Dendritic cell tumors can arise from Langerhans cells or other dendritic cell subtypes. Langerhans cell histiocytosis (LCH) is characterized by proliferation of Langerhans cells and commonly involves bone, skin, lungs and lymph nodes. It ranges from localized eosinophilic granuloma to disseminated disease. Langerhans cell sarcoma is a rare malignant neoplasm that retains the immunophenotype of Langerhans cells. Indeterminate dendritic cell tumors have features intermediate between LCH and dendritic cell sarcoma.
Lab Diagnosis of Chronic lymphoproliferative disorders (CLPD);Flowcytometric...Dr Siddartha
Lab Diagnosis of Chronic lymphoproliferative disorders (CLPD);Flowcytometric Evaluation
Basavatarakam Indo-American Cancer Hospital and Research Institute
Small round cell tumors are a group of highly aggressive cancers composed of small, undifferentiated cells. The diagnostic approach involves clinical findings, imaging, pathology, and molecular genetics testing. Key small round cell tumors in pediatric patients include Ewing sarcoma, neuroblastoma, nephroblastoma, rhabdomyosarcoma, medulloblastoma, retinoblastoma, and lymphoblastic lymphoma. Immunohistochemistry and genetic testing are used to determine the specific tumor type to help guide treatment.
This slide presentation summarizes the cytology findings of a liver biopsy from a 60-year-old male. Giemsa stained smears showed moderately cellular tumor cells arranged in clusters and fragments with ill-defined edges. The tumor cells had round to oval nuclei and granular cytoplasm. Background showed lymphocytes and hemorrhage. A diagnosis of metastatic tumor was made, with the top differential diagnoses being metastatic papillary urothelial carcinoma, neuroendocrine tumor, solid pseudo-papillary neoplasm of the pancreas, or epithelioid gastrointestinal stromal tumor. Additional slides showed examples of various liver lesions and metastases that can present on cytology to aid in diagnosis.
Tensins are proteins located at focal adhesions that link integrins to the actin cytoskeleton. There are four tensin family members that play important roles in cell adhesion, migration, proliferation and survival. Tensins help maintain tissue integrity but can also contribute to disease when their expression is altered, as seen in various cancers where different tensins may act as either tumor suppressors or oncogenes depending on the context. The functions and regulation of individual tensins can vary between tissues and disease states.
Myelodysplastic syndrome according to WHO 2016Madhuri Reddy
The document defines myelodysplastic syndromes as a group of clonal stem cell diseases characterized by cytopenia, dysplasia in one or more myeloid lineages, ineffective hematopoiesis, recurrent genetic abnormalities, and an increased risk of developing acute myeloid leukemia. It discusses the epidemiology, etiology, pathophysiology, cytogenetics, morphological features, clinical features, WHO classification, differential diagnosis, variants, immunophenotyping, management, and prognosis of MDS. The document provides details on the definition, evaluation, classification, genetic abnormalities, and clinical manifestations of myelodysplastic syndromes.
Myofibroblasts are cells that have characteristics of both fibroblasts and smooth muscle cells. They play an important role in wound healing by synthesizing the extracellular matrix and generating contraction forces. Myofibroblasts can originate from fibroblasts, smooth muscle cells, or stem cells. They express markers like alpha-smooth muscle actin, vimentin, and desmin. Myofibroblasts are involved in tissue remodeling but can also contribute to pathological fibrosis and scarring when their activity remains unchecked. They are involved in conditions like pulmonary fibrosis, liver fibrosis, atherosclerosis, and tumor formation. Their differentiation is regulated by growth factors and cytokines.
This document discusses soft tissue tumors. It defines soft tissue and describes its composition. It notes that soft tissue tumors can be caused by radiation, burns, trauma, viruses, or genetic syndromes. The document outlines various tumor types including liposarcomas, fibrosarcomas, and malignant fibrous histiocytomas. It describes histologic patterns seen in different tumors and discusses grading systems. Pseudosarcomas like nodular fasciitis are also summarized.
Lab Diagnosis of Chronic lymphoproliferative disorders (CLPD);Flowcytometric...Dr Siddartha
Lab Diagnosis of Chronic lymphoproliferative disorders (CLPD);Flowcytometric Evaluation
Basavatarakam Indo-American Cancer Hospital and Research Institute
Small round cell tumors are a group of highly aggressive cancers composed of small, undifferentiated cells. The diagnostic approach involves clinical findings, imaging, pathology, and molecular genetics testing. Key small round cell tumors in pediatric patients include Ewing sarcoma, neuroblastoma, nephroblastoma, rhabdomyosarcoma, medulloblastoma, retinoblastoma, and lymphoblastic lymphoma. Immunohistochemistry and genetic testing are used to determine the specific tumor type to help guide treatment.
This slide presentation summarizes the cytology findings of a liver biopsy from a 60-year-old male. Giemsa stained smears showed moderately cellular tumor cells arranged in clusters and fragments with ill-defined edges. The tumor cells had round to oval nuclei and granular cytoplasm. Background showed lymphocytes and hemorrhage. A diagnosis of metastatic tumor was made, with the top differential diagnoses being metastatic papillary urothelial carcinoma, neuroendocrine tumor, solid pseudo-papillary neoplasm of the pancreas, or epithelioid gastrointestinal stromal tumor. Additional slides showed examples of various liver lesions and metastases that can present on cytology to aid in diagnosis.
Tensins are proteins located at focal adhesions that link integrins to the actin cytoskeleton. There are four tensin family members that play important roles in cell adhesion, migration, proliferation and survival. Tensins help maintain tissue integrity but can also contribute to disease when their expression is altered, as seen in various cancers where different tensins may act as either tumor suppressors or oncogenes depending on the context. The functions and regulation of individual tensins can vary between tissues and disease states.
Myelodysplastic syndrome according to WHO 2016Madhuri Reddy
The document defines myelodysplastic syndromes as a group of clonal stem cell diseases characterized by cytopenia, dysplasia in one or more myeloid lineages, ineffective hematopoiesis, recurrent genetic abnormalities, and an increased risk of developing acute myeloid leukemia. It discusses the epidemiology, etiology, pathophysiology, cytogenetics, morphological features, clinical features, WHO classification, differential diagnosis, variants, immunophenotyping, management, and prognosis of MDS. The document provides details on the definition, evaluation, classification, genetic abnormalities, and clinical manifestations of myelodysplastic syndromes.
Myofibroblasts are cells that have characteristics of both fibroblasts and smooth muscle cells. They play an important role in wound healing by synthesizing the extracellular matrix and generating contraction forces. Myofibroblasts can originate from fibroblasts, smooth muscle cells, or stem cells. They express markers like alpha-smooth muscle actin, vimentin, and desmin. Myofibroblasts are involved in tissue remodeling but can also contribute to pathological fibrosis and scarring when their activity remains unchecked. They are involved in conditions like pulmonary fibrosis, liver fibrosis, atherosclerosis, and tumor formation. Their differentiation is regulated by growth factors and cytokines.
This document discusses soft tissue tumors. It defines soft tissue and describes its composition. It notes that soft tissue tumors can be caused by radiation, burns, trauma, viruses, or genetic syndromes. The document outlines various tumor types including liposarcomas, fibrosarcomas, and malignant fibrous histiocytomas. It describes histologic patterns seen in different tumors and discusses grading systems. Pseudosarcomas like nodular fasciitis are also summarized.
The document summarizes the International Academy of Cytology (IAC) System for classifying breast malignancy based on fine-needle aspiration cytology (FNAC) results. The system was developed at a meeting in Yokohama in 2016. It aims to standardize breast cytology reporting to improve diagnosis and patient management. The system categorizes FNAC results as insufficient, benign, atypical, suspicious of malignancy, or malignant, with associated risks of malignancy. Cytological features and management recommendations are provided for each category. The goal is to link cytology reports to optimal breast care.
Chronic myeloid leukemia (CML) is a cancer of the white blood cells characterized by the Philadelphia chromosome, which is present in over 80% of CML cases. CML progresses through three phases: chronic phase, accelerated phase, and blast phase. The chronic phase is typically when CML is diagnosed and treated, with tyrosine kinase inhibitors like imatinib being the standard first-line treatment. Without treatment, CML progresses to more advanced phases associated with worse outcomes.
Mast cells (MCs) play a broad role in both physiology and disease beyond just allergy. MCs originate from bone marrow progenitor cells and develop in tissues where they exist in different phenotypes. MCs can be activated through various stimuli to degranulate and release mediators that impact wound healing, homeostasis, the nervous system, host defense against parasites, bacteria, viruses, and venoms, as well as diseases like allergy, asthma, vascular disease, and fibrosis. MCs contribute to inflammation in conditions such as inflammatory bowel disease and some autoimmune/autoinflammatory diseases.
Chronic granulomatous disease is a rare inherited disorder characterized by defects in the NADPH oxidase system, which leads to recurrent infections. It is caused by mutations affecting components of the NADPH oxidase enzyme complex, resulting in the inability of phagocytes to produce reactive oxygen species to kill certain bacteria and fungi. Patients present with recurrent infections of the lungs, skin, lymph nodes, liver or bones by catalase-positive organisms. Treatment involves lifelong antibiotic prophylaxis, with hematopoietic stem cell transplantation or gene therapy as curative options.
This document provides an approach for evaluating undifferentiated tumors. It begins by categorizing undifferentiated tumors into 4 groups based on morphology: small round cell tumors, epithelioid cell tumors, spindle cell tumors, and pleomorphic tumors. It then outlines the diagnostic algorithm which involves determining the main lineage (epithelial, melanocytic, hematopoietic/lymphoid, or mesenchymal), specifying a diagnosis using immunohistochemistry and clinical correlation, and considering the differential diagnoses for each category. A variety of immunohistochemical markers are also described that can help identify the cell or tumor type.
This document provides information on chronic myelogenous leukemia (CML), including its definition, history, epidemiology, etiology, pathogenesis, clinical features, diagnosis, disease course, treatment, and more. Some key points:
- CML is a stem cell disease characterized by increased white blood cells, anemia, splenomegaly, and the Philadelphia chromosome.
- It has three phases: chronic, accelerated, and blast crisis. Treatment depends on the phase and may include tyrosine kinase inhibitors like imatinib, interferon, chemotherapy, and stem cell transplantation.
- The disease is caused by the BCR-ABL fusion gene which results in uncontrolled tyrosine kinase activity and increased proliferation of
This document discusses various non-neoplastic and neoplastic conditions that can cause lymphadenopathy. It focuses on filariasis as a cause of non-neoplastic lymphadenopathy. Filarial parasites can infect the lymphatics and lymph nodes, causing inflammation and blockage. On pathology, the lymph nodes show an intense inflammatory reaction around dead or dying larvae with eosinophils and multinucleated giant cells. Rarely, microfilaria can be seen embedded in the lymph node tissue. The document emphasizes that a diligent search is needed to identify the parasite and make an accurate diagnosis.
Lymph nodes are bean-shaped organs found throughout the body that filter lymph and house immune cells. A lymph node contains a fibrous capsule enclosing compartments of connective tissue and lymphocytes. The parenchyma is divided into an outer cortex and inner medulla. A normal lymph node contains mature lymphocytes, plasma cells, centrocytes, centroblasts, and immunoblasts. Lymphadenopathy refers to enlarged lymph nodes, which can be caused by infection, inflammation, autoimmune disease, or cancer metastasis. Physical examination of lymph nodes considers location, number, size, consistency, tenderness, and mobility to evaluate causes of lymphadenopathy.
Salivary glands produce saliva and are composed of major and minor glands. The major glands are the parotid, submandibular, and sublingual glands. Salivary gland cytology can detect both benign and malignant lesions. Common benign findings include pleomorphic adenoma, Warthin's tumor, and basal cell adenoma cells. Malignant lesions include adenoid cystic carcinoma, mucoepidermoid carcinoma, and adenocarcinoma. Fine needle aspiration cytology allows diagnosis of salivary gland lesions with high sensitivity and specificity to guide treatment.
Giant cell lesions of bone include both reactive and neoplastic conditions characterized by the presence of multinucleated giant cells. Reactive giant cell lesions include giant cell reparative granuloma and brown tumor of hyperparathyroidism. Benign neoplastic giant cell lesions include giant cell tumor and aneurysmal bone cyst. Giant cell tumor is the most common, occurring most frequently in long bones of the extremities in young and middle aged adults. Histologically it is characterized by uniformly distributed osteoclast-like giant cells and mononuclear stromal cells that express RANKL.
Reporting thyroid fine needle aspiration by the bethesda systemMonika Nema
This document summarizes guidelines for thyroid fine needle aspiration (FNA) cytopathology reports. It discusses classifications including nondiagnostic/unsatisfactory, benign thyroid lesions, atypia of undetermined significance/follicular lesion of undetermined significance, and includes examples of diagnostic criteria, imaging features and recommended reporting language for each classification. Thyroid FNA is presented as an accurate and cost-effective initial test for evaluating thyroid nodules that can help determine if surgery is needed.
This document provides information on endoscopic gastrointestinal biopsies and their interpretation. It discusses endoscopy techniques and tools used to visualize the gastrointestinal tract and obtain biopsies. Key points include types of endoscopes, handling of biopsy specimens, processing for histological examination, common indications for endoscopy of the upper gastrointestinal tract, and histological findings and interpretations for conditions of the esophagus and stomach, including chronic gastritis, Helicobacter pylori infection, Barrett's esophagus, and polypoid lesions.
This document discusses the role of immunohistochemistry (IHC) in diagnosing soft tissue tumours. It begins by defining soft tissue and the WHO classification of soft tissue tumours. IHC is an important ancillary technique that can be used to identify discrete tissue components using antigen-antibody binding. The document outlines the IHC protocol and discusses various markers that can help diagnose different types of soft tissue tumours, including markers for fibroblastic, adipocytic, vascular, neural, osseous and cartilaginous tumours. Specific markers and the tumours they are useful for identifying are provided. The document emphasizes that IHC should be used along with other techniques as markers sometimes show cross-reactivity.
This document summarizes key information about major and minor salivary glands including their location and cell types. It describes common benign and malignant epithelial tumors of the salivary glands such as pleomorphic adenoma, Warthin's tumor, oncocytoma, and mucoepidermoid carcinoma. For each tumor, the clinical features, microscopic appearance, differential diagnosis, and important histological characteristics are outlined. The document provides an overview of salivary gland anatomy, histology, and the spectrum of tumors that can arise in these glands.
Immunohistochemistry in diagnosis of soft tissue tumours seminarPannaga Kumar
This document discusses immunohistochemistry in the diagnosis of soft tissue tumors. It begins by introducing soft tissue and the classification of soft tissue tumors. It then discusses various ancillary techniques used, focusing on immunohistochemistry. It provides details on common markers used to identify muscle, neural, melanocytic, endothelial and other types of differentiation. It discusses the applications and diagnostic utility of various markers for different tumor types. In summary, the document is a comprehensive overview of immunohistochemistry techniques and markers useful in the diagnosis and classification of soft tissue tumors.
WHO BRAIN TUMOR CLASSIFICATION 5th EDITIONKanhu Charan
The document summarizes some of the key changes in the 2021 5th edition of the WHO brain tumor classification compared to previous editions. Some notable changes include recognizing the distinction between adult and pediatric diffuse gliomas, adding 22 new tumor types, revising the terminology for 13 tumor types, introducing essential and desirable diagnostic criteria, and classifying tumors based on a combination of histopathological and molecular features. Sellar tumors, meningiomas, and ependymomas were also revised in the new classification system.
The document discusses cytology of various bone lesions. It covers classification of bone tumors and describes cytological features of inflammatory conditions like osteomyelitis. It also discusses osteoid forming lesions such as fracture callus and osteoblastoma. Cartilage forming tumors described include chondroma, chondromyxoid fibroma and osteochondroma. Giant cell containing lesions and cystic bone lesions are also mentioned. The document provides cytological details of various bone tumors like osteosarcoma, chondrosarcoma and chondroblastoma through multiple case studies. It highlights differential diagnoses and ancillary techniques used in evaluation of bone lesions.
This document provides an overview of X-linked agammaglobulinemia (XLA). Some key points:
- XLA is a rare primary immunodeficiency caused by mutations in the BTK gene, which is important for B cell development and antibody production.
- It was first described in 1952 and only affects males. Clinical manifestations include recurrent bacterial infections from a young age due to lack of antibodies.
- Diagnosis involves detecting very low or absent antibody levels and identifying a mutation in the BTK gene in affected males. Treatment involves lifelong monthly immunoglobulin replacement therapy.
The document discusses the role of immunohistochemistry (IHC) in head and neck pathology. IHC uses antibodies to identify antigens in tissues and can help diagnose cancers and determine tumor type. It plays an important role in pathology subspecialties like oncology, neuropathology, and hematopathology. The document provides details on the principle of IHC, various tissue and tumor markers used in IHC, and how IHC can help diagnose lesions in the head and neck region like oral cancers, salivary gland tumors, melanomas, sarcomas, and lymphomas.
Hemophagocytic lymphohistiocytosis (hlh), Langerhans cell histiocytosis dr vi...Vijitha A S
Hemophagocytic lymphohistiocytosis (hlh)
Langerhans cell histiocytosis,Benign proliferation of mature histiocytes and uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes, and their hematopoietic precursors in the bonemarrow & other tissues
Management of acute lymphoblatic leukemia with light on etiology, clinical features, diagnosis and different aspects of management including chemotherapy and radiation therapy
The document summarizes the International Academy of Cytology (IAC) System for classifying breast malignancy based on fine-needle aspiration cytology (FNAC) results. The system was developed at a meeting in Yokohama in 2016. It aims to standardize breast cytology reporting to improve diagnosis and patient management. The system categorizes FNAC results as insufficient, benign, atypical, suspicious of malignancy, or malignant, with associated risks of malignancy. Cytological features and management recommendations are provided for each category. The goal is to link cytology reports to optimal breast care.
Chronic myeloid leukemia (CML) is a cancer of the white blood cells characterized by the Philadelphia chromosome, which is present in over 80% of CML cases. CML progresses through three phases: chronic phase, accelerated phase, and blast phase. The chronic phase is typically when CML is diagnosed and treated, with tyrosine kinase inhibitors like imatinib being the standard first-line treatment. Without treatment, CML progresses to more advanced phases associated with worse outcomes.
Mast cells (MCs) play a broad role in both physiology and disease beyond just allergy. MCs originate from bone marrow progenitor cells and develop in tissues where they exist in different phenotypes. MCs can be activated through various stimuli to degranulate and release mediators that impact wound healing, homeostasis, the nervous system, host defense against parasites, bacteria, viruses, and venoms, as well as diseases like allergy, asthma, vascular disease, and fibrosis. MCs contribute to inflammation in conditions such as inflammatory bowel disease and some autoimmune/autoinflammatory diseases.
Chronic granulomatous disease is a rare inherited disorder characterized by defects in the NADPH oxidase system, which leads to recurrent infections. It is caused by mutations affecting components of the NADPH oxidase enzyme complex, resulting in the inability of phagocytes to produce reactive oxygen species to kill certain bacteria and fungi. Patients present with recurrent infections of the lungs, skin, lymph nodes, liver or bones by catalase-positive organisms. Treatment involves lifelong antibiotic prophylaxis, with hematopoietic stem cell transplantation or gene therapy as curative options.
This document provides an approach for evaluating undifferentiated tumors. It begins by categorizing undifferentiated tumors into 4 groups based on morphology: small round cell tumors, epithelioid cell tumors, spindle cell tumors, and pleomorphic tumors. It then outlines the diagnostic algorithm which involves determining the main lineage (epithelial, melanocytic, hematopoietic/lymphoid, or mesenchymal), specifying a diagnosis using immunohistochemistry and clinical correlation, and considering the differential diagnoses for each category. A variety of immunohistochemical markers are also described that can help identify the cell or tumor type.
This document provides information on chronic myelogenous leukemia (CML), including its definition, history, epidemiology, etiology, pathogenesis, clinical features, diagnosis, disease course, treatment, and more. Some key points:
- CML is a stem cell disease characterized by increased white blood cells, anemia, splenomegaly, and the Philadelphia chromosome.
- It has three phases: chronic, accelerated, and blast crisis. Treatment depends on the phase and may include tyrosine kinase inhibitors like imatinib, interferon, chemotherapy, and stem cell transplantation.
- The disease is caused by the BCR-ABL fusion gene which results in uncontrolled tyrosine kinase activity and increased proliferation of
This document discusses various non-neoplastic and neoplastic conditions that can cause lymphadenopathy. It focuses on filariasis as a cause of non-neoplastic lymphadenopathy. Filarial parasites can infect the lymphatics and lymph nodes, causing inflammation and blockage. On pathology, the lymph nodes show an intense inflammatory reaction around dead or dying larvae with eosinophils and multinucleated giant cells. Rarely, microfilaria can be seen embedded in the lymph node tissue. The document emphasizes that a diligent search is needed to identify the parasite and make an accurate diagnosis.
Lymph nodes are bean-shaped organs found throughout the body that filter lymph and house immune cells. A lymph node contains a fibrous capsule enclosing compartments of connective tissue and lymphocytes. The parenchyma is divided into an outer cortex and inner medulla. A normal lymph node contains mature lymphocytes, plasma cells, centrocytes, centroblasts, and immunoblasts. Lymphadenopathy refers to enlarged lymph nodes, which can be caused by infection, inflammation, autoimmune disease, or cancer metastasis. Physical examination of lymph nodes considers location, number, size, consistency, tenderness, and mobility to evaluate causes of lymphadenopathy.
Salivary glands produce saliva and are composed of major and minor glands. The major glands are the parotid, submandibular, and sublingual glands. Salivary gland cytology can detect both benign and malignant lesions. Common benign findings include pleomorphic adenoma, Warthin's tumor, and basal cell adenoma cells. Malignant lesions include adenoid cystic carcinoma, mucoepidermoid carcinoma, and adenocarcinoma. Fine needle aspiration cytology allows diagnosis of salivary gland lesions with high sensitivity and specificity to guide treatment.
Giant cell lesions of bone include both reactive and neoplastic conditions characterized by the presence of multinucleated giant cells. Reactive giant cell lesions include giant cell reparative granuloma and brown tumor of hyperparathyroidism. Benign neoplastic giant cell lesions include giant cell tumor and aneurysmal bone cyst. Giant cell tumor is the most common, occurring most frequently in long bones of the extremities in young and middle aged adults. Histologically it is characterized by uniformly distributed osteoclast-like giant cells and mononuclear stromal cells that express RANKL.
Reporting thyroid fine needle aspiration by the bethesda systemMonika Nema
This document summarizes guidelines for thyroid fine needle aspiration (FNA) cytopathology reports. It discusses classifications including nondiagnostic/unsatisfactory, benign thyroid lesions, atypia of undetermined significance/follicular lesion of undetermined significance, and includes examples of diagnostic criteria, imaging features and recommended reporting language for each classification. Thyroid FNA is presented as an accurate and cost-effective initial test for evaluating thyroid nodules that can help determine if surgery is needed.
This document provides information on endoscopic gastrointestinal biopsies and their interpretation. It discusses endoscopy techniques and tools used to visualize the gastrointestinal tract and obtain biopsies. Key points include types of endoscopes, handling of biopsy specimens, processing for histological examination, common indications for endoscopy of the upper gastrointestinal tract, and histological findings and interpretations for conditions of the esophagus and stomach, including chronic gastritis, Helicobacter pylori infection, Barrett's esophagus, and polypoid lesions.
This document discusses the role of immunohistochemistry (IHC) in diagnosing soft tissue tumours. It begins by defining soft tissue and the WHO classification of soft tissue tumours. IHC is an important ancillary technique that can be used to identify discrete tissue components using antigen-antibody binding. The document outlines the IHC protocol and discusses various markers that can help diagnose different types of soft tissue tumours, including markers for fibroblastic, adipocytic, vascular, neural, osseous and cartilaginous tumours. Specific markers and the tumours they are useful for identifying are provided. The document emphasizes that IHC should be used along with other techniques as markers sometimes show cross-reactivity.
This document summarizes key information about major and minor salivary glands including their location and cell types. It describes common benign and malignant epithelial tumors of the salivary glands such as pleomorphic adenoma, Warthin's tumor, oncocytoma, and mucoepidermoid carcinoma. For each tumor, the clinical features, microscopic appearance, differential diagnosis, and important histological characteristics are outlined. The document provides an overview of salivary gland anatomy, histology, and the spectrum of tumors that can arise in these glands.
Immunohistochemistry in diagnosis of soft tissue tumours seminarPannaga Kumar
This document discusses immunohistochemistry in the diagnosis of soft tissue tumors. It begins by introducing soft tissue and the classification of soft tissue tumors. It then discusses various ancillary techniques used, focusing on immunohistochemistry. It provides details on common markers used to identify muscle, neural, melanocytic, endothelial and other types of differentiation. It discusses the applications and diagnostic utility of various markers for different tumor types. In summary, the document is a comprehensive overview of immunohistochemistry techniques and markers useful in the diagnosis and classification of soft tissue tumors.
WHO BRAIN TUMOR CLASSIFICATION 5th EDITIONKanhu Charan
The document summarizes some of the key changes in the 2021 5th edition of the WHO brain tumor classification compared to previous editions. Some notable changes include recognizing the distinction between adult and pediatric diffuse gliomas, adding 22 new tumor types, revising the terminology for 13 tumor types, introducing essential and desirable diagnostic criteria, and classifying tumors based on a combination of histopathological and molecular features. Sellar tumors, meningiomas, and ependymomas were also revised in the new classification system.
The document discusses cytology of various bone lesions. It covers classification of bone tumors and describes cytological features of inflammatory conditions like osteomyelitis. It also discusses osteoid forming lesions such as fracture callus and osteoblastoma. Cartilage forming tumors described include chondroma, chondromyxoid fibroma and osteochondroma. Giant cell containing lesions and cystic bone lesions are also mentioned. The document provides cytological details of various bone tumors like osteosarcoma, chondrosarcoma and chondroblastoma through multiple case studies. It highlights differential diagnoses and ancillary techniques used in evaluation of bone lesions.
This document provides an overview of X-linked agammaglobulinemia (XLA). Some key points:
- XLA is a rare primary immunodeficiency caused by mutations in the BTK gene, which is important for B cell development and antibody production.
- It was first described in 1952 and only affects males. Clinical manifestations include recurrent bacterial infections from a young age due to lack of antibodies.
- Diagnosis involves detecting very low or absent antibody levels and identifying a mutation in the BTK gene in affected males. Treatment involves lifelong monthly immunoglobulin replacement therapy.
The document discusses the role of immunohistochemistry (IHC) in head and neck pathology. IHC uses antibodies to identify antigens in tissues and can help diagnose cancers and determine tumor type. It plays an important role in pathology subspecialties like oncology, neuropathology, and hematopathology. The document provides details on the principle of IHC, various tissue and tumor markers used in IHC, and how IHC can help diagnose lesions in the head and neck region like oral cancers, salivary gland tumors, melanomas, sarcomas, and lymphomas.
Hemophagocytic lymphohistiocytosis (hlh), Langerhans cell histiocytosis dr vi...Vijitha A S
Hemophagocytic lymphohistiocytosis (hlh)
Langerhans cell histiocytosis,Benign proliferation of mature histiocytes and uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes, and their hematopoietic precursors in the bonemarrow & other tissues
Management of acute lymphoblatic leukemia with light on etiology, clinical features, diagnosis and different aspects of management including chemotherapy and radiation therapy
Follicular lymphoma is the most common indolent non-Hodgkin lymphoma in the United States, arising from germinal center B cells. It is characterized by a translocation involving BCL2, leading to overexpression of the BCL2 protein and promotion of cell survival. While often following an indolent course, transformation to aggressive diffuse large B-cell lymphoma can occur in 30-50% of cases, with reduced survival.
Acute lymphoblastic leukemia (ALL) is a cancer of the lymphoid line of blood cells characterized by the proliferation of immature lymphocytes in the bone marrow. Diagnosis requires identifying at least 20% lymphoblasts in the bone marrow. Testing includes bone marrow biopsy and aspiration with immunophenotyping, cytogenetics, lumbar puncture and other studies. Proper classification is important for determining prognosis and selecting optimal treatment strategies.
FSGS is a pattern of scarring within the glomeruli that can be caused by various factors. It represents a major cause of end-stage renal disease. There are several variants of FSGS including classic, perihilar, cellular, collapsing, and tip variants. FSGS can be primary, secondary to things like adaptive responses, viruses, drugs, or genetic mutations. Treatment depends on the underlying cause but may include immunosuppressive therapies or managing underlying conditions. Distinguishing FSGS from minimal change disease can sometimes be challenging as they can appear similar or transition between each other.
This document discusses the approach to diagnosing and treating histiocytosis syndromes in childhood. It is a diverse group of disorders involving an abnormal proliferation of histiocytes (monocyte-macrophage cells). The main types discussed are Langerhans cell histiocytosis (LCH), hemophagocytic lymphohistiocytosis (HLH), and reactive histiocytosis. LCH is characterized by Langerhans cells with Birbeck granules, while HLH involves an uncontrolled activation of macrophages and T-cells. Diagnosis involves biopsy and immunochemistry. Treatment depends on risk stratification and number of organ systems involved, ranging from monitoring to chemotherapy. Long-term follow-up is important due to the disease
This document provides an overview of acute leukemias, including:
1. Leukemias are malignant disorders of haematopoietic stem cells characterized by increased white blood cells in bone marrow and blood. The course can vary from weeks to years depending on type.
2. Acute lymphoblastic leukemia (ALL) comprises 80% of childhood acute leukemias and 20% of adult cases. Acute myeloid leukemia (AML) typically presents at age 60.
3. Treatment involves supportive care, infection prevention, and chemotherapy to induce and maintain remission, while hematopoietic stem cell transplantation offers a potential cure.
This document provides an overview of leukemia, including its definition, types, causes, symptoms, diagnosis, treatment, and nursing management. It discusses the main types of leukemia - acute lymphocytic leukemia, acute myeloid leukemia, chronic lymphocytic leukemia, and chronic myeloid leukemia. For each type, it covers clinical manifestations, diagnostic evaluation, medical management options like chemotherapy and stem cell transplantation, and associated nursing care. The document also reviews the TNM staging system and mentions a research study on imatinib therapy for chronic myeloid leukemia.
Hodgkin's lymphoma, also known as Hodgkin's disease, is a cancer that originates in the lymphatic system. It is characterized by the presence of Reed-Sternberg cells in the lymph nodes and other tissues. The disease has four main subtypes - nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte rich - which are distinguished based on the type of cells in the tissue around the Reed-Sternberg cells. The nodular sclerosis subtype, which involves bands of fibrosis dividing the lymph node tissue into nodules, accounts for about 40-70% of cases. Hodgkin's lymphoma most commonly presents with painless swelling of lymph nodes in the neck,
This document provides an overview of leukemia including its anatomy and physiology, definition, pathophysiology, types, causes and risk factors, clinical manifestations, diagnostic findings, medical management, and nursing management. It discusses the four main types of leukemia - acute lymphocytic leukemia, acute myeloid leukemia, chronic myeloid leukemia, and chronic lymphocytic leukemia. The document also outlines the nursing diagnoses that may be associated with caring for patients with leukemia.
1. The document discusses several qualitative disorders of white blood cells including lazy leukocyte syndrome, Chediak-Higashi syndrome, infectious mononucleosis, leukemia, and lymphoma.
2. Key details are provided about the clinical features, oral manifestations, diagnosis, and management of each condition. Lazy leukocyte syndrome involves defects in neutrophil migration leading to recurrent infections. Chediak-Higashi syndrome is a genetic disorder characterized by enlarged granules and recurrent infections.
3. Infectious mononucleosis is caused by the Epstein-Barr virus and presents with fever, pharyngitis, adenopathy, and often oral lesions. Leukemia is classified as acute or chronic
Lupus nephritis is an immune complex glomerulonephritis that commonly affects patients with systemic lupus erythematosus. It is defined by inflammation of the kidneys caused by the deposition of immune complexes. The pathogenesis involves an aberrant immune response against nuclear material from apoptotic cells. This can lead to activation of dendritic cells and production of autoantibodies, forming immune complexes that deposit in the kidneys and cause inflammation. A renal biopsy is used to classify lupus nephritis based on the type and severity of glomerular and tubulointerstitial lesions present. Classification guides treatment and predicts long term renal outcome.
Leukemia are neoplastic disorders of the hematopoietic system characterized by aberrant or arrested differentiation. There are two main types - acute and chronic leukemias. Acute leukemias are further classified as myeloid or lymphoid based on the lineage of the malignant cells. Chromosomal abnormalities are detected in the majority of acute leukemia cases and correlate with specific disease subtypes and clinical outcomes. Treatment involves induction chemotherapy followed by consolidation therapy and stem cell transplantation for eligible patients, with cure rates varying based on disease risk factors.
This document provides an overview of chronic lymphocytic leukemia (CLL). It defines CLL as the accumulation of mature B lymphocytes in the blood, bone marrow, lymph nodes, and spleen. CLL most commonly affects older adults and has an unknown cause. The document outlines the clinical presentation, diagnostic criteria, staging systems, complications, treatment approaches, and poor prognostic factors of CLL. It also discusses new therapies that aim to induce complete remission and eliminate minimal residual disease to improve survival outcomes in CLL patients.
This document discusses lymphoid disorders including lymphoid malignancies such as acute lymphoblastic leukemia (ALL), chronic lymphocytic leukemia (CLL), lymphoma, and multiple myeloma. It provides details on the classification, clinical manifestations, diagnosis, and management of each condition. ALL is characterized by excessive lymphoblast proliferation and can be T-cell or B-cell subtype. CLL is a malignant disorder of mature lymphocytes in blood. Lymphoma is caused by malignant lymphocytes accumulating in lymph nodes. Multiple myeloma is a plasma cell neoplasm characterized by monoclonal protein in serum/urine and bone lesions.
1. White blood cells (leukocytes) include granulocytes like neutrophils, eosinophils, and basophils which fight infection, and agranulocytes like monocytes and lymphocytes which are involved in immune responses.
2. Leukemia is a cancer of the blood cells characterized by abnormal proliferation of white blood cells. The four main types are acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, and chronic lymphocytic leukemia.
3. Factors involved in leukemia development include genetic mutations, chromosomal translocations, radiation exposure, certain chemicals, and some viruses. Maintaining overall health can help support white blood cell counts.
This document provides information on lupus nephritis (LN), including its history, definition, epidemiology, pathogenesis, and renal biopsy. Some key points:
- LN is an immune complex glomerulonephritis that commonly affects patients with systemic lupus erythematosus (SLE). It can lead to serious morbidity and mortality.
- The pathogenesis of LN involves autoimmune responses against apoptotic nuclear material, defects in clearance of apoptotic debris, and activation of innate and adaptive immune cells like myeloid dendritic cells and plasmacytoid dendritic cells.
- A renal biopsy is still the gold standard for diagnosing LN, and the histologic findings were first characterized
Hodgkins lymphoma pathogenesis and targets for therapyJan-Gert Nel
Hodgkin's lymphoma has progressed greatly in treatment since the mid-20th century with radiotherapy and chemotherapy. However, these treatments carry late toxicities. The pathogenesis of Hodgkin's lymphoma involves constitutive NF-kB activation in Reed-Sternberg cells, which promotes resistance to apoptosis. Epstein-Barr virus association varies by subtype and epidemiological factors. Emerging biologically-based treatment strategies target pathways like NF-kB, CD30, and the proteasome to induce apoptosis in Reed-Sternberg cells.
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...Oleg Kshivets
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2. INTRODUCTION
• Dendritic cells (DCs) are component of innate immune system
• Most important antigen-presenting cells
• Initiate T-cell responses against protein antigens
• Have numerous fine cytoplasmic processes that resemble
dendrites, from which they derive their name
3. Background
1868 Discovery of first dendritic cells : Langerhans cells Paul Langerhans
1973 identification of DCs in mouse lymphoid tissue Steinman and Cohn
1979 skin epidermal LCs derived from bone marrow cells Katz
1990s Generation of DCs in vitro from myeloid
hematopoietic progenitors or from monocytes
5. FUNCTIONS
• Antigen presentation and activation of T cells
• Inducing and maintaining immune tolerance
• Maintain immune memory in tandem with B cells
6.
7. TYPES
DCs are found in
steady state (non-inflammatory)
non steady state (inflammatory )
• Steady state DCs include
Conventional DCs (cDCs)-
a. the myeloid-like CD11c+CD11b+CD8α- DC in the circulation and
peripheral lymphoid tissues
b. the lymphoid like CD11c+CD11b-CD8α+ DC
Type-1 interferon-producing plasmacytoid DCs (pDCs) : CD11c low
DC
8. SUBTYPES
cDCs can also be divided into
subsets according to their tissue
localizations:
• Follicular dendritic cell (FDC)
• Interdigitating cell (IDC)
• Langerhans cell (LC)
• Veiled cell (VC)
• Mucosal dendritic cell- mucosal-
associated lymphoid tissue (MALT)
K
Tonsils
Thymus
Lymphatics
Adenoids
Lymph node
Thoracic
Duct
Langerhans
cells
Interstitial
Dendritic Cells
Veiled
cells
9.
10. LANGERHANS CELLS (LC)
• Specialized dendritic cells
in mucosal sites and skin
• Upon activation become
specialized for antigen
presentation to T cells
• Migrate to the lymph node
through lymphatics
14. PLASMACYTOID DENDRITIC CELLS (PDC)
• Distinct lineage of dendritic cells
• The histogenetic origin - controversial
• Likely of myelomonocytic lineage
• Interferon alpha-producing PDC precursors only acquire a
dendritic appearance in cell culture
• Circulate in the peripheral blood
• Have the capacity to enter lymph nodes and tissue through high
endothelial venules
15.
16.
17. FOLLICULAR DENDRITIC CELLS (FDC)
• Resident within primary and
secondary B-cell follicles
• Trap and present antigen to B
cells
• Appear to be closely related to
BM stromal progenitors with
features of myofibroblasts
• Non-migrating population
• Form a stable meshwork within
the follicle
18. FIBROBLASTIC
RETICULAR CELLS
(FRC)
• Involved in maintenance
of lymphoid integrity
• Produce and transport
cytokines and other
mediators
• They ensheath the
postcapillary venules
• Express SMA
19. DCs that are not found in the steady state but develop after
infection or inflammation include
1. Monocyte-derived DCs
2. TNF-producing and inducible nitric oxide synthase (iNOS)-
expressing DCs
22. LCH TERMINOLOGIES
• Eosinophilic granuloma : Solitary lesion/unifocal
• Hand –Schuller –Christian disease : multiple lesions/multifocal
• Letterer-Siwe disease : disseminated/visceral involvement
• The term HISTIOCYTOSIS X was coined by Lichtenstein in
1953
• The term LCH was used in 1985 to cover all these syndromes
23. EPIDEMIOLOGY
• Annual incidence of LCH is 5/10,00,000 ( mostly children)
• Median age at diagnosis is 30 months old
• multisystem disease usually found in children < 2 yr old
• multifocal single site disease usually in children 2-5 yr old
• M:F-3.7:1
• 70% of children have only 1 site of disease (bone most common)
• Primary LCH of lung associated with smoking
• Association with follicular lymphoma can be seen rarely
24. PATHOGENESIS
• IL1 loop model has been proposed
• High levels of the tyrosine phosphatase SH1 in lesional tissues
• Increased levels of IL17A in peripheral blood and lesional
tissues, particularly in patients with multiorgan disease
27. CLINICAL FEATURES
Presenting signs and symptoms varies depending upon areas
involved
• Unifocal disease :
older children or adults
lytic bone lesion eroding the cortex
solitary lesions at other sites - mass lesions or enlarged lymph
nodes
28. CLINICAL FEATURES
• Unisystem Multifocal Disease
young children
multiple or sequential destructive bone lesions, adjacent soft
tissue masses
Diabetes insipidus follows cranial involvement
• Multisystem Involvement
infants present with fever, cytopenias, skin ,bone lesions and
hepatosplenomegaly
29. LCH-BONES
• Presents as pain and swelling; rarely,
fracture
• Loose teeth if jaw involved
• Involved in 80% of patients
• 50% with 1 lesion; 50% with multiple
lesions
• 50% of all bone lesions found in skull and
facial bones
• 10% - vertebral body involvement
(collapsed vertebral body= “vertebra
plana”)
• Lesions in bones may last for years
30. LCH-SKIN
• Skin involved in over 50% of cases (2nd
most commonly involved organ)
• Crusty, petechial papules in scalp often
mistaken for seborrheic dermatitis
• Skin folds and diaper area frequently
involved
• Distal limbs usually spared
31. LCH-CNS
• Involvement of hypothalamic-pituitary axis (HPA) : 25% of LCH
cases
• May present with excessive thirst and large urine output,
manifestations of diabetes insipidus (permanent)
• Median age of onset : 4 years
• Seen on CT or MRI of brain
• In up to 10% of those with multisystem disease, other CNS
problems are found (degenerative CNS changes 5-15 years after
diagnosis)
32. LCH-LUNGS
• Lung involvement – 15% of LCH cases
• High-resolution CT scan : diffuse micronodular densities with cyst
formation-- honeycomb lung
• Almost never seen as the only site of involvement
• Can be asymptomatic or present with shortness of breath
• Confirmed by lung biopsy or bronchoalveolar lavage
33. LCH- LIVER/GIT
• Soft, enlarged liver often accompanied by enlarged spleen and
jaundice
• Decrease in proteins produced by liver (albumin, clotting factors)
• GI tract involvement is rarely reported but may present as
diarrhoea
34. LCH- BONE MARROW
• Hematologic problems are most often seen in those < 2 years old
• Found in patients with more severe disease
• It is characterized by anemia, neutropenia, and thrombocytopenia
• Erythrophagocytosis is a factor in the pathogenesis of anemia
• Because bone marrow and splenic involvement often occur
together, splenic sequestration further exaggerates the cytopenias
44. GENETIC PROFILE
• LCH has been shown to be clonal by X- linked androgen
receptor gene (HUMARA) assay
• Clonal IGH, IGK, or TR rearrangements, including some cases
with both T-cell and B-cell gene rearrangements : 30% cases
• BRAF V600E mutation : 50% cases ( no prognostic
significance)
• Somatic MAP2K1 mutations almost always occurring in BRAF
germline cases : 25% cases
45. PROGNOSIS
• The clinical course is related to staging of the disease at
presentation
• Survival for unifocal disease – 99%
• 66% mortality for young children with multisystem involvement
who do not respond promptly to therapy
46. THERAPEUTIC MODALITIES
LOCALISED
• biopsy or curettage
• radiation therapy (low dose)
• topical steroids
• intralesional steroid injections
SYSTEMIC
• oral or intravenous steroids
• oral or intravenous chemotherapy
• single agents (vinblastine, etoposide)
• combination chemotherapy
49. LANGERHANS CELL SARCOMA
• Malignant sarcoma with Langerhans cell phenotype
• Median age : 41 years (10-72)
SITES
• Skin and underlying soft tissue – most common
• Multiorgan involvement - LN, liver, spleen, lung and bone
• High stage disease seen in 44%
• Primary nodal disease – 22%
• Hepatosplenomegaly is noted in 22%
• Pancytopenia in 11%
54. Genetic profile
• BRAFV600E mutation found in one case
Prognosis
• LC sarcoma is an aggressive, high-grade malignancy, with >
50% mortality from progressive disease
56. INDETERMINATE DENDRITIC CELL TUMOUR
• Neoplasm derived from normal indeterminate cells, the
precursor of Langerhans cells
SITE
•Multiple generalized papules, nodules, or plaques on the skin
•Primary lymph node or splenic disease : rarely
CLINICAL FEATURES
• Systemic symptoms are usually not present
63. Interdigitating Dendritic Cell Sarcoma
• Neoplastic proliferation of spindle to oval cells with phenoyptic
features similar to those of interdigitating dendritic cells
• Extemely rare neoplasm
• Site :
solitary LN involvement (most common)
Extranodal : skin & soft tissue
69. PROGNOSIS
• The clinical course is generally aggressive, with about half of all
patients dying of the disease
• Commonly affected visceral organs include the liver, spleen,
kidney and lung
71. FOLLICULAR DENDRITIC CELL SARCOMA
• Neoplastic proliferation of spindle to oval cells showing
morphologic and phenotype features of follicular dendritic cells
• 10-20% cases associated with hyaline vascular type of
Castleman's disease
72. • Sites
• Lymph node disease in 31% of cases (mostly cervical LN)
• Extranodal disease in 58%
• Most commonly tonsil, GIT, soft tissue, mediastinum,
retroperitoneum, omentum and lung
• Both nodal and extranodal disease in 10%
73. CLINICAL FEATURES
• Patients most often present with a slow growing, painless mass
lesion
• Most patients have localized disease at presentation
• Systemic symptoms are uncommon
74.
75.
76.
77. IMMUNOPHENOTYPE
POSITIVE NEGATIVE
Follicular dendritic cell marker – CD
21, CD35, CD23
Myeloid markers : CD 13,CD 33, MPO
Clusterin (strong) HMB 45
EGFR CD1a
Desmoplakin , Vimentin, Fascin
HLA-DR , EMA
CD68 and S100 : variable
80. PROGNOSIS
• FDC sarcoma is usually treated by complete surgical excision,
with or without adjuvant radiotherapy or chemotherapy
• Associated with a worse prognosis
large tumour size (> 6 cm)
coagulative necrosis
high mitotic count (>5 mitoses/10 HPF)
significant cytological atypia
82. • Occurs in young to middle-aged adults, with a female predilection
• Consistently associated with EBV (monoclonal episomal form)
Site
It involves the liver or spleen or both
Rarely, it involves the GITas a polypoidal lesion
Clinical features
asymptomatic or abdominal distension or pain, and occasionally
systemic symptoms
85. IMMUNOPHENOTYPE
• Positive for FDC markers, such as CD21 and CD35
• Staining ranging from extensive to very focal
• Some cases, they may be negative for FDC markers but express
SMA
• This raises the possibility of fibroblastic reticular cell
differentiation
89. FIBROBLASTIC RETICULAR CELL TUMOUR
• Very rare tumor
• Also known as CK positive interstitial reticulum cell tumours
Site
• Can occur in the lymph nodes, spleen or soft tissue
92. ULTRASTRUCTURE
• The spindle cells show delicate cytoplasmic extensions and
features similar to those of myofibroblasts :
filaments with occasional fusiform densities
well-developed desmosomal attachments
rough endoplasmic reticulum
basal lamina-like material
93. PROGNOSIS
• Available data regarding outcome are extremely limited
• The clinical outcome is variable
• Some patients die of the disease
95. Disseminated Juvenile Xanthogranuloma
(JXG)
• Characterized by a proliferation of histiocytes similar to those of
the dermal JXG
• Foamy(xanthomatous) component with Touton-type giant cells
• Originate from dermal/interstitial dendritic cell
• Most deep, visceral and disseminated forms occur by the age of
10 years
• Fifty percent within the first year of life
96. ETIOLOGY
• Known association with neurofibromatosis type 1 (NF-1)
• Patients with both are at slightly higher risk of JMML
• Patients with both LCH and JXG are also encountered
97. SITE
• Skin and soft tissues of head and neck - most common
• Disseminated forms affect mucosal surfaces – most common
upper aerodigestive tract
• Other sites - CNS, dura, pituitary stalk, eye, liver, lung, lymph
node & bone marrow
98. CLINICAL FEATURES
• Skin papules small (1-2mm) and multiple
• Optic lesions can cause glaucoma
• CNS and pituitary lesions can cause diabetes insipidus, seizures,
hydrocephalus and mental status changes
• Benign but Macrophage activation syndrome can lead to
cytopenias, liver damage and death (in the systemic forms)
104. PROGNOSIS
• All clinical forms are benign
• Multiple lesions in brain, dura or pituitary can cause local
consequences and even death
• Systemic forms that involve liver and bone marrow have been
treated with LCH-type therapy
106. ERDHEIM-CHESTER DISEASE
• Clonal systemic proliferation of foamy (xanthomatous)
histiocytes with Touton giant cells
• Rare condition
• Mean patient age 55-60 years (< 15 years can be seen)
• M:F is 3:1
107. SITE
• Virtually any organ or tissue can be infiltrated by ECD
• Skeletal (> 95%), Cardiovascular (50%), retroperitoneal (33%)
• CNS, diabetes insipidus, and/or exophthalmos in 20-30% of
patients and cerebellum involvement
• Xanthelasma of eyelids /periorbital soft tissue
108. CLINICAL FEATURES
• Bone lesions can be asymptomatic or with mild pain and
affect distal limbs
• Multisystemic disease follows an aggressive course
• Cardiovascular involvement seen by MRI or CT
infiltration of the right atrium or AV groove with pericarditis,
effusion or tamponade
circumferential soft tissue sheathing of the thoracic and
abdominal aorta (Coated Aorta) &large arteries, leading to
renovascular hypertension
109. • Orbital infiltration (often bilateral) : exophthalmos, pain, oculomotor nerve
palsies, blindness, xanthelasma
• Pituitary gland infiltration causes diabetes insipidus,hyperprolactinaemia,
Gonadotropin insufficiency, hypotestosteronism
• CNS involvement :
Cerebellar and pyramidal syndromes
The most serious neurological complication is neurodegenerative cerebellar
disease (15- 20%)
CNS involvement is a major prognostic factor in ECD
116. PROGNOSIS
• The disease outcome correlates with sites of involvement
• Patients with CNS disease or multisystemic disease have a
worse outcome
• Disease activity is assessed by clinical examination, imaging and
C-reactive protein values
• ECD may respond to therapy with interferon alpha ( 5 year
survival -68%)
• Vemurafenib (inhibitor of BRAF) has recently been used with
promising results
118. BLASTIC PLASMACYTOID DENDRITIC CELL
NEOPLASM
• Clinically aggressive neoplasm derived from the precursors of
plasmacytoid dendritic cell
• Mean age 61-67 years
• M:F ratio is 3.3:1
• Mostly involves skin(64-100%) followed by bone marrow,
peripheral blood (60-90%) & lymph node (40-50%)
119. CLINICAL FEATURES
• Skin manifestations - most frequent
• The diagnosis is made on skin biopsy
• Three types of lesions
isolated (one or few) purplish nodule type (two thirds of cases)
: head & lower limbs
isolated (one or few) bruise-like papule type
disseminated type with purplish nodules and/or papules
and/or macules : most characteristic
120.
121. • In some patients lacking skin involvement and with leukaemic
presentation, the diagnosis is made based on peripheral blood
or bone marrow analyses
• In most cases, a fulminant leukaemic phase ultimately develops
• About 10-20% cases associated with or develop into other
myeloid disorders, most commonly CMML, MDS or AML
130. GENETIC PROFILE
• Two thirds of patients have an abnormal karyotype
• Complex karyotypes & recurrent translocations are common
• Gene expression analysis of BPDCN reveals a unique signature
proves that BPDCN originates from the myeloid lineage; in
particular, from resting PDCs
• TET2 - most commonly mutated gene
131. PROGNOSIS
• The clinical course is aggressive, with a median survival of
10.0-19.8 months
• Most cases (80-90%) show an initial response to multiagent
chemotherapy
• Relapses with subsequent resistance to drugs are regularly
observed have been associated with shorter survival
132. • Parameters associated with lower survival rate
High marrow or peripheral blood blastosis
low TdT expression
positivity for CD303
low Ki-67 proliferation index
CDKN2A/B deletions
mutations in DNA methylation pathway genes
136. CONCLUSION
• Dendritic neoplasms are rare neoplasms that represent less
than 1% of all the neoplasms seen in the lymph nodes or soft
tissues
• Immunohistochemistry remains the mainstay of diagnosis
• Collaborative efforts are needed to better treat patients with
these rare disorders
138. 9 year old female presented with
• Acute chest pain of few hours
• Difficulty in breathing duration
Systemic examination revealed decreased right sided breath sounds
Other systems revealed no abnormality
There was no history of fever or hemoptysis
Thorough workup revealed no bony or skin lesions
139. INVESTIGATIONS
Investigation Result
Chest X Ray Multiple pneumatocoeles and right
sided pneumothorax
CT scan Gross distortion of architecture with
multiple thin walled cysts bilaterally
Pulmonary function test Suggestive of both restrictive and
obstructive change
Blood workup for
bacterial and viral
infections
Negative
Bronchoalveolar lavage Unremarkable