Malignant  Diseases  of  the  Lymphatic  System  and  Soft  Tissues George A. Simpson, M.D., F.A.C.S.
Lymphomas – Sites of Lymphoid tissue <ul><li>Lymph nodes </li></ul><ul><li>White pulp of spleen </li></ul><ul><li>Waldeyer...
Lymphomas <ul><li>Definition: malignancy of lymphoid tissue </li></ul><ul><li>Can be T (thymus) or B (bursa) cells </li></...
Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I  Precurser B-cell tumors T-cell and putativ...
Hodgkin’s Lymphoma <ul><li>Age and incidence </li></ul><ul><ul><li>Young adulthood </li></ul></ul><ul><ul><li>Older age </...
Hodgkin’s Lymphoma <ul><li>Classification of Hodgkin’s Lymphoma </li></ul><ul><li>I  Lymphocyte predominance </li></ul><ul...
Symptoms of Hodgkin’s Lymphoma <ul><li>Asymptomatic, painless, discrete rubbery enlarged nodes </li></ul><ul><li>Usually n...
Hodgkins - Clinical Presentation <ul><li>Young people, hard lymph node usually in neck or supraclavicular fossa.  </li></u...
Hodgkin’s Lymphoma   <ul><li>Diagnosis </li></ul><ul><li>Biopsy of lymph node.   Reed-Sternberg cells mandatory </li></ul>...
Staging of Hodgkin’s Lymphoma <ul><li>Stage  Findings </li></ul><ul><li>I  Involvement of single lymph node region or sing...
Hodgkins - Staging <ul><li>Staging determines prognosis:  </li></ul><ul><ul><li>Laparotomy with tissue biopsies </li></ul>...
Hodgkins Lymphoma - Prognosis <ul><li>Staging and histology determines prognosis:  </li></ul><ul><ul><li>Lymphocyte –predo...
Normal Lymph Node
Hodgkins Disease with Reed-Sternberg Cells
Reed-Sternberg Cell
Hodgkin’s Disease <ul><li>Liver section with Hodgkin’s Disease </li></ul>
Hodgkin’s Lymphoma - Treatment <ul><li>Radiation –  for limited stage disease, above diaphragm, stage I & II </li></ul><ul...
Hodgkins
Non-Hodgkin’s Lymphomas <ul><li>Tend to be multicentric, and spread earlier </li></ul><ul><li>Etiology unknown: more frequ...
Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I  Precurser B-cell tumors T-cell and putativ...
Non - Hodgkin’s Lymphoma <ul><li>Incidence </li></ul><ul><ul><li>Childhood to old age </li></ul></ul><ul><ul><li>More prev...
Non-Hodgkin’s Lymphoma <ul><li>Histologic classification : </li></ul><ul><li>Usually disseminated throughout body </li></u...
Evaluation of Non-Hodgkins Lymphoma <ul><li>History and physical examination </li></ul><ul><li>CBC, liver function tests, ...
Nodular Lymphoma
Diffuse Lymphoma
High Grade Lymphoma
Non-Hodgkin’s Lymphoma - Treatment <ul><li>Surgery  –  For diagnosis,  (sometimes for Rx of local  focus or for compicatio...
Non-Hodgkin’s Lymphoma - Prognosis <ul><li>Favorable prognosis </li></ul><ul><li>Low grade tumors – 5 years even untreated...
Multiple Myeloma <ul><li>Definition: malignancy of plasma cells  from B cells-  010-15% of all hematologic cancers </li></...
Myeloma
Myeloma of Skull
Myeloma
Multiple myeloma - presentation <ul><li>bone pain </li></ul><ul><li>pathologic fractures: “moth holes” on X-ray </li></ul>...
Waldenstrom’s Macroglobulinemia <ul><li>Plasmacytoid lymphocyte </li></ul><ul><li>Produces large immunoglubulin molecules....
Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I  Precurser B-cell tumors T-cell and putativ...
Treatment of Non-Hodgkin’s Lymphoma   <ul><li>Surgery  – mostly for diagnosis, or small localized gastric and small  bowel...
Non- Hodgkin’s Lymphoma   <ul><li>Acute T-cell leukemia/lymphoma  </li></ul><ul><li>Large, red-violet tumor with surroundi...
Interesting Non-Hodgkins Lymphomas <ul><li>Burkitts lymphoma –  a B-cell malignancy </li></ul><ul><ul><li>Mostly in Africa...
Interesting Non-Hodgkins Lymphomas <ul><li>Burkitts lymphoma –  </li></ul><ul><li>a B-cell malignancy, seenmostly in Afric...
Lymphomas <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): patch stage </li></ul><ul><li>Generalized, flat, reddish-...
Lymphomas <ul><li>Cutaneous T-cell lymphoma </li></ul><ul><li>(mycosis fungoides): plaque stage  </li></ul><ul><li>Multipl...
Leukemia <ul><li>Acute T-cell leukemia/lymphoma  </li></ul><ul><li>Disseminated, red-violet papules </li></ul><ul><li>beco...
Lymphomas <ul><li>Cutaneous T-cell lymphoma </li></ul><ul><li>(mycosis fungoides): plaque stage </li></ul><ul><li>Multiple...
Lymphomas <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): tumor stage  </li></ul><ul><li>A fleshy, tomato-like, par...
Lymphomas   <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): leonine facies  </li></ul><ul><li>Diffuse infiltration ...
Lymphomas <ul><li>Cutaneous T-cell lymphoma: Sézary syndrome  </li></ul><ul><li>Sézary syndrome,  Sézary  erythroderma; ex...
Lymphomas <ul><li>Cutaneous B-cell lymphoma  </li></ul><ul><li>Multiple, well-defined, red-to-brownish and bluish nodules ...
Lymphomas <ul><li>Cutaneous B-cell lymphoma  </li></ul><ul><li>Multiple, skin-colored dermal tumors on the scalp. </li></ul>
Lymphomas <ul><li>Angiocentric lymphoma   Papules, nodules, tumors, and ulcers with </li></ul><ul><li>associated facial ed...
Lymphomas <ul><li>Langerhan’s cell lymphoma of infant’s scalp  </li></ul><ul><li>(Eosinophilic  granulomatosis) </li></ul>
Lymphomas <ul><li>Langerhan’s cell cytosis </li></ul><ul><li>Letterer-Siwe syndrome </li></ul><ul><li>(malignant eosinophi...
Lymphomas <ul><li>Langerhan’s cell histiocytosis of vulva </li></ul>
Sarcomas <ul><li>Etiology: </li></ul><ul><ul><li>Connective tissue of body </li></ul></ul><ul><ul><li>Genetic defects, (Vo...
Sarcoma – Risk Factors <ul><li>Occupational exposure  (asbestos  [pleural mesothelioma];  herbicides - Dioxin & Agent Oran...
Sarcomas <ul><li>Tissue Benign Malignant </li></ul><ul><li>Fibrous tissue Fibroma Fibrosarcoma </li></ul><ul><li>Adipose t...
Epidemiology of Sarcomas <ul><ul><ul><ul><ul><li>Location </li></ul></ul></ul></ul></ul><ul><li>Extremities  66% </li></ul...
Sarcoma - Evaluation of Patient <ul><li>History and physical  (1/3 asymptomatic at time of  discovery) </li></ul><ul><li>I...
Sarcoma <ul><li>Sarcoma of thorax </li></ul><ul><li>on chest x-ray </li></ul>
Sarcomas  <ul><li>Method of spread </li></ul><ul><li>Contiguous growth </li></ul><ul><li>Blood stream metastases main rout...
Sarcomas - Treatment <ul><li>Treatment factors: </li></ul><ul><li>Surgery is the effective treatment ( 2 cm margin) </li><...
Sarcomas - Prognosis <ul><li>Overall 5 year survival-  Superficial low grade  50%;  Advanced metastatic, deep, high grade ...
Specific Sarcomas <ul><li>Gastrointestinal Stromal Tumor-  Gastrointestinal neural  origin; tyrosine kinase receptor in me...
KAPOSI’S SARCOMA <ul><li>Skin section to show origin of Kaposi’s sarcoma cells from  </li></ul><ul><li>connective tissue i...
KAPOSI’S SARCOMA <ul><li>Kaposi’s Sarcoma of thigh.  </li></ul><ul><li>Non-AIDS related Kaposi’s sarcoma usually found on ...
KAPOSI’S SARCOMA <ul><li>Appearance of foot </li></ul><ul><li>lesion of Kaposi’s  </li></ul><ul><li>sarcoma. </li></ul>
KAPOSI’S SARCOMA <ul><li>Aids-related Kaposi’s sarcoma often begins in peri-oral mucosa. Palate is the most common site, w...
KAPOSI’S SARCOMA <ul><li>Microscopic view of Kaposi’s Sarcoma  – endothelial cells, fibroblasts, spindle cells, capillary ...
KAPOSI’S SARCOMA <ul><li>Lesions on back </li></ul>
KAPOSI’S SARCOMA <ul><li>Kaposi’s sarcoma lesion of shoulder </li></ul>
Kaposi’s Sarcoma <ul><li>Kaposi’s sarcoma  </li></ul><ul><li>lesion of arm </li></ul>
KAPOSI’S SARCOMA <ul><li>PERIANAL </li></ul>
Kaposi’s Sarcoma - Treatment <ul><li>Surgical excision for small localized lesions </li></ul><ul><li>Chemotherapy for adva...
Lymphomas <ul><li>Mastocytosis in child with solitary mastocytoma lesion </li></ul>
Lymphomas <ul><li>Lymphoma of submental area </li></ul>
Lymphomas   <ul><li>Mastocytosis in a child </li></ul>
Lymphomas <ul><li>Mastocytosis of back  </li></ul>
Lymphomas <ul><li>Generalized mastocytosis </li></ul><ul><li>(White areas are unaffected skin) </li></ul>
Lymphomas <ul><li>Lymphomatoid papulosis </li></ul>
Lymphomas <ul><li>Lymphomatoid papulosis  lesions of legs  </li></ul>
Lymphomas <ul><li>Lymphoma of right chest on x-ray </li></ul>
Myelogenous leukemia <ul><li>Acute myelogenous leukemia with leukemia cutis </li></ul><ul><li>Hundreds of tan-pink papules...
Leukemia <ul><li>Leukemia Cutis with  </li></ul><ul><li>ulceration and pustules  </li></ul><ul><li>of lower abdomen </li><...
Lymphomas  <ul><li>Lymphoma lesion of </li></ul><ul><li>mouth: eosinophilic granuloma </li></ul>
THE  END
Diagram of Skin Layers
<ul><li>Sézary syndrome,Sézary erythroderma;exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltr...
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Malignant Diseases Lymphatics & Soft Tissue #3

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Malignant Diseases Lymphatics & Soft Tissue #3

  1. 1. Malignant Diseases of the Lymphatic System and Soft Tissues George A. Simpson, M.D., F.A.C.S.
  2. 2. Lymphomas – Sites of Lymphoid tissue <ul><li>Lymph nodes </li></ul><ul><li>White pulp of spleen </li></ul><ul><li>Waldeyer’s ring </li></ul><ul><li>Thymus gland </li></ul><ul><li>Peyer’s patches (submucosa of respiratory and gastrointestinal tracts) </li></ul>
  3. 3. Lymphomas <ul><li>Definition: malignancy of lymphoid tissue </li></ul><ul><li>Can be T (thymus) or B (bursa) cells </li></ul><ul><li>T are “killers cells”; B make antibodies </li></ul><ul><li>B- center of lymphoid follicle; T - around the periphery </li></ul><ul><li>2 main types: Hodgkins vs. Non-Hodgkins </li></ul>
  4. 4. Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I Precurser B-cell tumors T-cell and putative NK neoplasms Hodgkin’s lymphoma </li></ul><ul><li>II Peripheral B-cell neoplasms I Precurser T-cell neoplasm I. Lymphocyte predominance </li></ul><ul><li>1. B-cell chronic lymphocytic leukemia II Peripheral T-cell and NK-cell neoplasms II. Nodular sclerosis </li></ul><ul><li>2. Lymphoplasmacytoid lymphoma 1. T-cell chronic lymphocytic leukemia III. Mixed cellularity </li></ul><ul><li>3. Mantle cell lymphoma 2. Large granular lymphocyte leukemia IV. Lymphodepletion </li></ul><ul><li>4. Follicle cell lymphoma 3. Mycosis fungoides or sezary syundrome V. Provisioal: Lymphocyte-rich 5. 5. 5. Marginal zone B-cell lymphoma 4. Peripheral T-cell lymphomas, unspecified classical Hodgkin’sclass 5. Angioimmunoblastic T-cell lymphoma </li></ul><ul><li>6. Provisional: splenic marginal 6 Angiocentric lymphoma.. </li></ul><ul><li>7. Hairy cell leukemia 7. Intestinal T-ce;; lymphoma (with or without . </li></ul><ul><li>8. Plasmacytoma or plasma cell myeloma associated enteropathy) </li></ul><ul><li>9. Diffuse large cell lymphoma 8. Adult T-cell lymphoma or leukemia </li></ul><ul><li>10. Burkitt’s lymphoma 9. Anaplastic large cell lymphoma </li></ul><ul><li>11. Provisional: High grade Burkitt-like 10. Provisional entity: Anaplastic large cell lymphoma, </li></ul><ul><li> Hodgkin;s-like </li></ul>
  5. 5. Hodgkin’s Lymphoma <ul><li>Age and incidence </li></ul><ul><ul><li>Young adulthood </li></ul></ul><ul><ul><li>Older age </li></ul></ul><ul><ul><li>(Rare before 10 years of age) </li></ul></ul><ul><li>Approximately 13% of all lymphomas </li></ul><ul><li>Twin “peaks” 10-20, then after age 50 </li></ul><ul><li>Unknown etiology, Epstein-Barr virus suspect </li></ul>
  6. 6. Hodgkin’s Lymphoma <ul><li>Classification of Hodgkin’s Lymphoma </li></ul><ul><li>I Lymphocyte predominance </li></ul><ul><li>II Nodular sclerosis </li></ul><ul><li>III Mixed cellularity </li></ul><ul><li>IV Lymphocyte depletion </li></ul><ul><li>V Provisional entity: lymphocyte –rich classical Hodgkin’s lymphoma </li></ul>
  7. 7. Symptoms of Hodgkin’s Lymphoma <ul><li>Asymptomatic, painless, discrete rubbery enlarged nodes </li></ul><ul><li>Usually noticed first in neck (90% supradiaphragmatic in young adults, 25% subdiaphragmatic in older adults) </li></ul><ul><li>Mediastinal adenopathy seen on routine chest x-ray </li></ul><ul><li>Dry, non-productive cough </li></ul><ul><li>Fever and night sweats (Pel-Ebstein cyclical fever rare) </li></ul><ul><li>Malaise </li></ul><ul><li>Weight loss </li></ul><ul><li>Waxing and waning of lymph node size </li></ul>
  8. 8. Hodgkins - Clinical Presentation <ul><li>Young people, hard lymph node usually in neck or supraclavicular fossa. </li></ul><ul><li>Old people, can be anywhere </li></ul><ul><li>Low grade fever ( Pel-Ebstein ), night sweats, weight loss. Abdominal pain with ETOH. </li></ul><ul><li>May have immune suppression </li></ul><ul><li>Diagnosis depends on pathognomonic Reed- Sternberg cell </li></ul>
  9. 9. Hodgkin’s Lymphoma <ul><li>Diagnosis </li></ul><ul><li>Biopsy of lymph node. Reed-Sternberg cells mandatory </li></ul><ul><li>Staging </li></ul><ul><li>Ultrasound and CT scan </li></ul><ul><li>Lymphogram </li></ul><ul><li>Bone marrow biopsy </li></ul><ul><li>Laparotomy (rarely used now) </li></ul><ul><li>Liver biopsy </li></ul><ul><li>Gallium scan </li></ul>
  10. 10. Staging of Hodgkin’s Lymphoma <ul><li>Stage Findings </li></ul><ul><li>I Involvement of single lymph node region or single extralymphatic organ (Ie) </li></ul><ul><li>II 2 or more lymph node regions on same side of diaphragm or localized involvement of an extralymphatic organ or site (IIe) </li></ul><ul><li>III Lymph node regions on both sides of diaphragm or localized involvement </li></ul><ul><li>of an extralymphatic organ . Spleen (IIIs) or both (IIIse) </li></ul><ul><li>IV Diffuse or disseminated involvement of one or more extralymphatic organs </li></ul><ul><li>with or without associated node involvement </li></ul><ul><li>Stage A Asymptomatic Stage B Fever, sweats, weight loss </li></ul>
  11. 11. Hodgkins - Staging <ul><li>Staging determines prognosis: </li></ul><ul><ul><li>Laparotomy with tissue biopsies </li></ul></ul><ul><ul><li>lymphangiogram, CAT scans may preclude laparotomy </li></ul></ul><ul><li>Stages: </li></ul><ul><ul><li>I - localized to one area </li></ul></ul><ul><ul><li>II - Above the diaphragm (or below) </li></ul></ul><ul><ul><li>III - Above and below the diaphragm </li></ul></ul><ul><ul><li>IV- infiltration of organs. </li></ul></ul><ul><ul><li>A is asymptomatic. B is with symptoms. </li></ul></ul>
  12. 12. Hodgkins Lymphoma - Prognosis <ul><li>Staging and histology determines prognosis: </li></ul><ul><ul><li>Lymphocyte –predominant group has best prognosis </li></ul></ul><ul><ul><li>nodular-sclerosis group – next best prognosis </li></ul></ul><ul><ul><li>then mixed cellularity and lymphocyte depleted </li></ul></ul><ul><li>80% of patients cured with adequate treatment </li></ul><ul><li>Risk of secondary tumor increases in time </li></ul><ul><ul><li>(perhaps caused by treatment - Same area as radiation, different cell type, three year interval ) </li></ul></ul><ul><li>Risk of leukemia -3% in 10 years </li></ul>
  13. 13. Normal Lymph Node
  14. 14. Hodgkins Disease with Reed-Sternberg Cells
  15. 15. Reed-Sternberg Cell
  16. 16. Hodgkin’s Disease <ul><li>Liver section with Hodgkin’s Disease </li></ul>
  17. 17. Hodgkin’s Lymphoma - Treatment <ul><li>Radiation – for limited stage disease, above diaphragm, stage I & II </li></ul><ul><li>Chemotherapy – with or without radiation (3000 to 4000 rads) </li></ul><ul><ul><ul><li>and combination of </li></ul></ul></ul><ul><ul><ul><li>(1) nitrogen Mustard, vincristine ( o ncovin), p rocarbazine, and p rednisone, a regimen called MOPP. 80% cure, but 2% chance of leukemia (17% with chemo and radiation). or </li></ul></ul></ul><ul><ul><ul><li>(2) doxorubicin ( a driamycin). b leomycin, v inblastine, and d acarbazine, called ABVD </li></ul></ul></ul>
  18. 18. Hodgkins
  19. 19. Non-Hodgkin’s Lymphomas <ul><li>Tend to be multicentric, and spread earlier </li></ul><ul><li>Etiology unknown: more frequent in immunocompromised patients such as: AIDS, renal transplant. </li></ul><ul><li>70-80% are B-cell, 1% histiocytes, the rest T-cells; 13% go to leukemic phase </li></ul><ul><li>Prognosis depends on histologic classification </li></ul>
  20. 20. Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I Precurser B-cell tumors T-cell and putative NK neoplasms Hodgkin’s Lymphmoma </li></ul><ul><li>II Peripheral B-cell neoplasms I Precurser T-cell neoplasm I. Lymphocyte predominance </li></ul><ul><li>1. B-cell chronic lymphocytic leukemia II Peripheral T-cell and NK-cell neoplasms II. Nodular sclerosis </li></ul><ul><li>2. Lymphoplasmacytoid lymphoma 1. T-cell chronic lymphocytic leukemia III. Mixed cellularity </li></ul><ul><li>3. Mantle cell lymphoma 2. Large granular lymphocyte leukemia IV. Lymphodepletion </li></ul><ul><li>4. Follicle cell lymphoma 3. Mycosis fungoides or Sezary syndrome V. Provisioal: Lymphocyte-rich 5. 5. 5. Marginal zone B-cell lymphoma 4. Peripheral T-cell lymphomas, unspecified classical Hodgkin’s class 5. Angioimmunoblastic T-cell lymphoma </li></ul><ul><li>6. Provisional: splenic marginal 6 Angiocentric lymphoma.. </li></ul><ul><li>7. Hairy cell leukemia 7. Intestinal T-cell lymphoma (with or without . </li></ul><ul><li>8. Plasmacytoma or plasma cell myeloma associated enteropathy) </li></ul><ul><li>9. Diffuse large cell lymphoma 8. Adult T-cell lymphoma or leukemia </li></ul><ul><li>10. Burkitt’s lymphoma 9. Anaplastic large cell lymphoma </li></ul><ul><li>11. Provisional: High grade Burkitt-like 10. Provisional entity: Anaplastic large cell lymphoma, </li></ul><ul><li> Plasma cells (Multiple myeloma) Hodgkin;s-like </li></ul>
  21. 21. Non - Hodgkin’s Lymphoma <ul><li>Incidence </li></ul><ul><ul><li>Childhood to old age </li></ul></ul><ul><ul><li>More prevalent in elderly </li></ul></ul><ul><ul><li>Etiology – unknown, but predisposing factors: </li></ul></ul><ul><ul><li>Occupation (chemicals, forestry, agriculture) </li></ul></ul><ul><ul><li>Inherited conditions (poor immune system) </li></ul></ul><ul><ul><li>Certain medications (Chemotherapy drugs) </li></ul></ul><ul><ul><li>HIV and AIDS patients susceptible </li></ul></ul><ul><ul><li>Organ transplant patients susceptible </li></ul></ul>
  22. 22. Non-Hodgkin’s Lymphoma <ul><li>Histologic classification : </li></ul><ul><li>Usually disseminated throughout body </li></ul><ul><li>Nodular vs. Diffuse </li></ul><ul><li>MALT ( m ucosa a ssociated l ymphatic t issue) outside the lymph nodes in skin, G.I tract, thyroid, breast </li></ul><ul><li>Mature (small) vs. Immature (large) </li></ul><ul><li>Small lymphocyte: cleaved vs. non-cleaved </li></ul><ul><li>Best prognosis is nodular, small cell </li></ul><ul><li>Worst prognosis is diffuse, large cell or immunoblastic (high grade) </li></ul>
  23. 23. Evaluation of Non-Hodgkins Lymphoma <ul><li>History and physical examination </li></ul><ul><li>CBC, liver function tests, chest x-ray, CT scan of chest, abdomen, and pelvis </li></ul><ul><li>Bone marrow biopsy </li></ul><ul><li>Lumbar puncture </li></ul><ul><li>Staging laparotomy usually not indicated </li></ul><ul><li> (disease is disseminated vs. localized disease of Hodgkin’s) </li></ul>
  24. 24. Nodular Lymphoma
  25. 25. Diffuse Lymphoma
  26. 26. High Grade Lymphoma
  27. 27. Non-Hodgkin’s Lymphoma - Treatment <ul><li>Surgery – For diagnosis, (sometimes for Rx of local focus or for compications- perforation, obstruction) </li></ul><ul><li>Radiation – for limited stage disease, above diaphragm, stage I & II; Superior vena cava obstruction, spinal cord compression </li></ul><ul><li>Chemotherapy - with or without radiation (3000 - 4000 rads) </li></ul><ul><ul><ul><li>and combination of </li></ul></ul></ul><ul><ul><ul><li>(1) nitrogen M ustard, vincristine ( O ncovin), p rocarbazine, and p rednisone, a regimen called MOPP. 80% cure, but 2% chance of leukemia (17% with chemo and radiation). or </li></ul></ul></ul><ul><ul><ul><li>(2) doxorubicin ( a driamycin). b leomycin, v inblastine, and d acarbazine, called ABVD </li></ul></ul></ul>
  28. 28. Non-Hodgkin’s Lymphoma - Prognosis <ul><li>Favorable prognosis </li></ul><ul><li>Low grade tumors – 5 years even untreated </li></ul><ul><li>Poor prognosis </li></ul><ul><li>high grade tumors (poor differentiation) – best response is to chemotherapy </li></ul><ul><li>older than 60 yrs. </li></ul><ul><li>systemic symptoms </li></ul><ul><li>bulky disease </li></ul><ul><li>extra-nodal disease </li></ul><ul><li>G.I or bone marrow involvement </li></ul>
  29. 29. Multiple Myeloma <ul><li>Definition: malignancy of plasma cells from B cells- 010-15% of all hematologic cancers </li></ul><ul><li>Clone of plasma cells produces immunoglobulins. Can be light chain, heavy chain, Kappa or Lambda. </li></ul><ul><li>Bence-Jones protein in urine </li></ul><ul><li>Produces bone lysis by OAF - Osteoclast Activation Factor: </li></ul>
  30. 30. Myeloma
  31. 31. Myeloma of Skull
  32. 32. Myeloma
  33. 33. Multiple myeloma - presentation <ul><li>bone pain </li></ul><ul><li>pathologic fractures: “moth holes” on X-ray </li></ul><ul><li>Increased calcium, protein, globulin </li></ul><ul><li>Problems include: hyperviscosity, Rouleaux , amyloidosis </li></ul><ul><li>Survival: 2-3 years. BMT gives best chance. </li></ul>
  34. 34. Waldenstrom’s Macroglobulinemia <ul><li>Plasmacytoid lymphocyte </li></ul><ul><li>Produces large immunoglubulin molecules. </li></ul><ul><li>Main problem is hyperviscosity. </li></ul><ul><li>Treated with plasmapheresis, sometimes chemotherapy </li></ul><ul><li>More benign course than myeloma </li></ul>
  35. 35. Classification of Lymphoid Tumors <ul><li>B-Cell neoplasms </li></ul><ul><li>I Precurser B-cell tumors T-cell and putative NK neoplasms Hodgkin’s lymphoma </li></ul><ul><li>II Peripheral B-cell neoplasms I Precurser T-cell neoplasm I. Lymphocyte predominance </li></ul><ul><li>1. B-cell chronic lymphocytic leukemia II Peripheral T-cell and NK-cell neoplasms II. Nodular sclerosis </li></ul><ul><li>2. Lymphoplasmacytoid lymphoma 1. T-cell chronic lymphocytic leukemia III. Mixed cellularity </li></ul><ul><li>3. Mantle cell lymphoma 2. Large granular lymphocyte leukemia IV. Lymphodepletion </li></ul><ul><li>4. Follicle cell lymphoma 3. Mycosis fungoides or sezary syundrome V. Provisioal: Lymphocyte-rich Marginal zone B-cell lymphoma 4. Peripheral T-cell lymphomas, unspecified classical Hodgkin’sclass 5. Angioimmunoblastic T-cell lymphoma </li></ul><ul><li>6. Provisional: splenic marginal 6 Angiocentric lymphoma.. </li></ul><ul><li>7. Hairy cell leukemia 7. Intestinal T-ce;; lymphoma (with or without . </li></ul><ul><li>8. Plasmacytoma or plasma cell myeloma associated enteropathy) </li></ul><ul><li>9. Diffuse large cell lymphoma 8. Adult T-cell lymphoma or leukemia </li></ul><ul><li>10. Burkitt’s lymphoma 9. Anaplastic large cell lymphoma </li></ul><ul><li>11. Provisional: High grade Burkitt-like 10. Provisional entity: Anaplastic large cell lymphoma, </li></ul><ul><li> Plasma cells (Multiple myeloma) Hodgkin;s-like </li></ul>
  36. 36. Treatment of Non-Hodgkin’s Lymphoma <ul><li>Surgery – mostly for diagnosis, or small localized gastric and small bowel lesions, or complications (perforation, etc,) </li></ul><ul><li>Radiation – for localized lesions (not common) and reducing mass pressure (superior vena cava or spinal cord obstruction) </li></ul><ul><li>Chemotherapy – Various combinations of several drugs. </li></ul><ul><ul><ul><ul><ul><li>Survival rates 26 to 73% high risk to low risk patients. </li></ul></ul></ul></ul></ul>
  37. 37. Non- Hodgkin’s Lymphoma <ul><li>Acute T-cell leukemia/lymphoma </li></ul><ul><li>Large, red-violet tumor with surrounding red papules and </li></ul><ul><li>nodules. </li></ul>
  38. 38. Interesting Non-Hodgkins Lymphomas <ul><li>Burkitts lymphoma – a B-cell malignancy </li></ul><ul><ul><li>Mostly in Africa and Caribbean; associated with Epstein-Barr virus, Involvement of mandible is common. Very responsive to chemotherapy. </li></ul></ul><ul><ul><li>Mycosis fungoides – cutaneous T-cell lymphoma. Circulating malignant cells with convoluted nuclei are called Sezary cells. Often misdiagnosed as eczema or dermatosis. </li></ul></ul><ul><ul><li>Langerhan’s cell lymphoma- Eosinophilic granuloma, associated with Hand-Schuller-Christian syndrome (Exophthalmus, diabetes insipidus, and destructive bone lesions </li></ul></ul><ul><ul><li>. </li></ul></ul>
  39. 39. Interesting Non-Hodgkins Lymphomas <ul><li>Burkitts lymphoma – </li></ul><ul><li>a B-cell malignancy, seenmostly in Africa and Caribbean; associated with Epstein-Barr virus, Involvement of mandible is common. Very responsive to chemotherapy. </li></ul><ul><ul><li>Burkitt's lymphoma is a form of malignant lymphoma reported in African children, frequently involving facial bones, ovaries, and abdominal lymph nodes, which are infiltrated by undifferentiated stem cells with scattered pale macrophages containing nuclear debris; undifferentiated cells show numerous mitoses from lymphoid germinal center B-cells. Geographical distribution of Burkitt's lymphoma suggests that it is found in areas with endemic malaria and caused by Epstein-Barr virus, a member of the Herpesviridae; occasional cases of lymphoma with similar features have been reported in the United States. </li></ul></ul><ul><ul><li>. </li></ul></ul>
  40. 40. Lymphomas <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): patch stage </li></ul><ul><li>Generalized, flat, reddish-to-brownish plaques with some scaling, mimicking </li></ul><ul><li>psoriasis. </li></ul>
  41. 41. Lymphomas <ul><li>Cutaneous T-cell lymphoma </li></ul><ul><li>(mycosis fungoides): plaque stage </li></ul><ul><li>Multiple annular and circinate plaques, with some psoriasiform scale on the buttocks. </li></ul>
  42. 42. Leukemia <ul><li>Acute T-cell leukemia/lymphoma </li></ul><ul><li>Disseminated, red-violet papules </li></ul><ul><li>becoming confluent on the </li></ul><ul><li>chest. </li></ul>
  43. 43. Lymphomas <ul><li>Cutaneous T-cell lymphoma </li></ul><ul><li>(mycosis fungoides): plaque stage </li></ul><ul><li>Multiple annular and circinate plaques, some with psoriasi-form scale, some with erosion, indurated upon palpation. </li></ul>
  44. 44. Lymphomas <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): tumor stage </li></ul><ul><li>A fleshy, tomato-like, partially eroded nodule arising from a larger, slightly elevated, </li></ul><ul><li>psoriasiform plaque with scaling and central atrophy. </li></ul>
  45. 45. Lymphomas <ul><li>Cutaneous T-cell lymphoma (mycosis fungoides): leonine facies </li></ul><ul><li>Diffuse infiltration of the skin has caused a coarsening of the facial features, with thickening </li></ul><ul><li>of the brows and glabella; the neck and trunk are also involved. </li></ul>
  46. 46. Lymphomas <ul><li>Cutaneous T-cell lymphoma: Sézary syndrome </li></ul><ul><li>Sézary syndrome, Sézary erythroderma; exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltration by atypical mononuclear cells (T lymphocytes with markedly convoluted or cerebriform nuclei) also found in the peripheral blood, and associated with alopecia, edema, and nail and pigmentary changes; a variant of mycosis fungoides. </li></ul><ul><li>Appearance </li></ul><ul><li>Generalized erythema, scaling, and thickening of the skin (erythroderma); the palms are hyperkeratotic. </li></ul><ul><li>Generalized lymphadenopathy was present. The buffy coat contained 45% atypical lymphocytes (Sézary cells). </li></ul>
  47. 47. Lymphomas <ul><li>Cutaneous B-cell lymphoma </li></ul><ul><li>Multiple, well-defined, red-to-brownish and bluish nodules on the face with associated edema. </li></ul>
  48. 48. Lymphomas <ul><li>Cutaneous B-cell lymphoma </li></ul><ul><li>Multiple, skin-colored dermal tumors on the scalp. </li></ul>
  49. 49. Lymphomas <ul><li>Angiocentric lymphoma Papules, nodules, tumors, and ulcers with </li></ul><ul><li>associated facial edema. The markings on the forehead are a tribal tatoo of this </li></ul><ul><li>North African woman. </li></ul>
  50. 50. Lymphomas <ul><li>Langerhan’s cell lymphoma of infant’s scalp </li></ul><ul><li>(Eosinophilic granulomatosis) </li></ul>
  51. 51. Lymphomas <ul><li>Langerhan’s cell cytosis </li></ul><ul><li>Letterer-Siwe syndrome </li></ul><ul><li>(malignant eosinophilic lymphoma often fatal in infants ) </li></ul>
  52. 52. Lymphomas <ul><li>Langerhan’s cell histiocytosis of vulva </li></ul>
  53. 53. Sarcomas <ul><li>Etiology: </li></ul><ul><ul><li>Connective tissue of body </li></ul></ul><ul><ul><li>Genetic defects, (Von Recklinghausen’s dsease, Gardner’s syndrome </li></ul></ul><ul><ul><li>Radiation, chemical exposure, trauma </li></ul></ul><ul><ul><li>Age and incidence : </li></ul></ul><ul><ul><li>Uncommon (Less than 1% of malignant tumors) </li></ul></ul><ul><ul><li>2 per 100.000 in U.S. </li></ul></ul><ul><ul><li>Uncommon in adults </li></ul></ul><ul><ul><li>Higher incidence in children under 15 yrs (6%) . </li></ul></ul>
  54. 54. Sarcoma – Risk Factors <ul><li>Occupational exposure (asbestos [pleural mesothelioma]; herbicides - Dioxin & Agent Orange; poly vinyl chloride-PVC- [causing hepatic angiosarcoma] ) </li></ul><ul><li>Radiation (x-ray treatment, radiologists, dentists) </li></ul><ul><ul><li>8x to 50x increase in incidence following radiation treatment, </li></ul></ul><ul><ul><li>same area as radiation, different cell type, three year interval ) </li></ul></ul><ul><li>Chronic lymphedema (Lymphangiosarcoma in Stewart-Treves syndrome post radical mastectomy) </li></ul><ul><li>Gene and chromosome abnormalities (Ewing’s sarcoma, clear cell sarcoma, myxoid liposarcoma, desmoplastic round cell sarcoma, Von Recklinghausen’s syndrome, Gardner’s syndrome, etc,) </li></ul>
  55. 55. Sarcomas <ul><li>Tissue Benign Malignant </li></ul><ul><li>Fibrous tissue Fibroma Fibrosarcoma </li></ul><ul><li>Adipose tissue Lipoma Liposarcoma </li></ul><ul><li>Striated muscle Rhabdomyoma Rabdomyosarcoma </li></ul><ul><li>Smooth muscle Leiomyoma Leiomyosarcoma </li></ul><ul><li>Synovial mesothelium Mesothelioma Synovial sarcoma </li></ul><ul><li>Blood vcessels Angioma Angiosarcoma </li></ul><ul><li>Lymph vessels Lymphoangioma Lymphangiosarcoma </li></ul><ul><li>Peripheral nerve Neuroma Malignant neurolemmoma </li></ul><ul><li> Schwannoma </li></ul><ul><li>Myofibroblast Malignant fibrous </li></ul><ul><li> histiocytoma </li></ul>
  56. 56. Epidemiology of Sarcomas <ul><ul><ul><ul><ul><li>Location </li></ul></ul></ul></ul></ul><ul><li>Extremities 66% </li></ul><ul><li>Trunk 20% </li></ul><ul><li>Retroperitoneum 13% </li></ul><ul><li>Cell type (over 50 subtypes) </li></ul><ul><li>Malignant fibrous histiocytoma 24% </li></ul><ul><li>Leiomyosarcoma 21% </li></ul><ul><li>Liposarcoma 19% </li></ul>
  57. 57. Sarcoma - Evaluation of Patient <ul><li>History and physical (1/3 asymptomatic at time of discovery) </li></ul><ul><li>Imaging studies </li></ul><ul><li>Core biopsy </li></ul><ul><li>Staging – based on tumor grade, tumor size, nodes and metastases </li></ul>
  58. 58. Sarcoma <ul><li>Sarcoma of thorax </li></ul><ul><li>on chest x-ray </li></ul>
  59. 59. Sarcomas <ul><li>Method of spread </li></ul><ul><li>Contiguous growth </li></ul><ul><li>Blood stream metastases main route </li></ul><ul><li>Occasional lymphatic spread </li></ul>
  60. 60. Sarcomas - Treatment <ul><li>Treatment factors: </li></ul><ul><li>Surgery is the effective treatment ( 2 cm margin) </li></ul><ul><li>Radiation has limited usefulness (adjuvant or neoadjuvant) </li></ul><ul><li>Age- better results in childhood </li></ul><ul><li>Degree and area of spread affects prognosis </li></ul><ul><ul><li>Grade – High grade has poorer results Grade I to IV </li></ul></ul><ul><ul><li>Grade I (low) well differentiated; Grade IV (high) poorly differentiated </li></ul></ul><ul><ul><li>Completeness of removal </li></ul></ul><ul><ul><li>Type of tumor ( this has less bearing than age, grade, or spread) </li></ul></ul>
  61. 61. Sarcomas - Prognosis <ul><li>Overall 5 year survival- Superficial low grade 50%; Advanced metastatic, deep, high grade 20% </li></ul><ul><li>Radiation has limited usefulness (adjuvant or neoadjuvant) </li></ul><ul><li>Age- better results in childhood </li></ul><ul><li>Degree and area of spread affects prognosis </li></ul><ul><ul><li>Grade – High grade has poorer results Grade I to IV </li></ul></ul><ul><ul><li>Grade I (low) well differentiated; Grade IV (high) poorly differentiated </li></ul></ul><ul><ul><li>Completeness of removal </li></ul></ul><ul><ul><li>Type of tumor ( this has less bearing than age, grade, or spread) </li></ul></ul><ul><li>Follow-up exams every 6 months x 4, then yearly </li></ul>
  62. 62. Specific Sarcomas <ul><li>Gastrointestinal Stromal Tumor- Gastrointestinal neural origin; tyrosine kinase receptor in membrane; Rx surgery and tyrosine kinase inhibitors </li></ul><ul><li>Dermatofibrosaccoma Protuberans skin and subcutaneous tumor; slow metastases; wide resection treatment of choice </li></ul><ul><li>Desmoid Tumor fibrous tissue malignancy,intermediate malignancy, surgery is Rx; may respond to NSAID’s or tamoxifen </li></ul><ul><li>Kaposi’s Sarcoma once in Jewish Mediterranean men or sub- Saharan Africans; AIDS increaced incidence; homosexual and bisexual men predominently; human herpesvirus 8 may be cause. </li></ul>
  63. 63. KAPOSI’S SARCOMA <ul><li>Skin section to show origin of Kaposi’s sarcoma cells from </li></ul><ul><li>connective tissue in the dermis layer of the skin </li></ul><ul><li>Kaposi’s sarcoma is considered an angiosarcoma </li></ul>
  64. 64. KAPOSI’S SARCOMA <ul><li>Kaposi’s Sarcoma of thigh. </li></ul><ul><li>Non-AIDS related Kaposi’s sarcoma usually found on lower extremities </li></ul>
  65. 65. KAPOSI’S SARCOMA <ul><li>Appearance of foot </li></ul><ul><li>lesion of Kaposi’s </li></ul><ul><li>sarcoma. </li></ul>
  66. 66. KAPOSI’S SARCOMA <ul><li>Aids-related Kaposi’s sarcoma often begins in peri-oral mucosa. Palate is the most common site, with rapid spread to lymph nodes </li></ul>
  67. 67. KAPOSI’S SARCOMA <ul><li>Microscopic view of Kaposi’s Sarcoma – endothelial cells, fibroblasts, spindle cells, capillary growth - Angiosarcomas </li></ul>
  68. 68. KAPOSI’S SARCOMA <ul><li>Lesions on back </li></ul>
  69. 69. KAPOSI’S SARCOMA <ul><li>Kaposi’s sarcoma lesion of shoulder </li></ul>
  70. 70. Kaposi’s Sarcoma <ul><li>Kaposi’s sarcoma </li></ul><ul><li>lesion of arm </li></ul>
  71. 71. KAPOSI’S SARCOMA <ul><li>PERIANAL </li></ul>
  72. 72. Kaposi’s Sarcoma - Treatment <ul><li>Surgical excision for small localized lesions </li></ul><ul><li>Chemotherapy for advanced cases ( vinblastine, bleomycin, doxorubicin) </li></ul><ul><li>Immunodeficiency complicates treatment </li></ul><ul><li>Radiation may shrink lesions. Palliation </li></ul>
  73. 73. Lymphomas <ul><li>Mastocytosis in child with solitary mastocytoma lesion </li></ul>
  74. 74. Lymphomas <ul><li>Lymphoma of submental area </li></ul>
  75. 75. Lymphomas <ul><li>Mastocytosis in a child </li></ul>
  76. 76. Lymphomas <ul><li>Mastocytosis of back </li></ul>
  77. 77. Lymphomas <ul><li>Generalized mastocytosis </li></ul><ul><li>(White areas are unaffected skin) </li></ul>
  78. 78. Lymphomas <ul><li>Lymphomatoid papulosis </li></ul>
  79. 79. Lymphomas <ul><li>Lymphomatoid papulosis lesions of legs </li></ul>
  80. 80. Lymphomas <ul><li>Lymphoma of right chest on x-ray </li></ul>
  81. 81. Myelogenous leukemia <ul><li>Acute myelogenous leukemia with leukemia cutis </li></ul><ul><li>Hundreds of tan-pink papules and a nodule on trunk of female </li></ul>
  82. 82. Leukemia <ul><li>Leukemia Cutis with </li></ul><ul><li>ulceration and pustules </li></ul><ul><li>of lower abdomen </li></ul>
  83. 83. Lymphomas <ul><li>Lymphoma lesion of </li></ul><ul><li>mouth: eosinophilic granuloma </li></ul>
  84. 84. THE END
  85. 85. Diagram of Skin Layers
  86. 86. <ul><li>Sézary syndrome,Sézary erythroderma;exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltration by atypical mononuclear cells (T lymphocytes with markedly convoluted or cerebriform nuclei) also found in the peripheral blood, and associated with alopecia, edema, and nail and pigmentary changes; a variant of mycosis fungoides. </li></ul>

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