BENIGN SOFT TISSUE TUMORS 1 FIBROUS ORIGIN FIBROMA

BENIGN SOFT TISSUE TUMORS 1. FIBROUS ORIGIN: FIBROMA GIANT CELL FIBROMA POF, COF CGCG, PGCG 2. MYOFIBROBLAST: MYOFIBROMA & MYOFIBROMATOSIS 3. FAT CELLS- LIPOMA, VERRUCIFORM XANTHOMA 4. BLOOD VESSEL- HEMANGIOMS VASCULAR MALFORMATION 5. NERVE ORIGIN – TRAUMATIC NEUROMA - Pallisaded encapsulated neuroma - Neurofibroma

MALIG SOFT TISSUE TUMORS 1. TUMORS OF LYMPHOCYTE ORIGIN LYMPHOMA BURKITT’S LYMPHOMA/ NHL HOGDKIN’S LYMPHOMA 2. TUMORS OF PLASMA CELL ORIGIN PLASMA CYTOMA MULTIPLE MYELOMA 3. TUMORS OF BONE FIBROSARCOMA 4. TUMORS OF BONE OSTEOSARCOMA 5. INTERMEDIATE MALIGNANCY OF BLOOD VESSELS HEMANGIO ENDOTHELIOMA HEMANGIO PERICYTOMA 4 MALIGNANT TUMORS OF BLOOD VESSELS KAPOSIS SARCOMA EWING’S SARCOMA

MALIGNANT LYMPHOMA • GROUP OF NEOPLASMS OF VARYING DEGREES OF MALIGNANCY DERIVED FROM BASIC CELLS OF LYMPHOID TISSUE • LYMPHOCYTES OR HISTIOCYTES IN ANY OF THEIR DEVELOPMENT STAGES

NON-HODGKIN’S LYMPHOMA • HETEROGENOUS GROUP OF LYMPHOPROLIFERATIVE MALIGNANCIES WHICH CAN INVOLVE BOTH LYMPH NODES & LYMPHOID ORGANS AS WELL AS EXTRANODAL ORGANS & TISSUES

CLASSIFICATION WORKING FORMULATION CLASSIFICATION • LOW GRADE • INTERMEDIATE • HIGH GRADE

RAPPAPORT CLASSIFICATION • NODULAR • DIFFUSE • LYMPHOCYTIC • HISTIOCYTIC

REAL [ REVISED EUROPEAN – AMERICAN LYMPHOMA] • B- CELL NEOPLASMS • T- CELL – NK CELL NEOPLASMS • HODGKIN LYMPHOMA

BURKITT’S LYMPHOMA • TUMOUR OF CHILDREN OF TROPICAL AFRICA • DENIS PARSONS BURKIT • ENDEMIC & NON ENDEMIC FORM • FASTEST GROWING MALIGNANCY IN HUMANS

C/F • MAX OR MAND • SPORADIC FORM – ABDOMINAL ORGANS

ETIOLOGY • EBV • MALARIA – IMMUNOSUPRESSION – INADEQUATE T CELL RESPONSE AGAINST B CELLS INFECTED LATENTLY WITH EBV.

AFRICAN FORM • SWELLING OF AFFECTED JAW OR OTHER FACIAL BONES • LOOSENING OF TEETH • SWELLING OF LYMPH NODE • NON TENDER & RAPIDLY GROWING IN NECK OR BELOW JAW • ABDOMINAL PRESENTATION – LESS COMMON

SPORADIC FORM • • ABDOMINAL TUMOURS SWELLING & PAIN BOWEL OBSTRUCTION METABOLIC DERANGEMENT & RENAL FUNCTION IMPAIRMENT

MAJOR SIGNS • INVOLVEMENT OF JAWS OR FACIAL BONES • ENLARGED CERVICAL LYMPH NODES • ABDOMINAL MASSES • ASCITIS

• ‘STARRY SKY APPEARANCE’ • SEEN IN AFRICAN JAW LYMPHOMA • SCATTERED MACROPHAGES WITH CLEAR CYTOPLASM OFTEN PHAGOCYTIC CELLULAR DEBRIS

TREATMENT • COMBINATION CHEMOTHERAPY & CNS PROPHYLAXIS

HODGKIN’S DISEASE • MAIN TYPES OF MALIGNANT LYMPHOMA • THOMAS HODGKIN • POTENTIALLY CURABLE MALIGNANT LYMPHOMA

ETIOLOGY • EBV • ASSOCIATED WITH AIDS • GENETIC PREDISPOSITION

C/F • BIMODAL DISTRIBUTION [ 15 -34 - >55 YRS] • MALES • COMMON IN WHITES • PAINLESS ENLARGEMENT OF ONE OR MORE CERVICAL L. N • FIRM, RUBBERY IN CONSISTENCY • OVERLYING SKIN NORMAL

• UNEXPLAINED WT. LOSS • FEVER • NIGHT SWEATS • CHEST • SHORTNESS OF BREADTH • ALCOHOL INDUCED PAIN • PRURITIS • BACK, ABDOMEN, BONE PAIN

O. M • SECONDARY INVOLVEMENT OF MAND & ALVEOLAR MUCOSA

H/P HISTOPATHOLOGICAL VARIANTS: 1. 2. 3. 4. NODULAR SCLEROSIS MIXED CELLULARITY LYMPHOCYTE DEPLETED NODULAR LYMPHOCYTE – PREDOMINANT H. D

H/P • NODULAR SCLEROSIS : 60 – 80% NODULAR PATTERN FIBROSIS DIVIDING IT INTO NODULES • LACUNAR TYPE REED STERNBERG CELL – “owls eye” Mono lobulated or multi lobulated nucleus, abundant & pale cytoplasm, small nucleolous • ADOLESCENTS & YOUNG ADULTS

TREATMENT • RADIATION THERAPY & COMBINATION CHEMOTHERAPY

MULTIPLE MYELOMA • MALIGNANCY OF PLASMA CELLS • SUBSET OF B CELLS

PATHOGENESIS • MUTATION OF TERMINALLY DIFFERENTIATED B CELLS • ETIOLOGY -RADIATION -CHEMICAL -OCCUPATION

C/F • OLDER PEOPLE [ 60 -65 YRS] • MEN > WOMEN • DIFFUSE DISEASE OF BONE MARROW • AXIAL SKELETON, VERTEBRAL COLUMN, RIBS, SKULL, PELVIS, FEMUR

SOLITARY PLASMA CELL MYELOMA/ PLASMACYTOMA • SOLITARY MASS OF NEOPLASTIC MONOCLONAL PLASMA CELLS IN EITHER BONE MARROW OR SOFT TISSUE SITE • SOFT TISSUE PLASMACYTOMA • PLASMACYTOMA OF SKELETAL SYSTEM

O. M • EXTRAMEDULLARY – GINGIVA, PALATE, FLOOR OF THE MOUTH, TONGUE, TONSILS, PNS • SESSILE OR POLYPOID REDDISH MASSES

R/F • DESTRUCTIVE INTRAMEDULLARY LESION • LYTIC DESTRUCTION OF BONE

LAB VALUES • BENCE JONES PROTEIN IN THE URINE OR SERUM • MONOCLONAL PROTEIN IN SERUM OR URINE • HYPERGLOBULINEMIA, ANEMIA

TREATMENT & PROGNOSIS • LOCAL RADIOTHERAPY • COMPLETE RESECTION

Malignant tumors of blood vessels • • Hemangioendothelioma Hemangiopericytoma Angiosarcoma Kaposi’s sarcoma

HEMANGIOENDOTHELIOMA • BEHAVIOUR B/W BENIGN HEMANGIOMA & MALIGNANT ANGIOSARCOMA. • ASSOCIATED WITH DEVELOPMANTAL ANOMALIES • CHROMOSOMAL TRANSLOCATIONS

C/F • • 2 ND – 3 RD DECADE OF LIFE NO GENDER PREDILECTION SKIN & SUBCUT TISSUE O. M – Gingiva, Tongue, Lips, Palate central • Localized swelling with occasional pain

MALIGNANT HEMANGIOENDOTHELIOMA • Flat or slightly raised • Dark red or bluish • Ulcerated • Tendency to bleed

H/F • Biphasic proliferation of venous & capillary vessles • Epitheloid hemangioendothelioma – epitheloid cells • Spindle cell hemangioendothelioma – spindle cells

TREATMENT & PROGNOSIS • WIDE SURGICAL EXCISION • METASTASIS ? ? ?

HEMANGIOPERICYTOMA • NEOPLASM WHICH IS BENIGN HAVING A DEFINITE MALIGNANT COUNTER-PART • H&N 16 -25% • Chromosomal Translocations

C/F • • Red or bluish mass Before 2 nd or after 7 th decade Soft rubbery, Painless Well demarcated Sessile or pedunculated Surface lobular or telangiectasias INFANTILE HEMANGIOPERICYTOMAMultiple, Congenital, Rapid enlargement

H/F • GROSS • Grayish white • Consistency- Solid or spongy Friable or granular

Tumour of pericytes Numerous vascular channels lined by plump endothelial cells Surronding oval or spindle cells with hyperchromatic nuclei Branching vascular channels – STAG HORN PATTERN

OLDER LESIONS Less cellularity Mucoid interstitial appearance Focal cartilage

MALIGNANT SOFT TISSUE TUMORS - FIBROBLASTIC ORIGIN