
- amplifications (1)
- anaplastic large cell lymphoma (ALCL) (1)
- anaplastic medulloblastoma (1)
- animal model (1)
- anti-CD30 drug conjugate (1)
- anti-inflammatory cytokines (1)
- antigen loss (1)
- antigens (1)
- antioxidant function (1)
- aortic adventitia (1)
- autoimmune disease (1)
- autologous transplantation (1)
- autophagy (1)
- biosynthesis (1)
- bone disease (1)
- bone marrow cells (1)
- boolean in silico models (1)
- brain tumor (1)
- breast cancer (1)
- c-myc (1)
- cancer care (1)
- cancer detection (1)
- cancer diagnosis (1)
- cancer metabolism (1)
- cancer stem cells (1)
- cancers and neoplasms (1)
- carcinogenesis (1)
- carcinoma (1)
- carcinomas (1)
- case report (1)
- caspase-3 (1)
- catenin (1)
- cell binding (1)
- cell cycle and cell division (1)
- cell death (1)
- cell of origin (1)
- cells (1)
- chemokine receptor (1)
- children (1)
- chromosomes (1)
- chronic IBD model (1)
- classical Hodgkin lymphoma (1)
- classification (1)
- combined therapy (1)
- comparative genomic hybridization (1)
- complement system (1)
- consensus DNA (1)
- cutaneous T-cell-lymphoma (1)
- cyclophsophamide (1)
- cyclosporin A (1)
- cytotoxicity (1)
- damage (1)
- damage responses (1)
- deletions (1)
- diagnostics (1)
- diffuse large B-cell lymphoma (1)
- disease (1)
- distinct (1)
- drug resistance (1)
- ectopic lymphoid follicle (1)
- effector Treg (eTreg) (1)
- enhancer (1)
- enzyme-linked immunoassays (1)
- ephitelial cells (1)
- epigenetics (1)
- epithelium (1)
- expression (1)
- extramedullary disease (1)
- features (1)
- fluorescence in situ hybridisation (1)
- follicular lymphoma (1)
- forecasting (1)
- gRNA-only (1)
- gastrointestinal infections (1)
- gene (1)
- genetic loci (1)
- genomic aberrations (1)
- germline mutation (1)
- glioblastoma (1)
- grade 3B (1)
- group 3 (1)
- growth patterns (1)
- helper T cells (1)
- hemophagocytosis (1)
- high numbers (1)
- high-dose chemotherapy (1)
- high-resolution analysis (1)
- human genome (1)
- imaging (1)
- immune checkpoint blockade (1)
- immunohistochemistry (1)
- immunohistochemistry techniques (1)
- immunotherapeutics (1)
- in vivo imaging (1)
- in-vitro (1)
- in-vivo (1)
- independent predictor (1)
- inflammation-induced tissue demage (1)
- invasion (1)
- involvement (1)
- kidney cancer (1)
- kidneys (1)
- kinase (1)
- large cell transformation (1)
- latency type (1)
- lesions (1)
- lineage (1)
- lipoblastoma (1)
- livin (1)
- lymph node stromal cells (1)
- lymph node transplantation (1)
- lymphohistiocytosis (1)
- lymphoid aggregate (1)
- lymphoid hyperplasia (1)
- lymphomatoid papulosis (1)
- mTOR (1)
- major histocompatibility complex (1)
- malignancies (1)
- malignant tumors (1)
- mantel cell lymphoma (1)
- mantle cell lymphoma (1)
- marcophages (1)
- marrow transplantation (1)
- mast cells (1)
- mastocytosis (1)
- measles virus (1)
- meningeal inflammation (1)
- mesenteric lymph node (1)
- mesentery (1)
- metabolism (1)
- methylation (1)
- miR-126 (1)
- miR-21 (1)
- miRNA (1)
- mice (1)
- microenvironment (1)
- mitochondrial DNA (1)
- mitosis (1)
- mitotic genes (1)
- molecular subtypes (1)
- monoclonal antibody (1)
- mouse models (1)
- mtDNA (1)
- mutant p53 (1)
- mutation (1)
- myasthenia (1)
- myasthenia gravis (1)
- mycosis fungoides (1)
- naive T-cell gene editing (1)
- network (1)
- neutral loss (1)
- next generation sequencing (1)
- nodular lymphcyte (1)
- notch signaling (1)
- nuclear localization (1)
- obinutuzumab (1)
- organoids (1)
- orthotopic xenograft (1)
- outcomes research (1)
- outreach (1)
- p53 (1)
- p53-dependent apoptosis (1)
- p53-inducible regulator (1)
- pan-RCC (1)
- pancreatic cancer (1)
- panel sequencing (1)
- panel-sequencing (1)
- panniculitis (1)
- pathology (1)
- pathway (1)
- patient access (1)
- pediatric (1)
- pediatric lymphoma (1)
- peripheral T-cell (1)
- plasma cells (1)
- pleural mesothelioma (1)
- poor prognosis (1)
- positron emission tomography (1)
- precision oncology (1)
- primary cutaneous follicular B-cell lymphoma (1)
- pro-inflammatory cytokines (1)
- prognostic factor (1)
- progressive multiple sclerosis (1)
- pseudolymphoma (1)
- psoas muscle (1)
- rare SNP (1)
- real world data (1)
- receptor tyrosine kinases (1)
- refractory/relapsed lymphoma (1)
- regression analysis (1)
- regulatory T-cells (1)
- relapse (1)
- renal cancer (1)
- renal cell carcinoma (1)
- restoration (1)
- retroperitoneal tumor (1)
- reverse transcriptase-polymerase chain reaction (1)
- ribosome (1)
- seminoma (1)
- senescence (1)
- serum (1)
- slice culture (1)
- stemness (1)
- suppression (1)
- surgery (1)
- surgical and invasive medical procedures (1)
- surgical oncology (1)
- systemic and cutaneous CD30+ lymphoproliferations (1)
- systemic sclerosis (1)
- target validation (1)
- targeted (1)
- targeted combination therapy (1)
- targeted sequencing (1)
- targeted therapies (1)
- theranostics (1)
- thymic carcinoma (1)
- thymic epithelial tumor (1)
- thymitis (1)
- thymoma (1)
- tofacitinib (1)
- transcription (1)
- transcriptional repression (1)
- transformation (1)
- translocation (1)
- treatment regimens (1)
- tumor heterogeneity (1)
- tumor microenvironment (1)
- tumor spheroids (1)
- tumor-vessel wall-interface model (1)
- tumorigenesis (1)
- tumormicroenvironment (1)
- tumors (1)
- ubiquitin (1)
- up regulation (1)
- vascular wall stem and progenitor cells (1)
- vascularization model (1)
- vasculogenesis (1)
- venetoclax (1)
Institute
- Pathologisches Institut (67)
- Medizinische Klinik und Poliklinik II (20)
- Comprehensive Cancer Center Mainfranken (10)
- Klinik und Poliklinik für Allgemein-, Viszeral-, Gefäß- und Kinderchirurgie (Chirurgische Klinik I) (7)
- Theodor-Boveri-Institut für Biowissenschaften (7)
- Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie (6)
- Urologische Klinik und Poliklinik (5)
- Medizinische Klinik und Poliklinik I (4)
- Kinderklinik und Poliklinik (3)
- Klinik und Poliklinik für Nuklearmedizin (3)
Sonstige beteiligte Institutionen
High programmed cell death 1 ligand 1 (PD-L1) protein expression and copy number alterations (CNAs) of the corresponding genomic locus 9p24.1 in Hodgkin- and Reed–Sternberg cells (HRSC) have been shown to be associated with favourable response to anti-PD-1 checkpoint inhibition in relapsed/refractory (r/r) classical Hodgkin lymphoma (cHL). In the present study, we investigated baseline 9p24.1 status as well as PD-L1 and major histocompatibility complex (MHC) class I and II protein expression in 82 biopsies from patients with early stage unfavourable cHL treated with anti-PD-1-based first-line treatment in the German Hodgkin Study Group (GHSG) NIVAHL trial (ClinicalTrials.gov Identifier: NCT03004833). All evaluated specimens showed 9p24.1 CNA in HRSC to some extent, but with high intratumoral heterogeneity and an overall smaller range of alterations than reported in advanced-stage or r/r cHL. All but two cases (97%) showed PD-L1 expression by the tumour cells in variable amounts. While MHC-I was rarely expressed in >50% of HRSC, MHC-II expression in >50% of HRSC was found more frequently. No obvious impact of 9p24.1 CNA or PD-L1 and MHC-I/II expression on early response to the highly effective anti-PD-1-based NIVAHL first-line treatment was observed. Further studies evaluating an expanded panel of potential biomarkers are needed to optimally stratify anti-PD-1 first-line cHL treatment.
Background
Lipoblastoma is a rare benign mesenchymal neoplasm of infancy that most commonly occurs on the extremities and trunk but can arise at variable sites of the body. Retroperitoneal lipoblastomas are particularly rare but can grow to enormous size, and preoperative diagnosis is difficult with diverse, mostly malignant differential diagnoses that would lead to aggressive therapy. Since lipoblastoma is a benign tumor that has an excellent prognosis after resection, correct diagnosis is crucial.
Case presentation
A case of a large retroperitoneal tumor of a 24-month old infant that was clinically suspicious of a malignant tumor is presented. Due to proximity to the right kidney, clinically most probably a nephroblastoma or clear cell sarcoma of the kidney was suspected. Radiological findings were ambiguous. Therefore, the mass was biopsied, and histology revealed an adipocytic lesion. Although mostly composed of mature adipocytes, in view of the age of the patient, the differential diagnosis of a (maturing) lipoblastoma was raised, which was supported by molecular analysis demonstrating a HAS2-PLAG1 fusion. The tumor was completely resected, and further histopathological workup led to the final diagnosis of a 13 cm large retroperitoneal maturing lipoblastoma. The child recovered promptly from surgery and showed no evidence of recurrence so far.
Conclusion
Although rare, lipoblastoma should be included in the differential diagnoses of retroperitoneal tumors in infants and children, and molecular diagnostic approaches could be a helpful diagnostic adjunct in challenging cases.
Background
Germinal center-derived B cell lymphomas are tumors of the lymphoid tissues representing one of the most heterogeneous malignancies. Here we characterize the variety of transcriptomic phenotypes of this disease based on 873 biopsy specimens collected in the German Cancer Aid MMML (Molecular Mechanisms in Malignant Lymphoma) consortium. They include diffuse large B cell lymphoma (DLBCL), follicular lymphoma (FL), Burkitt’s lymphoma, mixed FL/DLBCL lymphomas, primary mediastinal large B cell lymphoma, multiple myeloma, IRF4-rearranged large cell lymphoma, MYC-negative Burkitt-like lymphoma with chr. 11q aberration and mantle cell lymphoma.
Methods
We apply self-organizing map (SOM) machine learning to microarray-derived expression data to generate a holistic view on the transcriptome landscape of lymphomas, to describe the multidimensional nature of gene regulation and to pursue a modular view on co-expression. Expression data were complemented by pathological, genetic and clinical characteristics.
Results
We present a transcriptome map of B cell lymphomas that allows visual comparison between the SOM portraits of different lymphoma strata and individual cases. It decomposes into one dozen modules of co-expressed genes related to different functional categories, to genetic defects and to the pathogenesis of lymphomas. On a molecular level, this disease rather forms a continuum of expression states than clearly separated phenotypes. We introduced the concept of combinatorial pattern types (PATs) that stratifies the lymphomas into nine PAT groups and, on a coarser level, into five prominent cancer hallmark types with proliferation, inflammation and stroma signatures. Inflammation signatures in combination with healthy B cell and tonsil characteristics associate with better overall survival rates, while proliferation in combination with inflammation and plasma cell characteristics worsens it. A phenotypic similarity tree is presented that reveals possible progression paths along the transcriptional dimensions. Our analysis provided a novel look on the transition range between FL and DLBCL, on DLBCL with poor prognosis showing expression patterns resembling that of Burkitt’s lymphoma and particularly on ‘double-hit’ MYC and BCL2 transformed lymphomas.
Conclusions
The transcriptome map provides a tool that aggregates, refines and visualizes the data collected in the MMML study and interprets them in the light of previous knowledge to provide orientation and support in current and future studies on lymphomas and on other cancer entities.
Actin cytoskeleton deregulation confers midostaurin resistance in FLT3-mutant acute myeloid leukemia
(2021)
The presence of FMS-like tyrosine kinase 3-internal tandem duplication (FLT3-ITD) is one of the most frequent mutations in acute myeloid leukemia (AML) and is associated with an unfavorable prognosis. FLT3 inhibitors, such as midostaurin, are used clinically but fail to entirely eradicate FLT3-ITD+AML. This study introduces a new perspective and highlights the impact of RAC1-dependent actin cytoskeleton remodeling on resistance to midostaurin in AML. RAC1 hyperactivation leads resistance via hyperphosphorylation of the positive regulator of actin polymerization N-WASP and antiapoptotic BCL-2. RAC1/N-WASP, through ARP2/3 complex activation, increases the number of actin filaments, cell stiffness and adhesion forces to mesenchymal stromal cells (MSCs) being identified as a biomarker of resistance. Midostaurin resistance can be overcome by a combination of midostaruin, the BCL-2 inhibitor venetoclax and the RAC1 inhibitor Eht1864 in midostaurin-resistant AML cell lines and primary samples, providing the first evidence of a potential new treatment approach to eradicate FLT3-ITD+AML. Garitano-Trojaola et al. used a combination of human acute myeloid leukemia (AML) cell lines and primary samples to show that RAC1-dependent actin cytoskeleton remodeling through BCL2 family plays a key role in resistance to the FLT3 inhibitor, Midostaurin in AML. They showed that by targeting RAC1 and BCL2, Midostaurin resistance was diminished, which potentially paves the way for an innovate treatment approach for FLT3 mutant AML.
The molecular pathogenesis of thymomas and thymic arcinomas (TCs) is poorly understood and results of adjuvant therapy are unsatisfactory in case of metastatic disease and tumor recurrence. For these clinical settings, novel therapeutic strategies are urgently needed. Recently, limited sequencing efforts revealed that a broad spectrum of genes that play key roles in various common cancers are rarely affected in thymomas and TCs, suggesting that other oncogenic principles might be important.This made us re-analyze historic expression data obtained in a spectrumof thymomas and thymic squamous cell carcinomas (TSCCs) with a custom-made cDNA microarray. By cluster analysis, different anti-apoptotic signatures were detected in type B3 thymoma and TSCC, including overexpression of BIRC3 in TSCCs. This was confirmed by qRT-PCR in the original and an independent validation set of tumors. In contrast to several other cancer cell lines, the BIRC3-positive TSCC cell line, 1889c showed spontaneous apoptosis after BIRC3 knock-down. Targeting apoptosis genes is worth testing as therapeutic principle in TSCC.
Macroautophagy (hereafter referred to as autophagy) is a homeostatic process that preserves cellular integrity. In mice, autophagy regulates pancreatic ductal adenocarcinoma (PDAC) development in a manner dependent on the status of the tumor suppressor gene Trp53. Studies published so far have investigated the impact of autophagy blockage in tumors arising from Trp53-hemizygous or -homozygous tissue. In contrast, in human PDACs the tumor suppressor gene TP53 is mutated rather than allelically lost, and TP53 mutants retain pathobiological functions that differ from complete allelic loss. In order to better represent the patient situation, we have investigated PDAC development in a well-characterized genetically engineered mouse model (GEMM) of PDAC with mutant Trp53 (Trp53\(^{R172H}\)) and deletion of the essential autophagy gene Atg7. Autophagy blockage reduced PDAC incidence but had no impact on survival time in the subset of animals that formed a tumor. In the absence of Atg7, non-tumor-bearing mice reached a similar age as animals with malignant disease. However, the architecture of autophagy-deficient, tumor-free pancreata was effaced, normal acinar tissue was largely replaced with low-grade pancreatic intraepithelial neoplasias (PanINs) and insulin expressing islet β-cells were reduced. Our data add further complexity to the interplay between Atg7 inhibition and Trp53 status in tumorigenesis.
Chromosomal translocations affecting the MYC oncogene are the biological hallmark of Burkitt lymphomas but also occur in a subset of other mature B-cell lymphomas. If accompanied by a chromosomal break targeting the BCL2 and/or BCL6 oncogene these MYC translocation-positive (MYC+) lymphomas are called double-hit lymphomas, otherwise the term single-hit lymphomas is applied. In order to characterize the biological features of these MYC+ lymphomas other than Burkitt lymphoma we explored, after exclusion of molecular Burkitt lymphoma as defined by gene expression profiling, the molecular, pathological and clinical aspects of 80 MYC-translocation-positive lymphomas (31 single-hit, 46 double-hit and 3 MYC+-lymphomas with unknown BCL6 status). Comparison of single-hit and double-hit lymphomas revealed no difference in MYC partner (IG/non-IG), genomic complexity, MYC expression or gene expression profile. Double-hit lymphomas more frequently showed a germinal center B-cell-like gene expression profile and had higher IGH and MYC mutation frequencies. Gene expression profiling revealed 130 differentially expressed genes between BCL6(+)/MYC+ and BCL2(+)/MYC+ double-hit lymphomas. BCL2(+)/MYC+ double-hit lymphomas more frequently showed a germinal center B-like gene expression profile. Analysis of all lymphomas according to MYC partner (IG/non-IG) revealed no substantial differences. In this series of lymphomas, in which immunochemotherapy was administered in only a minority of cases, single-hit and double-hit lymphomas had a similar poor outcome in contrast to the outcome of molecular Burkitt lymphoma and lymphomas without the MYC break. Our data suggest that, after excluding molecular Burkitt lymphoma and pediatric cases, MYC+ lymphomas are biologically quite homogeneous with single-hit and double-hit lymphomas as well as IG-MYC and non-IG-MYC+ lymphomas sharing various molecular characteristics.
In Burkitt lymphoma (BL), a tumor of germinal center B cells, the pro-apoptotic properties of MYC are controlled by tonic B cell receptor (BCR) signals. Since BL cells do not exhibit constitutive NF-κB activity, we hypothesized that anti-apoptotic NFATc1 proteins provide a major transcriptional survival signal in BL. Here we show that post-transcriptional mechanisms are responsible for the calcineurin (CN) independent constitutive nuclear over-expression of NFATc1 in BL and Eµ-MYC – induced B cell lymphomas (BCL). Conditional inactivation of the Nfatc1 gene in B cells of Eµ-MYC mice leads to apoptosis of BCL cells in vivo and ex vivo. Inhibition of BCR/SYK/BTK/PI3K signals in BL cells results in cytosolic re-location of NFATc1 and apoptosis. Therefore, NFATc1 activity is an integrated part of tonic BCR signaling and an alternative target for therapeutic intervention in BL.
Primary involvement of skeletal muscle is a very rare event in ALK-1 positive anaplastic large cell lymphoma (ALCL). We describe a case of a 10-year old boy presenting with a three week history of pain and a palpable firm swelling at the dorsal aspect of the left thigh. Histological examination of the lesion revealed a tumoral and diffuse polymorphic infiltration of the muscle by large lymphoid cells. Tumor cells displayed eccentric, lobulated "horse shoe" or "kidney-shape" nuclei. The cells showed immunohistochemical positivity for CD30, ALK-1, CD2, CD3, CD7, CD8, and Perforin. Fluorescence in situ hybridization analysis revealed a characteristic rearrangement of the ALK-1 gene in 2p23 leading to the diagnosis of ALK-1 positive ALCL. Chemotherapy according to the ALCL-99-NHL-BFM protocol was initiated and resulted in a complete remission after two cycles. This case illustrates the unusual presentation of a pediatric ALCL in soft tissue with a good response to chemotherapy.