Lymphoma

LYMPHOMA
LYMPHOMAS—MESENCYMALLY/MESODERM DERIVED LYMPHORETICULAR MALIGNANCIES
STAIN  +VIMENTIN
CLASSIFICATION
B-CELL LINEAGE MORE COMMON BUT T-CELL TUMORS CAN OCCUR AND TEND TO BE MORE AGGRESSIVE
NON-HODGKIN’S (55%)—-MOST COMMON– SURVIVAL WORSE
-MULTIPLE SUBTYPES WITH EACH BEING GRADED AS LOW, INTERMEDIATE, OR HIGH GRADE TUMORS
-INTERMEDIATE SUBTYPE IS MOST COMMON IN THE H+N
NON-HODGKIN’S CAN HAVE A SIGN. PROPORTION OF EXTRANODAL DZ WHICH IS USUALLY IN WALDEYER’S RING
-RISK STEADILY INCREASES WITH AGE
-M>F
-MORE AGGRESSIVE IN YOUNGER PTS
PRESENTATION    -MOST COMMON IS A PAINLESS NECK MASS BUT CAN BE PAINFUL IF RAPIDLY GROWING WITH CENTRAL NECROSIS
CAN ALSO HAVE SORETHROAT, DYSPHAGIA, GLOBUS, CHL, EPISTAXIS, AND SINUSITIS
CONSTITUTIONAL (B-TYPE) Sx COMMON——UNLESS ISOLATED H&N D
-HODGKIN’S (45%)
MOST COMMONLY SEEN INITIALLY BY THE OTOLARYNGOLOGIST—PRESENT AS AN ASx  NECK MASS-85%
HODGKIN’S IS ALMOST ALWAYS NODAL DZ
BETTER SURVIVAL
BIMODAL INCIDENCE—10-20 (RARE UNDER 5) AND 50-60
MALE 3:1
REED STERNBERG CELLS—–LARGE MULTINUCLEATE, PERINUCLEAR HALOS—EOSINOPHILIC CYTOPLASM, GIANT “MIRROR IMAGE” NUCLEOLI—OWL EYE APPEARANCE
CELL TYPES:
-NODULAR SCLEROSISING – MOST COMMON(40%)–LACUNAR CELLS
-LYMPHOCYTE PREDOMINANT (2ND)–GOOD Px
-MIXED CELLULARITY
-LYMPHOCYTE DEPLETED—RARE
*HISTOLOGICALLY DISTINGUISHED BY THE  REED-STERNBERG CELLS
OTHER SUBTYPES
UNDIFF
NON-BURKITTS
BURKITTS—EXCLUSIVELY IN CHILDREN—FASTEST GROWING TUMOR
STARRY SKY PATTERN ON H & E STAIN
-ENDEMIC SUBTYPE–AFRICAN (I’VE SEEN THIS) EBV+, BETTER Px (OFTEN A FACIAL MASS)
-NON-ENDEMIC SUBTYPE (OFTEN AN ABD MASS), EBV-, WORSE Px
EXTRANODAL ANGIOCENTRIC PERIPHERAL T-CELL LYMPHOMAS
HISTIOCYTOSIS X = RETICULOENDOTHELIOSIS = (LCH) LANGERHANS CELL HISTIOCYTOSIS
NEW NOMENCLATURE –UNIFOCAL LCH, MULTIFOCAL LCH CONFINED TO SKELETAL SYSTEM, MULTIFOCAL LCH WITH SOFT TISSUE INVOLVEMENT
MAY TREAT ALL LIKE LYMPHOMAS = RT, CYTOXAN, STEROIDS, CURETTAGE
LETTERER-SIWE (“BETTER SEND A LETTER”)–ERICH LETTERER 1924
HAND-SCHULLER-CHRISTIAN Dz–ALFRED HAND–PEDIATRICIAN 1893
EOSINOPHILIC GRANULOMA (EG) OF BONE
POLYMORPHIC RETICULOSIS
SYNONOMOUS WITH MIDLINE GRANULOMATOUS OR DESTRUCTIVE DISEASES—LEATHAL MIDLINE GRANULOMA
REALLY AND EXTRANODAL PERIPHERAL ANGIOCENTRIC T-CELL LYMPHOMA
ORBITAL PSEUDOTUMOR—-INFLAMMATORY PSEUDOTUMOR
OFTEN PRESENT AS AN ORBITAL MASS—PERIVASCULAR CUFFING
ETIOLOGY-
-POSSIBLE ETIOLOGY INCLUDE
-ORGANIC CHEMICAL EXPOSURES
-RADIATION EXPOSURE
-VIRAL ILLNESS ESPECIALLY IN IMMUNOCOMPROMISED (EBV AND HTLV-1)
-ASSOCIATED DZ WITH NON-HODGKIN’S – RA, CELIAC DZ, SJOREN’S, AND HASHIMOTO’S THYROIDITIS
NON-HODGKIN’S    -RISK STEADILY INCREASES WITH AGE
MORE AGGRESSIVE IN YOUNGER PTS

EVALUATION—FNA-HELPS TO R/O CARCINOMA
-NODE BX —IMMUNOHISTOCHEMICAL STAINS
-B-CELL LINES – CD 19 AND 20
T-CELL LINES -CD 2, 3, 4, AND 8; TERMINAL DEOXYRIBOSYLTRANSFERASE
KERATIN, MUCIN, AND S-100 NEGATIVE
-FLOW CYTOMETRY
-ELECTRON MICROSCOPY -RARELY NEEDED BUT HELPS IN UNDIFFERENTIATED DZ
-CYTOGENETICS     -BURKITT’S – CHROMOSOME 8
-FOLLICULAR – CHROMOSOME 18
-MOST DZ HAVE CHROMOSOME 14 ABNL
STAGING -WORK UP REQUIRES
-CT SCAN OF CHEST, ABDOMEN, AND PELVIS
-LABS – CBC, SMAC, B2 MICROGLOBULIN, AND LDH
-BONE MARROW ASPIRATE
-SERUM PROTEIN ELECTROPHORESIS
-GI STUDY IF SUSPECT MALT
GRADING–RAPPAPORT (MORPHOLOGY) AND LUKES-COLLINS (IMMUNOLOGY) GRADING COMBINED TO FORM THE WORKING CLASSIFICATION GRADING SYSTEM

TREATMENT—-FOR LOCALIZED H+N DZ(STAGE I)–RT ONLY
-LOW GRADE – XRT ALONE WILL CURE 50%; CHEMO NOT SHOWN TO BE BENEFICIAL
-INTERMEDIATE GRADE (STAGE IIA AND GREATER) -RT AND CHEMO WITH CHOP (CYTOXAN, DOXORUBICIN, VINCRISTINE, AND PREDNISONE) AND USUALLY XRT WILL GET 80-90% 5 YR DZ FREE SURVIVAL
-HIGH GRADE – AGGRESSIVE CHEMO BUT PATIENTS WILL RARELY PRESENT WITH LIMITED  H+N DZ
MYCOSIS FUNGOIDES
AN UNCOMMONLYMPHORETICULAR MALIGNANCY FIRST MANIFEST IN THE SKIN
TREAT IN TOPICALLY FIRST IF POSSIBLE—-NO CURE FOR LEUKEMIC PHASE

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