A Proposal for a Standardized System of Reporting Human Lymph Node Morphology in Relation to Immunological Function'

Total Page:16

File Type:pdf, Size:1020Kb

A Proposal for a Standardized System of Reporting Human Lymph Node Morphology in Relation to Immunological Function' J Clin Pathol: first published as 10.1136/jcp.26.5.317 on 1 May 1973. Downloaded from J. clin. Path., 1973, 26, 317-331 A proposal for a standardized system of reporting human lymph node morphology in relation to immunological function' H. COTTIER, J. TURK, AND L. SOBIN2 From the Department of Pathology, University of Berne, Switzerland, the Department of Pathology, Royal College ofSurgeons, London, and the World Health Organization, Geneva SYNOPSIS This memorandum proposes a standardized system of reporting the histology of human lymph nodes based on commonly used simple staining techniques. The purpose is to provide a uniform, internationally acceptable system by which the histological structure of lymph nodes can be correlated with other parameters of immunological status. The proposed protocols are intended to provide information that is not available in conventional written reports that use such terms as 'hyperplasia' or 'nonspecific lymphadenitis'. The increasing use of immunological tests in of reporting. Correlation between lymph node copyright. clinical medicine has demonstrated a need for morphology and other parameters of immunological standardized reporting of the appearance of lymph response, associated with humoral antibody pro- node sections. Although there have been many duction and cell-mediated immunity, could assist advances in the knowledge of lymph node structure the physician in his final assessment and treatment in the past 10 years as a result of correlation be- of the patient. This approach may be of special tween lymph node changes and immunological value in the management of patients with disorders function, these have not been reflected in the reports such as neoplastic and infectious diseases. http://jcp.bmj.com/ generally issued from histopathology laboratories. In the present proposals care has been taken to Such changes are now well recognized by immuno- use terms that are descriptive rather than those logists but have not yet been adopted by histo- based on as yet debatable functional interpretations. pathologists, since until now emphasis has been Thus the term 'paracortical area' has been used in given almost exclusively to major pathological preference to 'thymus-dependent area' and the lesions rather, than to changes associated with term 'large lymphoid cell' in preference to 'immuno- variations in the immune response. The present blast', 'plasmablast', etc. on September 25, 2021 by guest. Protected proposals have been prepared in the hope that pathologists will also comment in their reports on Recommendations for Handling and Processing of structural variations associated with immunological the Tissue function. This could add a further dimension to the physician's analysis of the immunological Unusual care should be exerted during excision status of the patient analogous to information on of the lymph node. It is important that the node the endocrine state as determined by endometrial itself should not be handled with forceps as com- biopsies. Since the lymph node is one of the tissues pression of the tissue during the procedure can result most frequently examined by histopathologists, in distortion and prolonged manipulation during a considerable amount of information is currently surgery can produce acute inflammatory changes. being lost because of the lack of a consistent system Forceps should be applied only to the surrounding tissue and not to the node. It is strongly recommend- "Reprinted in slightly modified form, and with the addition of a bibliography, from Bulletin of the World Health Organization (1972), ed that the whole node should be removed rather 47, 375-406. than fragments of the tissue. 'Requests for reprints should be addressed to Dr L. Sobin, WHO, Once removed the node should be cut trans- 1211 Geneva, 27 Switzerland. versely through its short axis in order to permit 317 J Clin Pathol: first published as 10.1136/jcp.26.5.317 on 1 May 1973. Downloaded from 318 H. Cottier, J. Turk, and L. Sobini penetration of the fixative. For a comparable result of fixation, dehydration, and embedding assessment of immunological function it is neces- and an absolute assessment of size is omitted from sary to examine a transverse section cut along the the following description. short axis from the middle portion of the node; Recent developments in cell kinetics have shown this will contain representative areas of the cortex that the non-proliferating precursors of all immuno- and the medulla. logically active cells have the morphological ap- It is understood that as a routine procedure it pearance of small lymphocytes. These cells when may be difficult to use fixatives other than neutral proliferating and differentiating acquire a common formalin. However, Carnoy's solution is better appearance and are referred to here as 'large for the assessment of cellular morphology and is lymphoid cells'. During the cell-mediated immune especially useful for subsequent staining with response these cells will divide into elements that are methyl green and pyronin. Although for most pur- usually morphologically indistinguishable in tissue poses haematoxylin-eosin staining is adequate for sections from the precursor small lymphocyte. the examination of the tissue, Giemsa stain or Similar cells when proliferating and differentiating methyl green and pyronin allow very rapid assess- into plasma cells start off with the same appearance ment at low-power magnification of the immunolo- and, therefore, should also be called 'large lymphoid gically important cells and structures such as plasma cells'. It is recognized that in tissue sections there are cells and germinal centres. cells that appear identical under the light microscope with small lymphocytes but examination under the Recommended Nomenclature for Cell Types in the electron microscope and the use of immuno- Lymph Nodes as Seen in Histological Sections histological techniques indicate that they are involved in immunoglobulin synthesis. However, Ideally, the nomenclature of tissue structures and they cannot be recognized as such without the use cells of the lymphoid and plasma cell systems of these methods. should be based on functional as well as morpholo- gical criteria. Although one may say definitely LARGE LYMPHOID CELLS (figs 1, 3, 14) copyright. that a mature or immature plasma cell is producing These cells have large vesicular nuclei with prominent immunoglobulin, one cannot ascribe a function to nucleoli and moderate amounts of basophilic and a given large lymphoid cell or a small lymphocyte pyroninophilic cytoplasm. They are frequently seen purely on morphological appearance. For example, in mitosis. Cells with these characteristics occur small lymphocytes morphologically identical in during the proliferation of lymphocytes during a tissue sections can be the precursors of plasma cell-mediated immune response as well as in relation http://jcp.bmj.com/ cells involved in immunoglobulin synthesis or of to plasma cell proliferation and in germinal centres the effector cells of cell-mediated immunity. The in humoral antibody responses, when they may latter also have the morphological appearance of contain immunoglobulin. small lymphocytes. Similarly, it may be difficult to distinguish the large proliferating precursor cells of elements involved in cell-mediated immunity from MEDIUM-SIZED LYNMPHOCYTES (figs 1, 3) those involved in immunoglobulin synthesis. Even There is a range of nuclear and cytoplasmic size the precursors of other blood cell lines resemble between large lymphoid cells and small lymphocytes. on September 25, 2021 by guest. Protected may This large lymphoid cells or small lymphocytes. may reflect transition between a proliferating and Terms such as immunoblast, plasmablast, or resting state as well as some degree of functional haemocytoblast indicate a function that cannot be differentiation. The term 'medium-sized lymphocyte' directly determined from the histological examination is used to describe cells with any appearance in of lymphoid tissue alone. The use of these terms between that of large lymphoid cells and small is often based on circumstantial evidence and they lymphocytes. should be applied only to describe large lymphoid cells in certain controlled immunological situations. SMALL LYMPHOCYTES (figs 2, 3, 4) For practical reasons, the following nomenclature These are defined according to conventional has been based essentially on the morphological nomenclature. and staining characteristics of cells that are easily recognizable with the aid of simple staining methods MATURE PLASMA CELLS (fig 4) and the light microscope. In this way, it should This term is restricted to cells with a typical eccentric be possible to obtain agreement between patholo- cart-wheel nucleus of similar size to that of a small gists and immunologists on their microscopic lymphocyte, abundant basophilic and pyronino- findings. The sizes of cells are subject to change as a philic cytoplasm, and a pale paranuclear area. J Clin Pathol: first published as 10.1136/jcp.26.5.317 on 1 May 1973. Downloaded from copyright. Fig 2 Fig 1 Corticomedullary junction ofa lymph node. LL, large lymphoid cell; RE, reticulum cell or endothelial http://jcp.bmj.com/ cell (endothelial cells may resemble reticulum cells in tangential sections); NG, neutrophilic granulocyte. Note that in sections only a few micrometres in thickness, and without special staining, it is often difficult to distinguish between medium-sized lymphocytes and a cross-sectioned reticulum
Recommended publications
  • WSC 10-11 Conf 7 Layout Master
    The Armed Forces Institute of Pathology Department of Veterinary Pathology Conference Coordinator Matthew Wegner, DVM WEDNESDAY SLIDE CONFERENCE 2010-2011 Conference 7 29 September 2010 Conference Moderator: Thomas Lipscomb, DVM, Diplomate ACVP CASE I: 598-10 (AFIP 3165072). sometimes contain many PAS-positive granules which are thought to be phagocytic debris and possibly Signalment: 14-month-old , female, intact, Boxer dog phagocytized organisms that perhaps Boxers and (Canis familiaris). French bulldogs are not able to process due to a genetic lysosomal defect.1 In recent years, the condition has History: Intestine and colon biopsies were submitted been successfully treated with enrofloxacin2 and a new from a patient with chronic diarrhea. report indicates that this treatment correlates with eradication of intramucosal Escherichia coli, and the Gross Pathology: Not reported. few cases that don’t respond have an enrofloxacin- resistant strain of E. coli.3 Histopathologic Description: Colon: The small intestine is normal but the colonic submucosa is greatly The histiocytic influx is reportedly centered in the expanded by swollen, foamy/granular histiocytes that submucosa and into the deep mucosa and may expand occasionally contain a large clear vacuole. A few of through the muscular wall to the serosa and adjacent these histiocytes are in the deep mucosal lamina lymph nodes.1 Mucosal biopsies only may miss the propria as well, between the muscularis mucosa and lesions. Mucosal ulceration progresses with chronicity the crypts. Many scattered small lymphocytes with from superficial erosions to patchy ulcers that stop at plasma cells and neutrophils are also in the submucosa, the submucosa to only patchy intact islands of mucosa.
    [Show full text]
  • An Electron Microscope Study of Histiocyte Response to Ascites Tumor Homografts*
    An Electron Microscope Study of Histiocyte Response to Ascites Tumor Homografts* L. J. JOURNEYANDD. B. Aiviosf (Experimental Biology Department, Roswell Park Memorial Institute, fíujfaln.New York) SUMMARY When the ascites forms of the DBA/2 lymphoma L1210 or the C57BL E.L. 4 lymphoma are injected into C3H mice, host histiocytes (macrophages) accumulate and are responsible for the destruction of a large number of tumor cells. Many of the tumor cells, often apparently intact, are ingested. The ingestion process is rapid and depends upon invagination of an area of the histiocyte with simultaneous projection of cytoplasmic fimbriae which complete the encirclement. The earliest change seen in the enclosed cell is shrinkage; digestion of the cell wall and cytoplasmic elements fol lows. Phagocytosis accounts for only a proportion of the cells destroyed by histiocytes. Other cells are probably destroyed when their cell membrane is broken down in an area in contact with a histiocyte, apparently permitting fusion of the two cytoplasms. Weaver and his colleagues (13) observed that cytes and details some of the concomitant changes host cells were frequently found in close associa occurring in the histiocytes themselves. tion with tumor cells and described death of both host and incompatible tumor cell after a period of MATERIALS AND METHODS contact. Gorer (9) found that the predominant In a series of experiments 20 X IO7 cells from host cell in the ascites fluid during tumor rejection rapidly growing ascites populations of the lympho- was the histiocyte. This finding was confirmed, and mas E. L. 4 or L1210 native to C57BL and DBA/ some quantitative measurements were made by 2, respectively, were injected into the peritoneal one of us (1) and later by Weiser and his col cavity of young adult male C3H/He mice.
    [Show full text]
  • 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
    [Show full text]
  • Progress in Understanding the Pathogenesis of Langerhans Cell Histiocytosis: Back to Histiocytosis X?
    review Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? Marie-Luise Berres,1,2,3,4 Miriam Merad1,2,3 and Carl E. Allen5,6 1Department of Oncological Sciences, Mount Sinai School of Medicine, 2Tisch Cancer Institute, Mount Sinai School of Medicine, 3Immunology Institute, Mount Sinai School of Medicine, New York, NY, USA, 4Department of Internal Medicine III, University Hospital, RWTH Aachen, Aachen, Germany, 5Texas Children’s Cancer Center, and 6Baylor College of Medicine, Houston, TX, USA Summary Langerhans cell histiocytosis (LCH) is the most common his- tiocytic disorder, arising in approximately five children per Langerhans cell histiocytosis (LCH), the most common million, similar in frequency to paediatric Hodgkin lym- histiocytic disorder, is characterized by the accumulation of phoma and acute myeloid leukaemia (AML) (Guyot-Goubin CD1A+/CD207+ mononuclear phagocytes within granuloma- et al, 2008; Stalemark et al, 2008; Salotti et al, 2009). The tous lesions that can affect nearly all organ systems. Histori- median age of presentation is 30 months, though LCH is cally, LCH has been presumed to arise from transformed or reported in adults in approximately one adult per million, pathologically activated epidermal dendritic cells called Lan- both as unrecognized chronic paediatric disease and de novo gerhans cells. However, new evidence supports a model in disease (Baumgartner et al, 1997). There are occasional which LCH occurs as a consequence of a misguided differen- reports of affected non-twin siblings and multiple cases in tiation programme of myeloid dendritic cell precursors. one family, though it is not clear if this is significantly more Genetic, molecular and functional data implicate activation frequent than one would expect by chance (Arico et al, of the ERK signalling pathway at critical stages in myeloid 2005).
    [Show full text]
  • Histiocyte Society LCH Treatment Guidelines
    LANGERHANS CELL HISTIOCYTOSIS Histiocyte Society Evaluation and Treatment Guidelines April 2009 Contributors: Milen Minkov Vienna, Austria Nicole Grois Vienna, Austria Kenneth McClain Houston, USA Vasanta Nanduri Watford, UK Carlos Rodriguez-Galindo Memphis, USA Ingrid Simonitsch-Klupp Vienna, Austria Johann Visser Leicester, UK Sheila Weitzman Toronto, Canada James Whitlock Nashville, USA Kevin Windebank Newcastle upon Tyne, UK Disclaimer: These clinical guidelines have been developed by expert members of the Histiocyte Society and are intended to provide an overview of currently recommended treatment strategies for LCH. The usage and application of these clinical guidelines will take place at the sole discretion of treating clinicians who retain professional responsibility for their actions and treatment decisions. The following recommendations are based on current best practices in the treatment of LCH and are not necessarily based on strategies that will be used in any upcoming clinical trials. The Histiocyte Society does not sponsor, nor does it provide financial support for, the treatment detailed herein. 1 Table of Contents INTRODUCTION...................................................................................................... 3 DIAGNOSTIC CRITERIA......................................................................................... 3 PRETREATMENT CLINICAL EVALUATION ......................................................... 3 1. Complete History........................................................................................
    [Show full text]
  • Download PDF (3467K)
    Studies on Phagocytosis and Fate of Histiocyte in Subcutaneous Connective Tissue A Study of Histiocyte with Electron Microscope By Michio Kunichika Department of Anatomy, Yamaguchi University School of Medicine (Director : Prof. Gako Jimbo) Chapter I. Introduction So far the phagocyte has been studied with only the light microscope and the details of the ultramicroscopic structure, which exceeds the limitation of the resolution power of 0.2p, has not been clarified. In 1938, the electron microscope was invented by B or r i es and R u s k a and, in 1948, the ultra-microtom method was established by Peas e, Baker and others. Thus, in these several years, cytomor- phology has made a great progress. By means of the electron microscope (abbreviated as EM hereafter) the finer structure of the cell has gradually been clarified and it is generally accepted that the cell membrane is a real membrane of a definite thickness. At present the assumption that, when a foreign body is attached, the cell membrane once disappears allowing the invasion of the foreign body into the cytoplasm is untenable. Pal a d e also reported that the endplasmic reticulum was communicated with the pericellular space and, presumably, was the apparatus the function of which was exchange of substance with the medium around the cell. U c h i n o (1957) studied the phagocyte in the subcutaneous connective tissue and stated that foreign body particles were carried to the so-called food vacuole via E.R. and were filled in it when they were numer- ous and were distributed on the inner surface of the limiting membrane of the vacuole when they were rare, and that indian ink and sepia melanin granules were chemically stable and did not seem to have been influenced by the digesting action in food vacuole though phagocytized substance was usually digested in it.
    [Show full text]
  • Heterogeneity of HLA-DR-Positive Histiocytes in Human Intestinal Lamina Propria: a Combined Histochemical and Immunohistological Analysis
    J Clin Pathol: first published as 10.1136/jcp.36.4.379 on 1 April 1983. Downloaded from J Clin Pathol 1983;36:379-384 Heterogeneity of HLA-DR-positive histiocytes in human intestinal lamina propria: a combined histochemical and immunohistological analysis WS SELBY, LW POULTER, S HOBBS, DP JEWELL, G JANOSSY From the Department ofGastroenterology, John Radcliffe Hospital, Oxford, and Department of Immunology, Royal Free Hospital School of Medicine, London SUMMARY HLA-DR-positive histiocytes in the lamina propria of the human intestine have been characterised using combined histochemical and immunohistological techniques. In the small intestine, 80-90% of the HLA-DR+ histiocytes had irregular surfaces with stellate processes, and exhibited strong membrane adenosine triphosphatase (ATPase) activity, but weak acid phos- phatase (ACP) and non-specific esterase (NSE) activities (HLA-DR+ ACP+'- NSA+'- ATP++; type 1 cell). In contrast, in the lamina propria of the colon the majority (60-70%) of HLA-DR+ cells were large, round cells with strong ACP and NSE activities but no detectable ATPase activity (HLA-DR+ ACP++ NSE++ ATP+'-; type 2 cell). The colon also contained a population of type 1 cells (30-40%). In active inflammatory bowel disease affecting the colon a third population of HLA-DR+ histiocytes was seen. These cells were irregular in outline, with many processes, and were ACP++ NSE+ ATP+'- (type 3 cell). The type 3 cells appeared to replace type 2 cells. After treatment, the appearances returned to normal. These findings suggest that the different populations of HLA-DR+ histiocytes in the human intestine may have several functions, reflecting the different forms of antigen present in the intestine.
    [Show full text]
  • Histiocytic Disorders Amir H
    PHC15 12/06/2006 04:24PM Page 340 15 Histiocytic disorders Amir H. Shahlaee and Robert J. Arceci of phagocytosis of foreign material, antigen processing, and Introduction antigen presentation to lymphocytes. This system, recog- nized in part through the work of Metchnikoff, was originally The histiocytoses are a diverse group of hematologic dis- termed the reticuloendothelial system by Ludwig Aschoff orders defined by the pathologic infiltration of normal tissues in the early part of the twentieth century.3,4 The central cell by cells of the mononuclear phagocyte system (MPS). The of this system, the mononuclear phagocyte or histiocyte, heterogeneity of this family of disorders, a direct result of the represents a group of anatomically and functionally distinct biologic variability of the cells of the MPS and the tissues they cells arising from a common precursor, the hematopoietic inhabit, makes the study of these diseases one of the most stem cell. intriguing yet complex areas of modern hematology. Cells of the MPS have a wide range of morphologic, anatomic Advances in basic hematology and immunology over the and functional characteristics that make classification of this last two decades have significantly enhanced our under- system difficult. Our ability to identify and classify the cells standing of the histiocytic disorders. It is now accepted that of the MPS has advanced in parallel with developments in the pathogenic cells central to the development of the histio- basic hematology and immunology. As our knowledge of the cytoses arise from a common hematopoietic progenitor. More molecular biology regulating hematopoiesis has improved, specifically, the ability to molecularly identify the hemato- we have been able to identify specific characteristics that poietic cells has enabled us to classify the histiocytoses based have enabled us to classify the cells of the MPS.
    [Show full text]
  • Hematopathology
    320A ANNUAL MEETING ABSTRACTS expression of ALDH1 was analyzed using mouse monoclonal ALDH1 antibody (BD Conclusions: Based on the present results we conclude that miR29ab1 ko’s have Biosciences, San Jose, CA). Correlations between ALDH1 expression and clinical and decreased hematopoietic stem cell population compared to the wild types and that histological parameters were assessed by Pearson’s Chi-square and M-L Chi-square miR29ab1 might have an important role in the maintenance of this cell population. tests. Survival curves were generated using the Kaplan-Meier method and statistical Also, miR29 genes might regulate immunity and life span, since both miR29ab1 and differences by log rank test. miR29ab1/b2c ko’s seem to have markedly decreased life spans. Results: Majority of the tumors (116, 63%) showed stromal staining only, 21 (11%) tumors showed both epithelial and stromal expression, 47 (26%) tumors did not show 1347 The Majority of Immunohistochemically BCL2 Negative FL Grade either epithelial or stromal staining. The normal salivary gland showed epithelial I/II Carry A t(14;18) with Mutations in Exon 1 of the BCL2 Gene and Can expression only. Statistical analyses did not show any correlation between tumor pattern, Be Identifi ed with the BCL2 E17 Antibody tumor size, the presence of perineural invasion and the patterns of ALDH1 expression. P Adam, R Baumann, I Bonzheim, F Fend, L Quintanilla-Martinez. Eberhard-Karls- The survival analysis using Kaplan-Meier method and log rank test did not show any University, Tubingen, Baden-Wurttemberg, Germany. signifi cant differences among the three patterns of ALDH1 expression with survival.
    [Show full text]
  • I M M U N O L O G Y Core Notes
    II MM MM UU NN OO LL OO GG YY CCOORREE NNOOTTEESS MEDICAL IMMUNOLOGY 544 FALL 2011 Dr. George A. Gutman SCHOOL OF MEDICINE UNIVERSITY OF CALIFORNIA, IRVINE (Copyright) 2011 Regents of the University of California TABLE OF CONTENTS CHAPTER 1 INTRODUCTION...................................................................................... 3 CHAPTER 2 ANTIGEN/ANTIBODY INTERACTIONS ..............................................9 CHAPTER 3 ANTIBODY STRUCTURE I..................................................................17 CHAPTER 4 ANTIBODY STRUCTURE II.................................................................23 CHAPTER 5 COMPLEMENT...................................................................................... 33 CHAPTER 6 ANTIBODY GENETICS, ISOTYPES, ALLOTYPES, IDIOTYPES.....45 CHAPTER 7 CELLULAR BASIS OF ANTIBODY DIVERSITY: CLONAL SELECTION..................................................................53 CHAPTER 8 GENETIC BASIS OF ANTIBODY DIVERSITY...................................61 CHAPTER 9 IMMUNOGLOBULIN BIOSYNTHESIS ...............................................69 CHAPTER 10 BLOOD GROUPS: ABO AND Rh .........................................................77 CHAPTER 11 CELL-MEDIATED IMMUNITY AND MHC ........................................83 CHAPTER 12 CELL INTERACTIONS IN CELL MEDIATED IMMUNITY ..............91 CHAPTER 13 T-CELL/B-CELL COOPERATION IN HUMORAL IMMUNITY......105 CHAPTER 14 CELL SURFACE MARKERS OF T-CELLS, B-CELLS AND MACROPHAGES...............................................................111
    [Show full text]
  • Mast Cell Sarcoma: a Rare and Potentially Under
    Modern Pathology (2013) 26, 533–543 & 2013 USCAP, Inc. All rights reserved 0893-3952/13 $32.00 533 Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications Russell JH Ryan1, Cem Akin2,3, Mariana Castells2,3, Marcia Wills4, Martin K Selig1, G Petur Nielsen1, Judith A Ferry1 and Jason L Hornick2,5 1Pathology Service, Massachusetts General Hospital, and Harvard Medical School, Boston, MA, USA; 2Mastocytosis Center, Harvard Medical School, Boston, MA, USA; 3Department of Medicine, Harvard Medical School, Boston, MA, USA; 4Seacoast Pathology / Aurora Diagnostics, Exeter, NH and 5Department of Pathology, Brigham and Women’s Hospital, and Harvard Medical School, Boston, MA, USA Mast cell sarcoma is a rare, aggressive neoplasm composed of cytologically malignant mast cells presenting as a solitary mass. Previous descriptions of mast cell sarcoma have been limited to single case reports, and the pathologic features of this entity are not well known. Here, we report three new cases of mast cell sarcoma and review previously reported cases. Mast cell sarcoma has a characteristic morphology of medium-sized to large epithelioid cells, including bizarre multinucleated cells, and does not closely resemble either normal mast cells or the spindle cells of systemic mastocytosis. One of our three cases arose in a patient with a remote history of infantile cutaneous mastocytosis, an association also noted in one previous case report. None of our three cases were correctly diagnosed as mast cell neoplasms on initial pathological evaluation, suggesting that this entity may be under-recognized. Molecular testing of mast cell sarcoma has not thus far detected the imatinib- resistant KIT D816V mutation, suggesting that recognition of these cases may facilitate specific targeted therapy.
    [Show full text]
  • Histiocytosis an Introduction
    Br. J. Cancer SI -S3 '." Macmillan Press Ltd., 1994 Br. J. Cancer (1994), 70, (Suppl. XXIII) SI-S3 1994 Histiocytosis An Introduction Jon Pritchard FRCP' & Valerie Broadbent FRCP2 'Department of Haematology/Oncology, Hospitalfor Sick Children, Great Ormond Street, London, WCIN 3JH, England. 2Consultant Paediatric Oncologist, Paediatric Oncology Unit, Box No 181, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 2QQ, England. Summary The Histiocytoses are a group of rare and puzzling multisystem disorders, currently regarded as non-malignant but often treated with 'cancer chemotherapy'. In this article, the origins of histiocytes and of the Histiocyte Society's classification of the Histiocytoses are described with suggested minor modifications to the classification. The current nomenclature for the 2 principal diseases, now named 'Langerhans cell histiocytosis' and 'Haemophagocytic Lymphohistiocytosis', is less confusing than the terms originally chosen. The article sets the scene for the succeeding papers, which focus on 'Langerhans cell histiocytosis'. Histiocytosis Histiocytes (Gk = iatos = tissue, ic(6TTapo = cell) are formed three disorders known as "eosinophilic granuloma", "Hand- in the bone marrow (Figure 1) from a pluripotential self- Schuller-Christian Disease" and "Letterer-Siwe Disease" renewing haemopoietic stem cell via a colony-forming cell were actually part of a spectrum of one disease process, for (the CFU-GM) capable of differentiation only into granu- which he coined the term "Histiocytosis X" (Lichtenstein, locytes and cells of the monocyte lineage. Histiocytes are of 1953). Nezelof et al., identified a Langerhans-like cell as two types (a) mononuclear phagocytes and (b) 'professional' diagnostic of the process (Nezelof et al., 1973) and the desig- antigen-presenting cells.
    [Show full text]