DOCSLIB.ORG

(NCCN Guidelines®) B-Cell Lymphomas

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text

Load more

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas Version 2.2018 — February 26, 2018 NCCN.org Continue Version 2.2018, 02/26/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:56:57 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 Panel Members NCCN Guidelines Index Table of Contents B-Cell Lymphomas Discussion * Andrew D. Zelenetz, MD, PhD/Chair † Þ Beth Christian, MD † Ann S. LaCasce, MD † Memorial Sloan Kettering Cancer Center The Ohio State University Comprehensive Dana-Farber/Brigham and Women’s Cancer Center - James Cancer Hospital Cancer Center * Leo I. Gordon, MD/Co-Vice Chair ‡ ξ and Solove Research Institute Robert H. Lurie Comprehensive Cancer Amitkumar Mehta MD † ‡ Þ Center of Northwestern University Luis E. Fayad, MD ‡ Þ † University of Alabama at Birmingham The University of Texas Comprehensive Cancer Center Jeremy S. Abramson, MD † ‡ MD Anderson Cancer Center Massachusetts General Hospital Auayporn Nademanee, MD † ‡ ξ Cancer Center Martha J. Glenn, MD † ‡ Þ ξ City of Hope Comprehensive Cancer Center Huntsman Cancer Institute Ranjana H. Advani, MD † at the University of Utah Rachel Rabinovitch, MD § Stanford Cancer Institute University of Colorado Cancer Center Thomas M. Habermann, MD ‡ ξ C. Babis Andreadis, MD, MSCE ‡ † Mayo Clinic Cancer Center Nishitha Reddy, MD ‡ ξ UCSF Helen Diller Family Vanderbilt-Ingram Cancer Center Comprehensive Cancer Center Nancy Lee Harris, MD ≠ Erin Reid, MD ‡ Massachusetts General Hospital Cancer Center Nancy Bartlett, MD † UC San Diego Moores Cancer Center Siteman Cancer Center at Barnes- Francisco Hernandez-Ilizaliturri, MD † Stephen D. Smith, MD ‡ Jewish Hospital and Washington Roswell Park Cancer Institute University School of Medicine Fred Hutchinson Cancer Research Center/ Seattle Cancer Care Alliance Mark S. Kaminski, MD † Paolo Caimi, MD ‡ † University of Michigan Erin D. Snyder, MD Þ Case Comprehensive Cancer Center/ Comprehensive Cancer Center University of Alabama at Birmingham University Hospitals Seidman Cancer Comprehensive Cancer Center Center and Cleveland Clinic Taussig Christopher R. Kelsey, MD § Cancer Institute Duke Cancer Institute Lode J. Swinnen, MB, ChB ‡ ξ The Sidney Kimmel Comprehensive Julie E. Chang, MD ‡ Nadia Khan, MD † Cancer Center at Johns Hopkins University of Wisconsin Fox Chase Cancer Center Carbone Cancer Center Julie M. Vose, MD, MBA ‡ ξ Susan Krivacic, MPAff ¥ Fred & Pamela Buffett Cancer Center Julio C. Chavez, MD † Consultant Moffitt Cancer Center NCCN Continue † Medical oncology Þ Internal medicine Mary Dwyer, MS ‡ Hematology/Hematology oncology ¥ Patient advocacy Hema Sundar, PhD § Radiotherapy/Radiation oncology * Discussion Writing Committee ξ Bone marrow transplantation Member NCCN Guidelines Panel Disclosures ≠ Pathology Version 2.2018, 02/26/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:56:57 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 Table of Contents NCCN Guidelines Index Table of Contents B-Cell Lymphomas Discussion NCCN B-Cell Lymphoma Panel Members Summary of the Guidelines Updates Clinical Trials: NCCN believes that the best management for any patient with • Diagnosis (DIAG-1) cancer is in a clinical trial. • Follicular Lymphoma (FOLL-1) Participation in clinical trials is especially • Marginal Zone Lymphomas (MZL-1) encouraged. Gastric MALT Lymphoma (MALT-1) To find clinical trials online at NCCN Nongastric MALT Lymphoma (NGMLT-1) Member Institutions, click here: Nodal Marginal Zone Lymphoma (NODE-1) nccn.org/clinical_trials/physician.html. Splenic Marginal Zone Lymphoma (SPLN-1) NCCN Categories of Evidence and • Mantle Cell Lymphoma (MANT-1) Consensus: All recommendations are category 2A unless otherwise indicated. • Diffuse Large B-Cell Lymphoma (BCEL-1) • High-Grade B-Cell lymphomas with Translocations of MYC and BCL2 and/or See NCCN Categories of Evidence and Consensus. BCL6 (Double/Triple Hit Lymphoma) (HGBL-1) • Burkitt Lymphoma (BURK-1) NCCN Categories of Preference: • AIDS-Related B-Cell Lymphomas (AIDS-1) All recommendations are considered • Lymphoblastic Lymphoma (BLAST-1) appropriate. • Post-Transplant Lymphoproliferative Disorders (PTLD-1) See NCCN Categories of Preference • Castleman’s Disease (CD-1) • Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Classification and Staging (ST-1) Mature B-Cell and NK/T-Cell Neoplasms (NHODG-A) • Supportive Care for B-Cell Lymphomas (NHODG-B) Primary CNS Lymphoma (See NCCN Guidelines for CNS) • Lugano Response Criteria for Non-Hodgkin’s Lymphoma (NHODG-C) Waldenström’s Macroglobulinemia/Lymphoplasmacytic • Principles of Radiation Therapy (NHODG-D) Lymphoma (See NCCN Guidelines for WM/LPL) • Special Considerations for the Use of Small-Molecule Inhibitors (NHODG-E) The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2018. Version 2.2018, 02/26/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:56:57 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 Updates NCCN Guidelines Index Table of Contents B-Cell Lymphomas Discussion Updates in Version 2.2018 of the NCCN Guidelines for B-Cell Lymphomas from Version 2.2018 include: Mantle Cell Lymphoma MANT-A 1 of 4 • Heading was revised, "Suggested Treatment Regimens (in preference order)." MANT-A 2 of 4 • Heading was revised, "Suggested Treatment Regimens (in preference order)" and "in alphabetical order" and "in alphabetical order by category" was added to the regimen list as appropriate. Updates in Version 1.2018 of the NCCN Guidelines for B-Cell Lymphomas from Version 7.2017 include: Global changes • The common bullets related to diagnosis were removed for each subtype and FOLL-6 (continued) added to a new page, DIAG-1. For each subtype, the diagnosis section is now Footnote v was added, "If transformation is co-existing with extensive titled, "Additional Diagnostic Testing." FL, consider maintenance (see FOLL-5, Optional Extended Therapy)." • Workup, "CBC, differential, platelets" was changed to "CBC with differential." Footnote w was revised, "If proceeding to an autologous stem cell • Suggested treatment regimen references were updated throughout the rescue, consider additional cytoreductive systemic therapy ± ISRT guidelines. to induce CR prior to transplant. Axicabtagene ciloleucel is not an • Radioimmunotherapy was clarified as ibritumomab" tiuxetan" throughout the appropriate treatment option for patients with a CR." guidelines. Footnote x was added, "Repeat biopsy should be strongly considered if PET-positive prior to additional therapy. If biopsy negative, follow CR Follicular Lymphoma pathway." FOLL-2 • Workup, FOLL-7 • Histologic transformation to DLBCL after multiple lines of prior therapies, "Beta-2-microglobulin" was moved from Essential to Useful in Selected Cases and qualified with "necessary for calculation of FLIPI-2." Chemoimmunotherapy was clarified as "second-line therapy" on BCEL-C. (Also for NODE-5) FOLL-3 • Stage I, II After responsive disease, "± ISRT if not previously given" was added to Non-bulky was replaced and defined with "<7 cm" HDT/ASCR and HCT. (Also for NODE-5) Bulky was replaced and defined with ≥" 7 cm" FOLL-8 • Treatment, FOLL-4 • Stage III, IV "for patients with extensive local disease who are not candidates for excision or ISRT" was added to RCHOP. Indications for treatment ◊ 6th bullet was revised, "Steady or rapid progression." Change made "Observe" was added after "Excision (preferred)" throughout the guidelines as appropriate. • Footnote cc was revised by adding, "Localized disease (stage I,II) is more • Indication present, "Local ISRT (4–30 Gy) (palliation of locally symptomatic common than advanced-stage disease (stage III,IV)." disease)" was revised to "Palliative ISRT" and the dosing was added to • Footnote dd was revised by adding, "There are no data to support NHODG-D 3 of 4. (Also for FOLL-5, NODE-3, and NODE-4) maintenance therapy." FOLL-6 FOLL-B 1
Recommended publications
  • FT117 Langerhans Cell Histiocytosis with Histopathological Features

    FT117 Langerhans Cell Histiocytosis with Histopathological Features

    FT117 Langerhans cell histiocytosis with histopathological features, single center experience Histopatolojik özellikleriyle langerhans hücreli histiyositoz, tek merkez deneyimi Fahriye KILINÇ Necmettin Erbakan Üniversitesi, Meram Tıp Fakültesi, Tıbbi Patoloji Anabilim Dalı, Konya Aim: Langerhans cell histiocytosis (LCH) is a rare histiocytic disease, occurring in 2-10 children per million and 1-2 adults per million, and may have a wide variety of clinical manifestations. Infiltration can develop in almost any organ (the most commonly reported organs are bone, skin, lymph nodes, lungs, thymus, liver, spleen, bone marrow and central nervous system). We aimed to evaluate the histopathological features of the lesions and review the literature in pediatric patients referred to our department for pathological examination and diagnosed as LCH. Materials and Methods: Retrospectively, childhood cases diagnosed with LCH in 2012-2019 were screened by hospital automation system. Age, gender, lesion localizations of the cases were recorded and histopathological features were reviewed. Results: 5 male and 5 female total of 10 cases were detected. The youngest 3 were under the age of 1, the oldest was 16 years old. Localization; 6 of the cases were bone (2 femur, 3 skull bone, 1 scapula), 2 skin, 1 bone and lymph node, 1 lung and lymph node. Histopathology revealed histiocytic cells with grooved nuclei, eosinophilic cytoplasm with eosinophils, and neutrophils in some cases. Immunohistochemical CD1a staining was positive in all cases and positivities were present with S100 in applied 9 cases, CD68 in 4. Ki67 proliferation index was studied in 2 patients with bone localization, 15% and 20%. Conclusion: The term LCH is due to the morphological and immunophenotypic similarity of the infiltrating cells of this disease to Langerhans cells specialized as dendritic cells in the skin and mucous membranes.
  • Skin Biopsy Diagnosis of Langerhans Cell Neoplasms

    Skin Biopsy Diagnosis of Langerhans Cell Neoplasms

    Chapter 3 Skin Biopsy Diagnosis of Langerhans Cell Neoplasms Olga L. Bohn, Julie Teruya-Feldstein and Sergio Sanchez-Sosa Additional information is available at the end of the chapter http://dx.doi.org/10.5772/55893 1. Introduction This chapter reviews the clinical presentation, histopathology, immunoprofile and molecular features of Langerhans cell neoplasms of the skin including Langerhans cell histiocytosis (LCH) and its malignant counterpart, Langerhans cell sarcoma (LCS). Biopsy of the skin is a useful method to confirm LCH/LCS diagnosis, as cutaneous involvement is seen in more than 50% cases. Skin can be the only presenting site of LCH, but it is usually seen as an integral part of multisystemic disease involvement. Langerhans cells (LC) are bone marrow-derived antigen presenting cells [1]. Although LC, dendritic cells and monocytic/histiocytic cells share a common multipotential progenitor cells that reside in the bone marrow, to the date, myeloid derived macrophages and dendritic cells constitute divergent lines of differentiation from bone marrow precursors [2]. However, recent evidence demonstrates that LC can be generated from lymphoid-committed CD4low precur‐ sors, suggesting the role of lineage plasticity/ trans-differentiation and clonal infidelity [3-4]. LC can be found in the epidermis and mucosal lining of multiple organs including cervix, vagina, stomach and esophagus. The specific immunophenotypic profile is helpful distin‐ guishing LCs, as they can express CD1a and langerin (CD207); in addition the detection of Birbeck granules, seen in both pathological and resting LC is a prominent feature [5]. LCH encompasses a spectrum of disease characterized by an uncontrolled proliferation of LC [5].
  • (Rituxan®), Rituximab-Abbs (Truxima®), Rituximab-Pvvr (Ruxience®) Prior Authorization Drug Coverage Policy

    (Rituxan®), Rituximab-Abbs (Truxima®), Rituximab-Pvvr (Ruxience®) Prior Authorization Drug Coverage Policy

    1 Rituximab Products: Rituximab (Rituxan®), Rituximab-abbs (Truxima®), Rituximab-pvvr (Ruxience®) Prior Authorization Drug Coverage Policy Effective Date: 2/1/2021 Revision Date: n/a Review Date: 7/2/20 Lines of Business: Commercial Policy type: Prior Authorization This Drug Coverage Policy provides parameters for the coverage of rituximab (Rituxan®), rituximab-abbs (Truxima®), and rituximab-pvvr (Ruxience®). Consideration of medically necessary indications are based upon U.S. Food and Drug Administration (FDA) indications, recommended uses within the Centers of Medicare & Medicaid Services (CMS) five recognized compendia, including the National Comprehensive Cancer Network (NCCN) Drugs & Biologics Compendium (Category 1 or 2A recommendations), and peer-reviewed scientific literature eligible for coverage according to the CMS, Medicare Benefit Policy Manual, Chapter 15, section 50.4.5 titled, “Off- Label Use of Anti-Cancer Drugs and Biologics.” This policy evaluates whether the drug therapy is proven to be effective based on published evidence-based medicine. Drug Description1-3 Rituximab (Rituxan®), rituximab-abbs (Truxima®), and rituximab-pvvr (Ruxience®) are monoclonal antibodies that target the CD20 antigen expressed on the surface of pre-B and mature B- lymphocytes. Upon binding to cluster of differentiation (CD) 20, rituximab mediates B-cell lysis. Possible mechanisms of cell lysis include complement dependent cytotoxicity (CDC) and antibody dependent cell mediated cytotoxicity (ADCC). B cells are believed to play a role in the pathogenesis of rheumatoid arthritis (RA) and associated chronic synovitis. In this setting, B cells may be acting at multiple sites in the autoimmune/inflammatory process, including through production of rheumatoid factor (RF) and other autoantibodies, antigen presentation, T-cell activation, and/or proinflammatory cytokine production.
  • Histiocytic and Dendritic Cell Lesions

    Histiocytic and Dendritic Cell Lesions

    1/18/2019 Histiocytic and Dendritic Cell Lesions L. Jeffrey Medeiros, MD MD Anderson Cancer Center Outline 2016 classification of Histiocyte Society Langerhans cell histiocytosis / sarcoma Erdheim-Chester disease Juvenile xanthogranuloma Malignant histiocytosis Histiocytic sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma Rosai-Dorfman disease Hemophagocytic lymphohistiocytosis Writing Group of the Histiocyte Society 1 1/18/2019 Major Groups of Histiocytic Lesions Group Name L Langerhans-related C Cutaneous and mucocutaneous M Malignant histiocytosis R Rosai-Dorfman disease H Hemophagocytic lymphohistiocytosis Blood 127: 2672, 2016 L Group Langerhans cell histiocytosis Indeterminate cell tumor Erdheim-Chester disease S100 Normal Langerhans cells Langerhans Cell Histiocytosis “Old” Terminology Eosinophilic granuloma Single lesion of bone, LN, or skin Hand-Schuller-Christian disease Lytic lesions of skull, exopthalmos, and diabetes insipidus Sidney Farber Letterer-Siwe disease 1903-1973 Widespread visceral disease involving liver, spleen, bone marrow, and other sites Histiocytosis X Umbrella term proposed by Sidney Farber and then Lichtenstein in 1953 Louis Lichtenstein 1906-1977 2 1/18/2019 Langerhans Cell Histiocytosis Incidence and Disease Distribution Incidence Children: 5-9 x 106 Adults: 1 x 106 Sites of Disease Poor Prognosis Bones 80% Skin 30% Liver Pituitary gland 25% Spleen Liver 15% Bone marrow Spleen 15% Bone Marrow 15% High-risk organs Lymph nodes 10% CNS <5% Blood 127: 2672, 2016 N Engl J Med
  • Cutaneous Neonatal Langerhans Cell Histiocytosis

    Cutaneous Neonatal Langerhans Cell Histiocytosis

    F1000Research 2019, 8:13 Last updated: 18 SEP 2019 SYSTEMATIC REVIEW Cutaneous neonatal Langerhans cell histiocytosis: a systematic review of case reports [version 1; peer review: 1 approved with reservations, 1 not approved] Victoria Venning 1, Evelyn Yhao2,3, Elizabeth Huynh2,3, John W. Frew 2,4 1Prince of Wales Hospital, Randwick, Sydney, NSW, 2033, Australia 2University of New South Wales, Sydney, NSW, 2033, Australia 3Sydney Children's Hospital, Randwick, NSW, 2033, Australia 4Department of Dermatology, Liverpool Hospital, Sydney, Sydney, NSW, 2170, Australia First published: 03 Jan 2019, 8:13 ( Open Peer Review v1 https://doi.org/10.12688/f1000research.17664.1) Latest published: 03 Jan 2019, 8:13 ( https://doi.org/10.12688/f1000research.17664.1) Reviewer Status Abstract Invited Reviewers Background: Cutaneous langerhans cell histiocytosis (LCH) is a rare 1 2 disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Although some cases spontaneously version 1 resolve, no consistent variables have been identified that predict which published report report cases will manifest with systemic disease later in childhood. 03 Jan 2019 Methods: A systematic review (Pubmed, Embase, Cochrane database and all published abstracts from 1946-2018) was undertaken to collate all reported cases of cutaneous LCH in the international literature. This study 1 Jolie Krooks , Florida Atlantic University, was registered with PROSPERO (CRD42016051952). Descriptive statistics Boca Raton, USA and correlation analyses were undertaken. Bias was analyzed according to Milen Minkov , Teaching Hospital of the GRADE criteria. Medical University of Vienna, Vienna, Austria Results: A total of 83 articles encompassing 128 cases of cutaneous LCH were identified.
  • Beyond Langerhans Cell Histiocytosis Related to Smoking

    Beyond Langerhans Cell Histiocytosis Related to Smoking

    Radiología. 2019;61(3):215---224 www.elsevier.es/rx RADIOLOGY THROUGH IMAGES Pulmonary histiocytosis: Beyond Langerhans cell ଝ histiocytosis related to smoking a b a c d b,e,∗ C. Trejo Gallego , J. Bueno , E. Cruces , E.B. Stelow , N. Mancheno˜ , L. Flors a Servicio de Radiología, Hospital Universitario Morales Meseguer, Universidad de Murcia, Murcia, Spain b Department of Radiology and Medical Imaging, University of Virginia Health System, Charlottesville, VA, United States c Department of Pathology, University of Virginia Health System, Charlottesville, VA, United States d Servicio de Anatomía Patológica, Hospital Universitario y Politécnico la Fe, Valencia, Spain e Department of Radiology, One Hospital Dr, University of Missouri Health System, Columbia, MO, United States Received 21 October 2017; accepted 16 November 2018 Available online 24 January 2019 KEYWORDS Abstract Langerhans cells Objective: To review the imaging findings for the different types of pulmonary histiocy- histiocytosis; tosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis Erdheim---Chester related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans disease; cell histiocytosis in Rosai---Dorfman disease and Erdheim---Chester disease. We also review the Sinus histiocytosis; etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. Computed Conclusion: Langerhans cell histiocytosis, Rosai---Dorfman disease, and Erdheim---Chester dis- tomography ease are idiopathic
  • Histiocytic Sarcoma in a 3-Year-Old Male: a Case Report

    Histiocytic Sarcoma in a 3-Year-Old Male: a Case Report

    Histiocytic Sarcoma in a 3-Year-Old Male: A Case Report Samuel Buonocore, MD*; Alfredo L. Valente, MD‡; Daniel Nightingale, MD‡; Jeffrey Bogart, MD§; and Abdul-Kader Souid, MD, PhD* ABSTRACT. We describe a pediatric patient with his- CASE REPORT tiocytic sarcoma involving the T6 and L4 vertebral bodies This previously healthy 3-year-old boy experienced intermit- and the lungs. His tumor progressed during chemother- tent low back pain radiating to the right inguinal region for ϳ2 apy designed for Langerhans’ cell histiocytosis and sar- months. His symptoms initially responded to ibuprofen. The pain coma. High-dose radiation, on the other hand, was intensity increased over a 2-week period, and he refused to walk. effective. Pediatrics 2005;116:e322–e325. URL: www. Review of systems was significant for pain with urination. With the exception of being unable to stand, his physical examination pediatrics.org/cgi/doi/10.1542/peds.2005-0026; sarcoma, was unremarkable. The laboratory tests showed normal blood histiocytes, Langerhans’ cell histiocytosis, histiocytic sar- counts and normal liver and renal function. An MRI showed coma. collapse of the T6 and L4 vertebral bodies and a soft tissue mass in the anterior epidural space at the level of L4 (Fig 1 A and B). The chest and abdominal computed tomography (CT) scans were nor- ABBREVIATIONS. LCH, Langerhans’ cell histiocytosis; CT, com- mal. Bone marrow aspiration revealed no malignant infiltration. A puted tomography; 2CdA, 2-chlorodeoxyadenosine. technetium bone scan showed increased uptake limited to the T6 and L4 regions. CT-scan–guided needle biopsy of the L4 mass revealed infiltrative proliferation of the bone and soft tissue by istiocytic and dendritic neoplasms are rare, sheets and clusters of large ovoid cells with abundant eosinophilic cytoplasm (Fig 2A).
  • Langerhans Cell Sarcoma with an Aberrant Cytoplasmic CD3 Expression Zhaodong Xu1*, Ruth Padmore1, Carolyn Faught2, Lisa Duffet2 and Bruce F Burns3

    Langerhans Cell Sarcoma with an Aberrant Cytoplasmic CD3 Expression Zhaodong Xu1*, Ruth Padmore1, Carolyn Faught2, Lisa Duffet2 and Bruce F Burns3

    Xu et al. Diagnostic Pathology 2012, 7:128 http://www.diagnosticpathology.org/content/7/1/128 CASE REPORT Open Access Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression Zhaodong Xu1*, Ruth Padmore1, Carolyn Faught2, Lisa Duffet2 and Bruce F Burns3 Abstract: Langerhans cell sarcoma is a rare and aggressive high grade hematopoietic neoplasm with a dismal prognosis. It has a unique morphological and immunotypic profile with a CD1a/ langerin/S100 + phenotype. T cell lineage markers except for CD4 in Langerhans cell sarcoma have not been documented previously. We report a case of 86 year-old male of Caucasian descent who presented with an enlarging right neck mass over 2 months with an underlying unknown cause of anemia. Computed tomography scan of the neck, chest and abdomen revealed generalized lymphadenopathy and mild splenomegaly suspicious for lymphoma. Diagnostic core biopsy performed on right neck mass revealed a possible T cell lymphoma with expression of T cell lineage specific marker CD3 but conclusive diagnosis could not be made due to insufficient core biopsy sample. Further excisional biopsy performed on a left inguinal node showed a hematopoietic neoplasm with features of Langerhans cell sarcoma with a focal cytoplasmic CD3 expression in 30-40% of the tumor cells. PCR for T cell receptor (TCR) gene rearrangement failed to demonstrate a clonal gene rearrangement in the tumor cells arguing against a T cell lineage transdifferentiation, suggesting an aberrant CD3 expression. To the best of our knowledge, this case represents the first report of Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression. Virtual slides: http://www.diagnosticpathology.diagnomx.eu/vs/2065486371761991 Keywords: Langerhans cell sarcoma (LCS), Langerhans cell histiocytosis (LCH), CD3, Aberrant expression, Lineage plasticity, Transdifferentiation Background langerin/S100+ [2,3] but without B- and T-cell lineage According to the most recent WHO Classification of markers except for CD4 [4].
  • Histiocytic Sarcoma Originating in the Lung in a 16-Year-Old Male

    Histiocytic Sarcoma Originating in the Lung in a 16-Year-Old Male

    J Clin Exp Hematop Vol. 55, No. 1, June 2015 Case Study Histiocytic Sarcoma Originating in the Lung in a 16-Year-Old Male Sakura Tomita,1) Go Ogura,1) Chie Inomoto,1) Hiroshi Kajiwara,1) Ryota Masuda,2) Masayuki Iwazaki,2) Masaru Kojima,3) and Naoya Nakamura1) We report a 16-year-old male with histiocytic sarcoma (HS) originating in the lung. Partial resection of the lung was performed for a 3-cm mass with a clear boundary detected in the right inferior pulmonary lobe on a health checkup. Histologically, the tumor infiltrated into the surrounding tissue, and was comprised of spindle cells, mainly, and foam cells accompanied by mild nuclear atypia. The tumor cells were immunohistochemically positive for CD68 and CD163, indicating histiocytic lineage and the MIB-1-positive rate was low. Spindle cell morphology of HS is quite rare and only 3 cases of pulmonary HS have previously been reported. 〔J Clin Exp Hematop 55(1) : 45-49, 2015〕 Keywords: histiocytic sarcoma, lung, spindle cells, foamy cells spindle cells, mainly, and foam cells. INTRODUCTION Histiocytic sarcoma (HS) is a malignant hematopoietic CASE REPORT tumor consisting of cells similar to mature histiocytes.1-4 It is extremely rare and the age of onset widely ranges from 6 The patient was a 16-year-old male who exhibited an months to 89 years, with no gender difference; the incidence abnormal shadow detected on a health checkup. He had no is high in adults, showing a large peak at 50-69 years, but also particular past or familial medical history.
  • Delivering Novel Targeted Therapies to Cancer Patients

    Delivering Novel Targeted Therapies to Cancer Patients

    NEXT-GENERATION RADIOIMMUNOTHERAPIES FOR NON-HODGKIN’S LYMPHOMA PATIENTS SEPTEMBER 2019 Nordic Nanovector ASA Kjelsåsveien 168 B, 0884 Oslo, Norway www.nordicnanovector.com IR contact: [email protected] Forward-looking statements This slide presentation contains certain forward-looking statements. These statements are based on management's current expectations and are subject to uncertainty and changes in circumstances, since they relate to events and depend on circumstances that will occur in the future and which, by their nature, will have an impact on Nordic Nanovector's business, financial condition and results of operations. The terms "anticipates", "assumes", "believes", "can", "could", "estimates", "expects", "forecasts", "intends", "may", "might", "plans", "should", "projects", "targets", "will", "would" or, in each case, their negative, or other variations or comparable terminology are used to identify forward looking statements. These forward-looking statements are not historic facts. There are a number of factors that could cause actual results and developments to differ materially from those expressed or implied in the forward-looking statements. Factors that could cause these differences include, but are not limited to, risks associated with implementation of Nordic Nanovector's strategy, risks and uncertainties associated with the development and/or approval of Nordic Nanovector's product candidates, ongoing and future clinical trials and expected trial results, the ability to commercialise Betalutin®, technology changes and new products in Nordic Nanovector's potential market and industry, Nordic Nanovector's freedom to operate (competitors patents) in respect of the products it develops, the ability to develop new products and enhance existing products, the impact of competition, changes in general economy and industry conditions, and legislative, regulatory and political factors.
  • Unitedhealthcare Cancer Therapy Pathways Program Regimens

    Unitedhealthcare Cancer Therapy Pathways Program Regimens

    UnitedHealthcare Cancer Therapy Pathways Program Overview 2 Breast Cancer 3 Pancreatic Cancer 15 Melanoma 19 Colon/Rectal Cancer 23 Hepatobiliary Cancers 30 Lung Cancer, Small Cell 35 Lung Cancer, Non-Small Cell 40 Ovarian, Fallopian and Primary Peritoneal Cancer 56 Head and Neck Cancer 67 Multiple Myeloma 74 Lymphoma, Diffuse Large B-Cell 86 Lymphoma, Follicular 92 Lymphoma, Marginal Zone 97 Lymphoma, Mantle Cell 101 Change control 1 OVERVIEW Cancer Therapy Pathways Program With the rapid approval of new therapies, along with the rising cost of cancer care, pathways serve a critical role in the delivery of high-quality and high-value cancer treatments while reducing an unwarranted variation in care. The UnitedHealthcare Cancer Therapy Pathways Program aims to improve quality and value in cancer care by identifying anti-cancer regimens supported by evidence-based guidelines to help reduce total cost of care and improve outcomes. The program’s regimens are selected on the basis of clinical benefit (efficacy) and side-effect profile (toxicity). Among regimens with comparable efficacy and toxicity, additional consideration is given to the frequency of hospitalizations during therapy, duration of therapy, drug costs and total cost of care. Care decisions are between the physician and the patient The Cancer Therapy Pathways Program is not a substitute for the experience and judgment of a physician or other health care professional. Any clinician participating in the program must use independent medical judgment in the context of individual
  • Pancreatic Cancer Treatment Using Na+/K+ Atpase

    Pancreatic Cancer Treatment Using Na+/K+ Atpase

    (19) & (11) EP 1 796 688 B1 (12) EUROPEAN PATENT SPECIFICATION (45) Date of publication and mention (51) Int Cl.: of the grant of the patent: A61K 31/704 (2006.01) A61P 35/00 (2006.01) 18.05.2011 Bulletin 2011/20 (86) International application number: (21) Application number: 05794315.1 PCT/US2005/031331 (22) Date of filing: 02.09.2005 (87) International publication number: WO 2006/028969 (16.03.2006 Gazette 2006/11) (54) PANCREATIC CANCER TREATMENT USING NA+/K+ ATPASE INHIBITORS PANKREASKREBSBEHANDLUNG MIT NA+/K+-ATPASE-HEMMERN TRAITEMENT DU CANCER DU PANCREAS AU MOYEN D’INHIBITEURS D’APTASE NA+/K+ (84) Designated Contracting States: • SHARMA, Ajay AT BE BG CH CY CZ DE DK EE ES FI FR GB GR Sudbury , MA 01776 (US) HU IE IS IT LI LT LU LV MC NL PL PT RO SE SI SK TR (74) Representative: Tomkinson, Alexandra et al Bailey Walsh & Co (30) Priority: 02.09.2004 US 606684 P 5 York Place Leeds LS1 2SD (GB) (43) Date of publication of application: 20.06.2007 Bulletin 2007/25 (56) References cited: WO-A-00/45165 WO-A-03/099011 (73) Proprietor: Bionaut Pharmaceuticals Inc WO-A-2005/060951 WO-A-2005/077925 Stoneham, MA 02180 (US) US-A1- 2005 182 105 (72) Inventors: • KHODADOUST, Mehran - c/o BT Intern. Ltd. London EC4M 7SB (GB) Note: Within nine months of the publication of the mention of the grant of the European patent in the European Patent Bulletin, any person may give notice to the European Patent Office of opposition to that patent, in accordance with the Implementing Regulations.