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Neoplasia II Paraneoplastic Syndrome Presented by Dr. Sahar Gamal Elbager Assistant Professor of Molecular Haematology Dean of MLS Head of Haematology Department University of Medical Sciences and Technology (...

Neoplasia II Paraneoplastic Syndrome Presented by Dr. Sahar Gamal Elbager Assistant Professor of Molecular Haematology Dean of MLS Head of Haematology Department University of Medical Sciences and Technology (UMST) Neoplasia Upon completion of these lectures, the student should: Know the paraneoplastic syndrome definition and significance. Know the pathophysiology and pathogenesis of paraneoplastic syndrome Know the classification of paraneoplastic syndrome. Dr.Sahar Elbager 2 Paraneoplastic Syndrome Definition A paraneoplastic syndrome is a disease or symptom that is the consequence of cancer in the body, but not due to the local presence of cancer cells. Paraneoplastic syndrome are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor Dr.Sahar Elbager 3 Paraneoplastic Syndrome What is the significance of paraneoplastic syndrome? 1. Paraneoplastic syndrome may the earliest manifestation of neoplasm (proceed tumor diagnosis) 2. Paraneoplastic syndrome cause significant clinical problems & may even be lethal 3. Paraneoplastic syndrome may mimic metastatic disease & therefore confound treatment. Dr.Sahar Elbager 4 Pathogenesis of Paraneoplastic Syndrome Paraneoplastic syndrome is mediated through: 1. Cross reacting antibodies Immune system may produce antibodies to fight and destroy the tumor cells, unfortunately the antibodies cross react with the normal tissues and destroy them causing paraneoplastic syndromes Dr.Sahar Elbager 5 Pathogenesis of Paraneoplastic Syndrome Paraneoplastic syndrome is mediated through: 2.Production o& release of physiologically active substance from the tumor cells It may produce hormones , hormones precursors, enzymes, cytokines that Interference with normal metabolic pathways (These serves as tumor markers ) 3. Idiopathic Dr.Sahar Elbager 6 Pathogenesis of Paraneoplastic Syndrome Dr.Sahar Elbager 7 Classification of Paraneoplastic Syndrome 1.Endocrine 2. Hematological 3.Neurological 4.Mucocutaneous 5.Others Dr.Sahar Elbager 8 Endocrinopathies Most common paraneoplastic syndromes. Responsible cancer are not of endocrine origin. The secretory activity of such tumors is referred to as ectopic hormone production. Ectopic or inappropriate hormone production (paraneoplastic endocrine syndrome) is the synthesis of hormones by tumors from tissues that do not normally produce the hormone. Dr.Sahar Elbager 9 Endocrinopathies 1. Cushing syndrome 2. Syndrome of inappropriate antidiuretic hormone (SIADH ) 3. Hypercalcemia 4. Hypoglycemia 5. Polycythemia 6. Hyperaldosteronism 7. Carcinoid syndrome Dr.Sahar Elbager 10 Cushing Syndrome The Most common endocrinopathy: Underlying cancers: Small cell carcinoma of lungs Pancreatic carcinoma Neural tumors Mechanism: Excessive production of corticotrophin & corticotrophin like peptides Dr.Sahar Elbager 11 Cushing Syndrome The Most common endocrinopathy: Underlying cancers: Small cell carcinoma of lungs Pancreatic carcinoma Neural tumors Mechanism: Excessive production of corticotrophin & corticotrophin like peptides Dr.Sahar Elbager 12 Neurological 1. Peripheral neuropathies 2. Subacute cerebellar degeneration 3. Polymyopathy Mechanism: Poorly understood In some cases, the antibodies produced against antigens on cancer cells cross reacts to antigens on cells of nervous system. Dr.Sahar Elbager 13 Subacute Cerebellar Degeneration Underlying cancers: 1. SCLC 2. Breast cancer 3. Ovarian/uterus/ cancer 4. Hodgkins lymphoma Mechanism: Immunological Dr.Sahar Elbager 14 Mucocutaneous Acanthosis Nigricans Thickened darker skin in neck and armpits Hyperkeratotic skin Underlying cancers: Gastrointestinal tumours, prostate, breast or ovarian cancer Mechanism: 1. Immunological 2. Secretion of epidermal growth factor Dr.Sahar Elbager 15 Hematological 1. Venous thrombosis 2. Disseminated Intravascular Coagulation (DIC ) 3. Polycythemia 4. Anemia 5. Granulocytosis 6. Thrombocytosis 7. Autoimmune thrombocytopenia Dr.Sahar Elbager 16 Hematological Venous thrombosis/DIC Mechanism: Hypercoagulability abnormalities blood composition Increased clotting factors Chemotherapy: endothelial damage (Tissue factor) Prolonged immobilisation Dr.Sahar Elbager 17 Venous Thrombosis Underlying cancers: 1. Pancreatic carcinoma 2. Bronchogenic carcinoma Dr.Sahar Elbager 18 DIC Underlying cancers: 1. Acute promyelocytic leukemia 2. Prostatic carcinoma Dr.Sahar Elbager 19 Polycythemia Vera Underlying cancers: 1. Renal carcinoma. 2. Cerebellar carcinoma. 3. Hepatocellular carcinoma. Mechanism: May occur in response to hypoxia or ectopic production of EPO or erythropoietin- like substances. Dr.Sahar Elbager 20 Thank you Dr.Sahar Elbager 21

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