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CONTEXT.: Urinary and Male Genital Tumours is the 8th volume of the World Health Organization Classification of Tumours series, 5th edition. Released in hard copy in September 2022, it presents an update to the classification of male genital and urinary tumors in the molecular age. Building upon previous volumes in this series, significant effort has been made to harmonize terminology across organ systems for biologically similar tumors (eg, neuroendocrine tumors). Genomic terminology has been standardized and genetic syndromes covered more comprehensively. This review presents a concise summary of this volume highlighting new entities, notable modifications relative to the 4th edition, and elements of relevance to routine clinical practice. OBJECTIVE.: To provide a comprehensive update on the World Health Organization classification of urinary and male genital tumors, highlighting updated diagnostic criteria and terminology. DATA SOURCES.: The 4th and 5th editions of the World Health Organization Classification of Tumours: Urinary and Male Genital Tumours. CONCLUSIONS.: The World Health Organization has made several changes in the 5th edition of the update on urinary and male genital tumors that pathologists need to be aware of for up-to-date clinical practice.
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Histiocytic neoplasms in the children are very rare, and histiocytoses can occur in the perinatal period. The presumed origins and presentation of specific histiocytoses in the pediatric age group are described. Common and newly described histiocytoses are presented including Langerhans cell histiocytosis, Rosai-Dorfman disease, histiocytic sarcoma, ALK positive histiocytosis, and hemophagocytic lymphohistiocytosis. Molecular findings common to pediatric histiocytoses are also discussed.
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Histiocitose de Células de Langerhans , Histiocitose Sinusal , Histiocitose , Humanos , Criança , Histiócitos , Transdiferenciação Celular , Histiocitose de Células de Langerhans/diagnósticoRESUMO
Histiocytic, dendritic, and stromal cell lesions that occur in the spleen are challenging diagnostically, not well studied due to their rarity, and therefore somewhat controversial. New techniques for obtaining tissue samples also create challenges as splenectomy is no longer common and needle biopsy does not afford the same opportunity for examination of tissue. Characteristic primary splenic histiocytic, dendritic, and stromal cell lesions are presented in this paper with new molecular genetic findings in some entities that help differentiate these lesions from those occurring in non-splenic sites, such as soft tissue, and identify possible molecular markers for diagnosis.
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Neoplasias Esplênicas , Humanos , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/patologia , Esplenectomia/métodos , BiomarcadoresRESUMO
BACKGROUND: Oral ulcers represent a full thickness loss of the mucosal epithelium leading to exposure of the submucosal connective tissue. These are common and usually self-limited lesions, although they may sometimes result from neoplasms, most commonly squamous cell carcinoma. Lymphoproliferative disorders may be difficult to diagnose in apthous ulcers since they mimic reactive inflammation. METHODS: This review presents ten rare oral lymphoid proliferations which should not be missed when assessing oral ulcer biopsies. RESULTS: The ten lesions include several with diagnostic cells which look similar to the histiocytes of a reactive inflammatory ulcer, including Rosai-Dorfman disease, reticulohistiocytoma, Langerhans cell histiocytosis, and traumatic ulcerative granuloma. Other lesions, such as EBV-positive mucocutaneous ulcer, extranodal marginal zone lymphoma of mucosal-associated lymphoid tissue, and plasmablastic lymphoma have lymphoid and/or plasma cell differentiation that mimic the reactive lymphocytes and plasma cells found in reactive ulcers. Two dendritic cell lesions, follicular dendritic cell sarcoma and blastic plasmacytoid dendritic cell neoplasm, both have distinct phenotypes which are required to make an accurate diagnosis. CONCLUSION: Each of these lesions are diagnosed by evaluating their histology, along with their phenotypic profile, which is sometimes enhanced by pertinent molecular findings.
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Transtornos Linfoproliferativos , Úlceras Orais , Humanos , Úlceras Orais/patologia , Biópsia , Transtornos Linfoproliferativos/patologia , HistiócitosRESUMO
Splenic lesions are commonly discovered incidentally at imaging, without clinical signs or symptoms that may aid in diagnosis. As such, the differential diagnosis and subsequent management are based primarily on imaging characteristics. Much has been written about the myriad pathologic conditions that can occur in the spleen; however, there is little guidance on the approach to an incidental splenic mass. Applying an approach frequently used in imaging to the splenic mass-based on the number and consistency of lesions and refined by supplementary imaging features-allows formulation of a useful differential diagnosis. Solitary cystic masses include true cysts, pseudocysts, and parasitic cysts. When multiple cystic lesions are present, the differential diagnosis expands to include infectious lesions (abscess or microabscesses) and lymphangioma (a benign cystic neoplasm). Hemangioma is the most common solitary solid mass, although other vascular lesions (hamartoma, sclerosing angiomatoid nodular transformation) and nonvascular lesions (inflammatory pseudotumor, lymphoma) manifest as solitary and solid. When multiple solid masses are present, diffuse inflammatory disease (sarcoidosis), littoral cell angioma, and lymphoma should be considered. Malignancies, such as angiosarcoma or metastasis, can manifest as solitary or multiple and solid or cystic masses but are typically associated with symptoms or widespread primary malignancy. Careful assessment of the multimodality imaging characteristics of splenic lesions based on this approach aids the radiologist faced with the incidental splenic lesion. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.
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Cistos , Linfoma , Esplenopatias , Neoplasias Esplênicas , Abscesso , Cistos/diagnóstico por imagem , Diagnóstico Diferencial , Humanos , Imagem Multimodal , Esplenopatias/diagnóstico por imagem , Esplenopatias/patologia , Neoplasias Esplênicas/diagnóstico por imagem , Neoplasias Esplênicas/patologiaRESUMO
CONTEXT.: Intravascular large B-cell lymphoma (IVLBCL) is a rare hematopathologic entity, posing both a clinical and histologic challenge for diagnosis. Numerous pitfalls can hinder making the diagnosis. OBJECTIVE.: To summarize recent developments in literature pertaining to IVLBCL and point out key pitfalls pathologists should be prepared to encounter. DATA SOURCES.: Literature review via PubMed search and hospital (Darnall Medical Library) resources. CONCLUSIONS.: The 3 primary pitfalls of IVLBCL include masking of IVLBCL, mimicry by IVLBCL, and mimicry of IVLBCL. These scenarios illustrate the importance of histologic pattern recognition and subsequent usage of immunohistochemistry, especially in context of a clinical history that may be noncharacteristic.
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Linfoma Difuso de Grandes Células B , Humanos , Imuno-Histoquímica , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/patologiaRESUMO
CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.
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Leucemia Linfocítica Crônica de Células B , Transtornos Linfoproliferativos , Linfócitos B/patologia , Humanos , Imunofenotipagem , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/patologia , Baço/patologiaRESUMO
Lymphoproliferative processes which occur in the gastrointestinal tract range from benign reactive processes such as follicular hyperplasia (rectal tonsil) to high grade malignant lymphomas and histiocytic sarcoma. The WHO Classification of Tumors: Digestive System Tumors, 5th Edition was published in 2019 and shows several impactful changes as compared to the 4th Edition published in 2010. WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues 2017 also included detailed changes in hematopoietic neoplasms within the gastrointestinal tract. New entities or renamed hematolymphoid lesions include monomorphic epitheliotropic intestinal T-cell lymphoma, duodenal-type follicular lymphoma, intestinal T-cell lymphoma, NOS and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. A brief overview of WHO classification of digestive tumors and WHO classification of tumors of hematopoietic and lymphoid tissue is discussed focusing on the changes in the most recent WHO texts. In depth discussions will be presented in other papers in this series.
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Gastroenteropatias , Neoplasias Gastrointestinais , Linfoma de Células B , Transtornos Linfoproliferativos , HumanosRESUMO
CONTEXT.: The World Health Organization Classification of Tumours: Digestive System Tumors, 5th edition, was published in 2019 and shows several impactful changes as compared with the 4th edition published in 2010. Changes include a revised nomenclature of serrated lesions and revamping the classification of neuroendocrine neoplasms. Appendiceal goblet cell adenocarcinoma is heavily revised, and intrahepatic cholangiocarcinoma is split into 2 subtypes. New subtypes of colorectal carcinoma and hepatocellular carcinoma are described. Precursor lesions are emphasized with their own entries, and both dysplastic and invasive lesions are generally recommended to be graded using a 2-tier system. Hematolymphoid tumors, mesenchymal tumors, and genetic tumor syndromes each have their own sections in the 5th edition. New hematolymphoid lesions include monomorphic epitheliotropic intestinal T-cell lymphoma; duodenal-type follicular lymphoma; intestinal T-cell lymphoma, not otherwise specified; and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. This paper will provide an in-depth look at the changes in the 5th edition as compared with the 4th edition. OBJECTIVE.: To provide a comprehensive, in-depth update on the World Health Organization classification of digestive tumors, including changes to nomenclature, updated diagnostic criteria, and newly described entities. DATA SOURCES.: The 5th edition of the World Health Organization Classification of Tumours: Digestive System Tumours, as well as the 4th edition. CONCLUSIONS.: The World Health Organization has made many key changes in its newest update on tumors of the digestive system. Pathologists should be aware of these changes and incorporate them into their practice as able or necessary.
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Neoplasias do Sistema Digestório/classificação , Neoplasias do Sistema Digestório/patologia , Biomarcadores Tumorais/metabolismo , Neoplasias do Sistema Digestório/diagnóstico , Neoplasias do Sistema Digestório/metabolismo , Humanos , Guias de Prática Clínica como Assunto , Organização Mundial da SaúdeRESUMO
T-cell lymphoproliferative processes in the spleen are rare and it is important to study normal T cell subsets in the spleen to understand the splenic milieu in which they arise. True malignant T-cell processes including hepatosplenic T-cell lymphoma and T-cell large granular lymphocytic leukemia occur in the spleen, but other atypical reactive T-cell proliferations and those of uncertain significance also have been described. Proper distinction of florid T cell responses from malignant T-cell neoplasms has important therapeutic implications for the patient.
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Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Baço/imunologia , Baço/patologia , Linfócitos T/imunologia , Linfócitos T/patologia , HumanosRESUMO
CONTEXT.: Even though immunohistochemistry is routinely used by pathologists, evaluation of immunohistochemistry in splenic lesions remains difficult for many. Classification of benign and splenic lesions often requires a combination of hematoxylin-eosin evaluation, immunophenotyping, and sometimes molecular testing. Immunohistochemical staining is essential in evaluating many splenic lesions, and requires an understanding of the normal compartments of the spleen. OBJECTIVE.: To address different immunohistochemical features used for identification and subclassification of different lesions of the spleen, as well as in the normal compartments of the spleen. DATA SOURCES.: The information outlined in this review article is based on our experiences with a variety of spleen cases, on the current World Health Organization classification of hematopoietic and lymphoid tumors, and on a review of English-language articles published during 2018. CONCLUSIONS.: Features for phenotyping normal spleen as well as a variety of splenic lesions, including littoral cell angioma and splenic marginal zone lymphoma, are discussed. Suggested immunopanels are provided to assist in the diagnosis of different lesions of the spleen.
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Imuno-Histoquímica/métodos , Imunofenotipagem/métodos , Baço/imunologia , Baço/metabolismo , Esplenopatias/diagnóstico , Antígenos de Superfície/análise , Diagnóstico Diferencial , Citometria de Fluxo , Hemangioma/classificação , Hemangioma/diagnóstico , Humanos , Linfoma/classificação , Linfoma/diagnóstico , Transtornos Linfoproliferativos/diagnóstico , Baço/patologia , Esplenopatias/classificação , Neoplasias Esplênicas/classificação , Neoplasias Esplênicas/diagnósticoAssuntos
Coristoma/patologia , Hamartoma/patologia , Doenças Linfáticas/patologia , Humanos , Linfonodos , BaçoRESUMO
Castleman disease is a complex lymphoproliferative disease pathologically divided into two subtypes, the hyaline vascular variant (HVV) and the plasma cell variant (PCV). The HVV is the most common, is thought to represent a benign neoplasm of lymph node stromal cells, and is treated with surgical resection. It is most commonly found in the mediastinum, where it classically appears as a unicentric, avidly enhancing mass at computed tomography (CT) and magnetic resonance imaging. This appearance can mimic other avidly enhancing mediastinal masses, and location, clinical history, laboratory data, and nuclear medicine single photon emission CT (SPECT) and positron emission tomography (PET) studies can help narrow the differential diagnosis. Multicentric Castleman disease (MCD), which in the majority of cases is composed of the PCV, is an aggressive lymphoproliferative disorder associated with human herpesvirus infection, interleukin 6 dysregulation, and other systemic disorders. While it can be difficult to differentiate MCD from lymphoma, the presence of avidly enhancing lymph nodes can suggest the diagnosis. The purpose of this article is to review the clinical, immunologic, and pathologic findings associated with both unicentric Castleman disease and MCD and discuss how the imaging findings correlate with the pathophysiology of the disease.
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Hiperplasia do Linfonodo Gigante/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/patologia , Diagnóstico por Imagem , Diagnóstico Diferencial , HumanosRESUMO
An overview of the pathology of extranodal lymphoma is presented. The emphasis of this presentation is on the classification system of extranodal lymphomas, including both B-cell and T-cell lymphomas, based on their morphology, phenotype, and molecular alterations.
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Biomarcadores Tumorais/análise , Linfoma de Células B/química , Linfoma de Células B/patologia , Linfoma de Células T/química , Linfoma de Células T/patologia , Linfócitos B/química , Linfócitos B/patologia , Diagnóstico Diferencial , Medicina Baseada em Evidências , Humanos , Linfócitos T/química , Linfócitos T/patologiaRESUMO
This review examines handling and processing of spleen biopsies and splenectomy specimens with the aim of providing the pathologist with guidance in optimizing examination and diagnosis of splenic disorders. It also offers recommendations as to relevant reporting factors in gross examination, which may guide diagnostic workup. The role of splenic needle biopsies is discussed. The International Spleen Consortium is a group dedicated to promoting education and research on the anatomy, physiology, and pathology of the spleen. In keeping with these goals, we have undertaken to provide guidelines for gross examination, sectioning, and sampling of spleen tissue to optimize diagnosis (Burke). The pathology of the spleen may be complicated in routine practice due to a number of factors. Among these are lack of familiarity with lesions, complex histopathology, mimicry within several types of lesions, and overall rarity. To optimize diagnosis, appropriate handling and processing of splenic tissue are crucial. The importance of complete and accurate clinical history cannot be overstated. In many cases, significant clinical history such as previous lymphoproliferative disorders, hematologic disorders, trauma, etc, can provide important information to guide the evaluation of spleen specimens. Clinical information helps plan for appropriate processing of the spleen specimen. The pathologist should encourage surgical colleagues, who typically provide the specimens, to include as much clinical information as possible.
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Biópsia/métodos , Manejo de Espécimes/métodos , Baço/patologia , Baço/cirurgia , Esplenectomia/métodos , Biópsia por Agulha Fina/métodos , Biópsia por Agulha Fina/normas , Biópsia com Agulha de Grande Calibre/métodos , Biópsia com Agulha de Grande Calibre/normas , Guias como Assunto , Humanos , Manejo de Espécimes/normas , Esplenectomia/normasRESUMO
Epstein Barr virus (EBV)-related lymphoproliferative processes occur in the head and neck ranging from reactive processes such as infectious mononucleosis to high grade malignant lymphomas. EBV is a ubiquitous herpes virus that infects more than 90% of adults worldwide, and is generally transferred though saliva. Primary infection can occur throughout life. EBV is the first virus linked to malignancies, both epithelial and lymphoid. Both T and B cell lymphomas can be associated with EBV and evidence shows that an individual's response to the acute EBV infection may be critical in the development of subsequent lymphoma. Currently, in situ hybridization for EBER is the most sensitive available test to detect EBV and should be routinely performed in lymphoproliferative lesions of the head and neck. Immunohistochemistry for EBV related proteins, such as LMP1, is much less sensitive than EBER in situ hybridization, but can help determine latency patterns of EBV infection. Although relatively rare, primary EBV-related lymphomas must be considered in the differential of atypical lymphoid proliferations in the head and neck. We present selected EBV-related disorders of the head and neck discussing etiology as well as differential diagnosis.
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Infecções por Vírus Epstein-Barr/patologia , Neoplasias de Cabeça e Pescoço/patologia , Herpesvirus Humano 4/isolamento & purificação , Tecido Linfoide/patologia , Linfoma/patologia , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/virologia , Neoplasias de Cabeça e Pescoço/imunologia , Neoplasias de Cabeça e Pescoço/virologia , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/imunologia , Herpesvirus Humano 4/patogenicidade , Humanos , Imuno-Histoquímica , Hibridização In Situ , Tecido Linfoide/imunologia , Tecido Linfoide/virologia , Linfoma/imunologia , Linfoma/virologia , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Proteínas Virais/genética , Proteínas Virais/isolamento & purificaçãoRESUMO
CONTEXT: Diffuse large B-cell lymphoma is the most commonly diagnosed subtype of lymphoma worldwide. The current World Health Organization (WHO) classification includes several subtypes, based on a combination of clinical, immunohistochemical, and genetic differences. Immunohistochemical staining is essential in evaluating diffuse large B-cell lymphoma and many related large B-cell lymphomas and aggressive B-cell lymphomas. OBJECTIVE: To address different immunohistochemical features used for identification, subclassification, prognosis and in some cases, therapy, of diffuse large B-cell lymphoma and related lymphomas. DATA SOURCES: The information outlined in this review article is based on our experiences with routine cases, on the current WHO classification of hematopoietic and lymphoid tumors, and on a review of English-language articles published throughout 2014. CONCLUSIONS: Features and diagnostic criteria of diffuse large B-cell lymphoma, aggressive variants of B-cell lymphomas, including Burkitt lymphoma and "double-hit" lymphomas, are discussed. Identification of cell of origin (germinal center type versus activated B-cell type) is discussed at length. Finally, practical approaches for diagnosis are discussed.
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Imuno-Histoquímica/métodos , Linfoma de Células B/imunologia , Linfoma Difuso de Grandes Células B/imunologia , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/imunologia , Biomarcadores Tumorais/metabolismo , Diagnóstico Diferencial , Humanos , Linfoma de Células B/diagnóstico , Linfoma de Células B/terapia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/terapia , PrognósticoRESUMO
AIMS: Studies have indicated that the t(14;18)(q32;q21)/IGH-MALT1 translocation is present in extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT lymphoma). However, only a few studies have investigated the incidence of t(14;18)/IGH-MALT1 in primary gastrointestinal MALT lymphomas or in diffuse large B-cell lymphomas (DLBCL). The overall significance of t(14;18)/IGH-MALT1 in gastrointestinal MALT lymphomas is not clear. We examined 41 gastrointestinal MALT lymphoma and 23 DLBCL cases, with the aim of further understanding the role of t(14;18)/IGH-MALT1 in these diseases. METHODS AND RESULTS: Fluorescence in-situ hybridization (FISH) assays for the detection of t(14;18)/IGH-MALT1 and t(11;18)(q21;q21)/API2-MALT1, along with immunostaining and histological evaluations, were performed on selected cases. Of the 64 analysed cases, one gastric MALT lymphoma and one colonic MALT lymphoma were positive for t(14;18)/IGH-MALT1. CONCLUSIONS: We describe what are, to our knowledge, the first reported primary colonic MALT lymphoma carrying t(14;18)(q32;q21)/IGH-MALT1, and one of the few reported cases of gastric MALT lymphoma with this translocation. As this translocation is seen in only a few gastrointestinal MALT lymphomas, it is not useful as a diagnostic marker for routine clinical services. Although these findings suggest that t(14;18)/IGH-MALT1 is a rare molecular event in gastrointestinal MALT lymphomas and DLBCLs, further studies to elucidate the role of this genetic alteration in these diseases are indicated.
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Cromossomos Humanos Par 14 , Cromossomos Humanos Par 18 , Linfoma de Zona Marginal Tipo Células B/genética , Proteínas de Fusão Oncogênica/genética , Translocação Genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
AIMS: Autoimmune diseases (AD) are associated with lymphadenopathy and splenomegaly. Changes in the spleen have not been characterized completely in AD; we describe splenectomy specimens from five patients with chronic AD, highlighting the presence of necrotizing histiocytosis. METHODS AND RESULTS: Of the patients (three males and two females; mean 40 years), four had systemic lupus erythematosus; one had rheumatoid arthritis. All had moderate splenomegaly (213-803 g, mean 421 g). Four cases exhibited necrosis with apoptosis and karyorrhectic debris occurring in the white pulp and minimal acute inflammation; one showed florid follicular hyperplasia. Splenic involvement ranged from focal to extensive. Plasma cells were negative for IgG4. Haematoxylin bodies were not identified. Stains for infectious organisms were negative. Immunohistochemical studies showed that lymphocytes surrounding the necrosis were a mixture of CD4(+) and CD8(+) T cells; CD123-positive plasmacytoid dendritic cells were not present, and staining for kappa and lambda light chains showed no clonality. 16S rDNA PCR was performed; no amplification was seen in three of four cases tested for bacteria specific rDNA. Epstein-Barr virus-encoded RNA (EBER) in situ hybridization studies highlighted rare positive cells in four cases. CONCLUSIONS: Splenomegaly in AD is thought to be hyperplasic, but we present four cases showing histiocytic necrosis, a finding which should be considered part of the spectrum of AD in the spleen.
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Artrite Reumatoide/patologia , Linfadenite Histiocítica Necrosante/patologia , Lúpus Eritematoso Sistêmico/patologia , Adulto , Artrite Reumatoide/imunologia , Artrite Reumatoide/metabolismo , Linfócitos T CD4-Positivos/imunologia , Linfócitos T CD8-Positivos/imunologia , Doença Crônica , Células Dendríticas/metabolismo , Células Dendríticas/patologia , Feminino , Herpesvirus Humano 4/genética , Linfadenite Histiocítica Necrosante/imunologia , Linfadenite Histiocítica Necrosante/metabolismo , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/metabolismo , Masculino , Pessoa de Meia-Idade , Baço/metabolismo , Baço/patologiaRESUMO
Hemangiopericytomas and solitary fibrous tumors are uncommon neoplasms found in many locations, including the orbit. Both mesenchymal neoplasms share several clinicopathologic features, thus prompting intense debate as to whether they are variants of the same entity or merit separate designations in the orbit. These 2 entities, with the addition of giant cell angiofibroma of orbit, are of benign- to uncertain-behavior, CD34-positive, collagen-rich, specialized fibroblastic tumors, which may have overlapping or histologically identical features. In addition, so-called fibrous histiocytoma of orbit, a previous designation, has overlapping morphologic features with these tumors. To date, a large series of these collagen-rich fibroblastic tumors of the orbit has not been fully explored. Forty-one fibroblastic orbital tumors, originally diagnosed as hemangiopericytomas (n = 16), fibrous histiocytomas (n = 9), mixed tumors (hemangiopericytomas/fibrous histiocytoma) (n = 14), and giant cell angiofibromas of orbit (n = 2) between 1970 and 2009, were retrieved from our consultation files, the Ophthalmic Registry, at the Armed Forces Institute of Pathology. Slides and clinical records were reviewed, analyzed, and compared. Immunochemistry was performed for CD34, CD99, Bcl-2, Ki-67, and p53. Upon histologic review, all cases were reclassified as solitary fibrous tumor (41/41). The patients included 23 (56%) males, 17 (41%) females, and 1 unknown, with a mean age at presentation of 40.7 years (range, 16-70 years). The sites of involvement were the right orbit in 18 (44%) cases and the left in 16 (39%) cases. Tumors ranged in size from 0.4 to 5.0 cm (mean, 2.2 cm). Seventeen (41%) patients presented with an orbital mass, 8 (20%) with proptosis, 2 (5%) with painful mass, and 2 (5%) with painless mass. Duration of symptoms ranged from 3 to 96 months, with a mean of 23 months (median, 9 months). Microscopically, all lesions showed considerable similarity, varying in degree of cellularity, stromal collagen, and the presence of giant cells. Overlapping features with soft tissue giant cell fibroblastoma were observed. Immunochemistry revealed positivity for CD34 in all cases (100%), p53 in 85%, CD99 in 67.5%, and Bcl-2 in 47.5%. Although Ki-67 labeling was seen in all cases, it ranged from less than 1% in 54.3% of cases to 5% to 10% in 20% of cases. Taken together, the findings of this study suggest that orbital hemangiopericytoma and some cases previously designated as fibrous histiocytoma, giant cell angiofibroma of orbit, and solitary fibrous tumor have overlapping morphologic and immunohistochemical features and should be designated as solitary fibrous tumor. Adipocytes and unusual multivacuolated adipocytic cells may be present in these tumors, as well stromal myxoid change; and even stromal intramembranous ossification can be observed. There are overlapping features of orbital solitary fibrous tumor with another CD34-positive specialized fibroblastic tumor of soft tissue, giant cell fibroblastoma. Morphologic criteria for uncertain behavior to low-grade malignant ocular solitary fibrous tumors can be made by cytologic atypia and increased mitotic activity, but overall outcome for malignant solitary fibrous tumors of the eye should be further explored.