ICD-O-3 Implementation Guidelines
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Hodgkin Lymphoma
Hodgkin Lymphoma • Lymphoid neoplasm derived from germinal center B cells. • Neoplastic cells (i.e. Hodgkin/Reed-Sternberg/LP cells) comprise the minority of the infiltrate. • Non-neoplastic background inflammatory cells comprise the majority of infiltrate. • Two biologically distinct types: 1. Classical Hodgkin Lymphoma (CHL) 2. Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) Classical Hodgkin Lymphoma • Neoplastic B (Hodgkin) cells are often take the form of Reed-Sternberg cells or variants – Classic RS, mummified and lacunar cells. • The majority of background, non-neoplastic small lymphocytes are T cells. • Unique immunophenotype that differs from most B cell lymphomas. • Divided into 4 histologic subtypes according to the background milieu: – Nodular Sclerosis – Mixed Cellularity – Lymphocyte Rich – Lymphocyte Deplete CHL - RS Cell Variants Classic RS Cell Lacunar Cells Mummified Cell Hsi ED and Golblum JR. Foundations in Diagnostic Pathology: Hematopathology. 2nd Ed. 2012 CHL - Immunophenotype CD45 CD3 CD20 Pax-5 CD30 CD15 CHL - Histologic Subtypes Nodular Sclerosis • Architecture is effaced by prominent nodules separated by dense bands of collagen. • Mixed inflammatory infiltrate composed of T cells, granulocytes, and histiocytes. • Lacunar variants are the predominant form of RS cells. Mixed Cellularity • Architecture is effaced by a more diffuse infiltrate without bands of fibrosis. • Background of lymphocytes, plasma cells, histiocytes and eosinophils. • Approximately 75% of cases are positive for EBV-encoded RNA or protein (LMP1) Lymphocyte Rich • Architecture is effaced by a nodular to vaguely nodular infiltrate of lymphocytes. • Nodules may contain regressed germinal centers. • The majority of background, non- neoplastic lymphocytes are B cells; T cells form rosettes around neoplastic cells. • Granulocytes and histiocytes are rare. Regressed germinal center Lymphocyte Deplete • Architecture is effaced by disordered fibrosis and necrosis. -
Eyelid Conjunctival Tumors
EYELID &CONJUNCTIVAL TUMORS PHOTOGRAPHIC ATLAS Dr. Olivier Galatoire Dr. Christine Levy-Gabriel Dr. Mathieu Zmuda EYELID & CONJUNCTIVAL TUMORS 4 EYELID & CONJUNCTIVAL TUMORS Dear readers, All rights of translation, adaptation, or reproduction by any means are reserved in all countries. The reproduction or representation, in whole or in part and by any means, of any of the pages published in the present book without the prior written consent of the publisher, is prohibited and illegal and would constitute an infringement. Only reproductions strictly reserved for the private use of the copier and not intended for collective use, and short analyses and quotations justified by the illustrative or scientific nature of the work in which they are incorporated, are authorized (Law of March 11, 1957 art. 40 and 41 and Criminal Code art. 425). EYELID & CONJUNCTIVAL TUMORS EYELID & CONJUNCTIVAL TUMORS 5 6 EYELID & CONJUNCTIVAL TUMORS Foreword Dr. Serge Morax I am honored to introduce this Photographic Atlas of palpebral and conjunctival tumors,which is the culmination of the close collaboration between Drs. Olivier Galatoire and Mathieu Zmuda of the A. de Rothschild Ophthalmological Foundation and Dr. Christine Levy-Gabriel of the Curie Institute. The subject is now of unquestionable importance and evidently of great interest to Ophthalmologists, whether they are orbital- palpebral specialists or not. Indeed, errors or delays in the diagnosis of tumor pathologies are relatively common and the consequences can be serious in the case of malignant tumors, especially carcinomas. Swift diagnosis and anatomopathological confirmation will lead to a treatment, discussed in multidisciplinary team meetings, ranging from surgery to radiotherapy. -
Mimics of Lymphoma
Mimics of Lymphoma L. Jeffrey Medeiros MD Anderson Cancer Center Mimics of Lymphoma Outline Progressive transformation of GCs Infectious mononucleosis Kikuchi-Fujimoto disease Castleman disease Metastatic seminoma Metastatic nasopharyngeal carcinoma Thymoma Myeloid sarcoma Progressive Transformation of Germinal Centers (GC) Clinical Features Occurs in 3-5% of lymph nodes Any age: 15-30 years old most common Usually localized Cervical LNs # 1 Uncommonly patients can present with generalized lymphadenopathy involved by PTGC Fever and other signs suggest viral etiology Progressive Transformation of GCs Different Stages Early Mid-stage Progressive Transformation of GCs Later Stage Progressive Transformation of GCs IHC Findings CD20 CD21 CD10 BCL2 Progressive Transformation of GCs Histologic Features Often involves small area of LN Large nodules (3-5 times normal) Early stage: Irregular shape Blurring between GC and MZ Later stages: GCs break apart Usually associated with follicular hyperplasia Architecture is not replaced Differential Diagnosis of PTGC NLPHL Nodules replace architecture LP (L&H) cells are present Lymphocyte- Nodules replace architecture rich classical Small residual germinal centers HL, nodular RS+H cells (CD15+ CD30+ LCA-) variant Follicular Numerous follicles lymphoma Back-to-back Into perinodal adipose tissue Uniform population of neoplastic cells PTGC –differential dx Nodular Lymphocyte Predominant HL CD20 NLPHL CD3 Lymphocyte-rich Classical HL Nodular variant CD20 CD15 LRCHL Progressive Transformation of GCs BCL2+ is -
Hodgkin's Disease Variant of Richter's Syndrome in Chronic Lymphocytic Leukaemia Patients Previously Treated with Fludarabin
research paper Hodgkin’s disease variant of Richter’s syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine Dominic Fong,1 Alexandra Kaiser,2 Summary Gilbert Spizzo,1 Guenther Gastl,1 Alexandar Tzankov2 The transformation of chronic lymphocytic leukaemia (CLL) into large-cell 1Division of Haematology and Oncology and lymphoma (Richter’s syndrome, RS) is a well-documented phenomenon. 2Department of Pathology, Innsbruck Medical Only rarely does CLL transform into Hodgkin’s lymphoma (HL). To further University, Innsbruck, Austria analyse the clinico-pathological and genetic findings in the HL variant of RS, we performed a single-institution study in four patients, who developed HL within a mean of 107 months after diagnosis of CLL. All were treated with fludarabine. Three cases were Epstein–Barr virus (EBV)-associated mixed cellularity (MC) HL, the fourth was nodular sclerosis (NS) HL without EBV association. The sites involved by HL included supra- and infradiaphragmal lymph nodes and the tonsils; stage IV disease was also documented. All patients presented with CLL treatment-resistant lymphadenopathies and B- symptoms. In two of the MC cases, molecular analysis performed on CLL samples and microdissected Hodgkin and Reed–Sternberg cells (HRSC) suggested a clonal relationship, while in NS no indication of a clonal relationship was detected. In summary, HL can occur in CLL patients at any Received 15 December 2004; accepted for site, up to 17 years after initial diagnosis, especially after treatment with publication 1 February 2005 fludarabine. The majority present with B-symptoms and CLL treatment- Correspondence: A. Tzankov MD, Department resistant lymphadenopathy, are of the MC type, clonally related to CLL and of Pathology, Innsbruck Medical University, might be triggered by an EBV infection. -
Genetics of Skin Appendage Neoplasms and Related Syndromes
811 J Med Genet: first published as 10.1136/jmg.2004.025577 on 4 November 2005. Downloaded from REVIEW Genetics of skin appendage neoplasms and related syndromes D A Lee, M E Grossman, P Schneiderman, J T Celebi ............................................................................................................................... J Med Genet 2005;42:811–819. doi: 10.1136/jmg.2004.025577 In the past decade the molecular basis of many inherited tumours in various organ systems such as the breast, thyroid, and endometrium.2 syndromes has been unravelled. This article reviews the clinical and genetic aspects of inherited syndromes that are Clinical features of Cowden syndrome characterised by skin appendage neoplasms, including The cutaneous findings of Cowden syndrome Cowden syndrome, Birt–Hogg–Dube syndrome, naevoid include trichilemmomas, oral papillomas, and acral and palmoplantar keratoses. The cutaneous basal cell carcinoma syndrome, generalised basaloid hallmark of the disease is multiple trichilemmo- follicular hamartoma syndrome, Bazex syndrome, Brooke– mas which present clinically as rough hyperker- Spiegler syndrome, familial cylindromatosis, multiple atotic papules typically localised on the face (nasolabial folds, nose, upper lip, forehead, ears3 familial trichoepitheliomas, and Muir–Torre syndrome. (fig 1A, 1C, 1D). Trichilemmomas are benign ........................................................................... skin appendage tumours or hamartomas that show differentiation towards the hair follicles kin consists of both epidermal and dermal (specifically for the infundibulum of the hair 4 components. The epidermis is a stratified follicle). Oral papillomas clinically give the lips, Ssquamous epithelium that rests on top of a gingiva, and tongue a ‘‘cobblestone’’ appearance basement membrane, which separates it and its and histopathologically show features of 3 appendages from the underlying mesenchymally fibroma. The mucocutaneous manifestations of derived dermis. -
CD20-Positive Peripheral T-Cell Lymphoma: Report of a Case After Nodular Sclerosis Hodgkin’S Disease and Review of the Literature Renee L
CD20-Positive Peripheral T-Cell Lymphoma: Report of a Case after Nodular Sclerosis Hodgkin’s Disease and Review of the Literature Renee L. Mohrmann, M.D., Daniel A. Arber, M.D. Division of Pathology, City of Hope National Medical Center, Duarte, California CASE REPORT We present a case of peripheral T-cell lymphoma co-expressing CD3 and CD20, as well as demon- A 47-year-old man presented in 1993 with a brief strating T-cell receptor gene rearrangement, in a history of right axillary lymph node enlargement patient who had been diagnosed with nodular scle- and mild fatigue. Biopsy showed nodular sclerosis rosis Hodgkin’s disease 5 years previously. Although Hodgkin’s disease. He was treated with six courses 15 cases of CD20-positive T-cell neoplasms have of mechlorethamine, vincristine, procarbazine, been previously reported in the literature, this is the prednisone/doxorubicin, bleomycin, vinblastine first report of CD20-positive T-cell lymphoma oc- chemotherapy over a period of 6 months. Clinical curring subsequent to treatment of Hodgkin’s dis- remission was achieved for 5 years. In early 1998, the patient noticed enlargement of lymph nodes in ease. The current case affords an opportunity to the posterior cervical region, which were followed review the rarely reported expression of CD20 in clinically for several months. Weight loss of 15 lbs., T-cell neoplasms as well as the relationship between fatigue, and flu-like symptoms ensued. The lymph Hodgkin’s disease and subsequently occurring non- nodes became firmer to palpation and were biop- Hodgkin’s lymphomas. In addition, the identifica- sied, showing peripheral T-cell lymphoma, diffuse tion of this case supports the suggestion that the use large-cell type. -
CD20 Over-Expression in Hodgkin-Reed-Sternberg Cells of Classical Hodgkin Lymphoma: the Neglected Quest Daniel Benharroch1, Karen Nalbandyan1, Irina Lazarev2
Journal of Cancer 2015, Vol. 6 1155 Ivyspring International Publisher Journal of Cancer 2015; 6(11): 1155-1159. doi: 10.7150/jca.13107 Research Paper CD20 Over-Expression in Hodgkin-Reed-Sternberg Cells of Classical Hodgkin Lymphoma: the Neglected Quest Daniel Benharroch1, Karen Nalbandyan1, Irina Lazarev2 1. Department of Pathology, Soroka University Medical Center, Beer-Sheva and Faculty of Health Sciences, Ben Gurion University of the Negev, Beer-Sheva, Israel 2. Department of Oncology, Soroka University Medical Center, Beer-Sheva and Faculty of Health Sciences, Ben Gurion University of the Negev, Beer-Sheva, Israel Corresponding author: Prof. Daniel Benharroch, Dept. of Pathology, Soroka University Medical Center, P.O.Box 151, Beer-Sheva, 84101, Israel. Tel: 97286400920. Fax:86232770. e-mail: [email protected] © 2015 Ivyspring International Publisher. Reproduction is permitted for personal, noncommercial use, provided that the article is in whole, unmodified, and properly cited. See http://ivyspring.com/terms for terms and conditions. Received: 2015.07.01; Accepted: 2015.08.08; Published: 2015.09.15 Abstract We have scrutinized a previously analyzed cohort of classical Hodgkin lymphoma patients for evidence of a CD20 over-expression. This was pursued in order to determine whether all the 24 (12.6%) CD20+++ patients had clinical and/or biological profiles which would warrant a separate consideration and treatment or would carry a different outcome from our 166 CD20 (-) classical Hodgkin lymphoma patients. Except for an older age and a significantly lower expression of non-sialyl-CD15 antigen, both previously described in classical Hodgkin lymphoma, no justification to exclude these CD20+++ patients from the cohort at large is apparent. -
Cylindroma of the Breast: a Case Report and Review of the Literature Amr Mahmoud*1, David H Hill2, Martin J O'sullivan2 and Michael W Bennett1
Diagnostic Pathology BioMed Central Case Report Open Access Cylindroma of the breast: a case report and review of the literature Amr Mahmoud*1, David H Hill2, Martin J O'Sullivan2 and Michael W Bennett1 Address: 1BreastCheck, National Cancer Screening Service/Mercy University Hospital, Cork, Ireland and 2BreastCheck, National Cancer Screening Service/South Infirmary Victoria University Hospital, Cork, Ireland Email: Amr Mahmoud* - [email protected]; David H Hill - [email protected]; Martin J O'Sullivan - [email protected]; Michael W Bennett - [email protected] * Corresponding author Published: 2 September 2009 Received: 25 April 2009 Accepted: 2 September 2009 Diagnostic Pathology 2009, 4:30 doi:10.1186/1746-1596-4-30 This article is available from: http://www.diagnosticpathology.org/content/4/1/30 © 2009 Mahmoud et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Cylindroma of the breast is a very rare lesion which is morphological and immunophenotypically identical to benign dermal cylindroma. We report a breast cylindroma in a previously healthy 62 year old female detected through a national breast screening program. The patient had no significant family or past medical history, and specifically no history of breast or skin diseases. The tumor consisted of well circumscribed islands of epithelial cells surrounded by a dense membrane material, and focally containing hyaline globules. At low power the islands of tumour cells formed a "jig-saw" pattern, which is typical of cylindroma, but was present within normal breast parenchyma and no had direct connection with the overlying skin. -
An Uncommon Malignant Adnexal Neoplasm at an Unusual Location
DOI: 10.7860/JCDR/2019/41333.12966 Case Report Syringocystadenocarcinoma Papilliferum- An Uncommon Malignant Adnexal Pathology Section Neoplasm at an Unusual Location SARANYA SHANKAR1, BN KUMARGURU2, CA ARATHI3, AS RAMASWAMY4, R PRASHANTH5 ABSTRACT Syringocystadenocarcinoma Papilliferum (SCACP) is a rare adnexal malignant neoplasm. Most of the cases present with a long standing mass with a sudden progression in size. A 60-year-old female presented with history of swelling over the antero- lateral aspect of left thigh since three years. The lesion was associated with history of pain and ulceration. Grossly, the external surface showed three ulcerated areas. The largest ulcer measured 4×5.5 cm. On cut section, the tumour showed variegated appearance consisting of grey-white to pink areas, multiple cysts, focal necrosis and hemorrhagic areas. Microscopically, tumour cells were arranged in papillary configuration, solid sheets, tubular and acinar pattern. Individual tumour cells showed pleomorphic vesicular nucleus and mitotic figures. The intervening connective tissue showed dense chronic inflammatory cell infiltrate composed of lymphocytes, plasma cells and eosinophils. Also seen were areas of necrosis and Gamna-Gandy body. Based on histological features, a diagnosis of malignant appendageal tumour of the skin with apocrine differentiation, favouring SCACP was offered. By Immunohistochemistry (IHC), tumour cells showed focal positivity for CEA and negative for GCDFP-15. IHC may be helpful, but a pathologist has to primarily depend on the histopathological characteristics of the lesion for diagnosing the condition. Keywords: Cysts, Necrosis, Plasma cells, Ulcer CASE REPORT appearance consisting of grey-white to pink areas, multiple cysts, A 60-year-old female presented with history of swelling over focal necrosis and hemorrhagic areas. -
Disseminated Syringomas of the Upper Extremities in a Young Woman
Open Access Case Report DOI: 10.7759/cureus.3619 Disseminated Syringomas of the Upper Extremities in a Young Woman Kavina Patel 1 , Ashley D. Lundgren 2 , Ammar M. Ahmed 2 , Anthony C. Soldano 3 1. Dermatology, University of Texas Health Science Center, San Antonio, USA 2. Dermatology, Dell Medical School - The University of Texas, Austin, USA 3. Dermatology, Clinical Pathology Associates, Austin, USA Corresponding author: Kavina Patel, [email protected] Abstract Syringomas are benign, eccrine sweat gland tumors frequently found on the eyelids and neck in post- pubescent women and may present in healthy individuals or be associated with various medical comorbidities. We present a case of an otherwise healthy 19-year-old female with an abrupt onset of disseminated syringomas on the bilateral forearms and dorsal hands. Eruptive acral syringomas have not been previously reported in adolescents, and this diagnosis should be considered in patients presenting with a papular eruption on the hands and forearms. Categories: Dermatology Keywords: acral syringoma, upper extremity, eruptive, papule, eccrine gland Introduction Syringomas are benign, adnexal tumors of unknown etiology that derive from eccrine sweat ducts [1]. They are most commonly located bilaterally on the inferior eyelids of post-pubescent women and present as multiple, white to yellow, 1 to 3-millimeter papules; on occasion, they are found in alternate locations such as the cheeks, axillae, abdomen, chest, and groin [2]. A clear cell variant, where cells have colorless cytoplasm due to glycogen build-up, is commonly associated with diabetes mellitus [3]. Eruptive syringomas are a less common presentation and may surface abruptly in adolescents, typically on the abdomen. -
Current Diagnosis and Treatment Options for Cutaneous Adnexal Neoplasms with Apocrine and Eccrine Differentiation
International Journal of Molecular Sciences Review Current Diagnosis and Treatment Options for Cutaneous Adnexal Neoplasms with Apocrine and Eccrine Differentiation Iga Płachta 1,2,† , Marcin Kleibert 1,2,† , Anna M. Czarnecka 1,* , Mateusz Spałek 1 , Anna Szumera-Cie´ckiewicz 3,4 and Piotr Rutkowski 1 1 Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; [email protected] (I.P.); [email protected] (M.K.); [email protected] (M.S.); [email protected] (P.R.) 2 Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland 3 Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; [email protected] 4 Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, 00-791 Warsaw, Poland * Correspondence: [email protected] or [email protected] † Equally contributed to the work. Abstract: Adnexal tumors of the skin are a rare group of benign and malignant neoplasms that exhibit morphological differentiation toward one or more of the adnexal epithelium types present in normal skin. Tumors deriving from apocrine or eccrine glands are highly heterogeneous and represent various histological entities. Macroscopic and dermatoscopic features of these tumors are unspecific; therefore, a specialized pathological examination is required to correctly diagnose patients. Limited Citation: Płachta, I.; Kleibert, M.; treatment guidelines of adnexal tumor cases are available; thus, therapy is still challenging. Patients Czarnecka, A.M.; Spałek, M.; should be referred to high-volume skin cancer centers to receive an appropriate multidisciplinary Szumera-Cie´ckiewicz,A.; Rutkowski, treatment, affecting their outcome. -
Marketing Fragment 6 X 10.5.T65
Cambridge University Press 978-0-521-68563-4 - A Practical Guide to Human Cancer Genetics, Third Edition Shirley Hodgson, William Foulkes, Charis Eng and Eamonn Maher Index More information Index Note: page numbers in italics refer to figures and tables N-acetyltransferase alcohol, laryngeal cancer 29 acetylation 61 Allgrove syndrome 43 activity 118 alpha-1 antitrypsin deficiency, hepatocellular achalasia, oesophageal cancer 43 carcinoma 49 acoustic neuroma alphafetoprotein 214 NF2 235–6 alveolar cell carcinoma 30, 31 see also vestibular schwannoma amine compounds, bladder cancer 118, 119 acromegaly, familial 37 Amsterdam Criteria 203 actinic keratosis 148 androgen-insensitivity states, incomplete 111 acute lymphoblastic leukaemia (ALL) angiomyolipomas 247 classification 233 aniridia, sporadic 112, 113, 114 cytogenetic analysis 123–4 anticipation translocations 168 AML 123 acute myeloid leukaemia (AML) 183–4 leukaemia 122 anticipation 123 APC gene classification 120 mutations 190 Klinefelter syndrome 214 benign 191 Kostman syndrome 215 Gardner syndrome 186 acute myelomonocytic leukaemia 233, 234 Turcot syndrome 251 acute promyelocytic leukaemia 168 polymorphism in colon cancer 60 adenocarcinoma, see renal cell carcinoma APKD1 gene 249–50 adenosine deaminase deficiency 245 arsenic 51, 147 adrenal gland tumours asbestos 30 adrenocortical adenoma/carcinoma astrocytoma 13–14 39–40 ataxia telangiectasia 167–9 phaeochromocytoma 37–9 breast cancer 168 adrenocortical hyperplasia 40 gastric adenocarcinoma 46 aflatoxin 48, 51 locus 168 Aicardi syndrome