Tumor Miller Review



Resection

- Radical – entire muscle

- Wide – outside reactive zone (MRI)

- Marginal – through reactive zone (MRI)

- Intralesional

Staging

- Osteosarcomas present most often in stage IIB

- I – low grade

- II – high grade (B is extra-compartmental)

- III – mets

- AJCC Staging System

o Histo grade (high or low)

▪ Most important determinant for prognosis

o Size (big or small)

o Depth (deeper is worse)

- Fibrous dysplasia is sclerotic in femoral neck

Periacetabular lesions – often chondrosarcoma

Lodwick classification – radiology

- I – geographic w/ sclerotic rim, II – distinct border, III – indistinct border

Big 5 (can look like anything)

- Metastatic CA

- Cartilage lesions

- Fibrous dysplasia

- Infection

- EG

Common agents for Osteosarcoma

- MTX

- Doxorubicin – cardiotoxicity

- Bleomycin – lung prob

Chemotx

- Cells most sensitive in G1-S phase

- Bone sarcomas – Chemo, Radiation, Chemo

- Soft tissue sarcomas – Radiation

Radiation

- Dose measured in grays

o 1 rad = 1 centigray

- typically 180-200 cGy/day, 5 days a week

- if prior exposure less than 45 Gy, wound will heal

- 45-55 Gy, probably will heal

- over 65 Gy, will not heal

- radiation for round cell lesion – ewing’s, LA, Myeloma, and mets

- risk of post-radiation sarcoma 13%

o high grade lesions – poor prognosis

Molecular biology

- Chromosomal translocations – up to 95% of sarcomas

- Ewings 11, 22

- Alveolar Rhabdo 2, 13

- Synovial Sarcoma X, 18

- Myxoid Liposarcoma 12, 16

- Chondrosarcoma 9, 22

- CCSA 12, 22

- Tumor suppressor genes

o Rb – recessive suppressor

▪ Basis for all retinoblastomas

▪ 15% get other neoplasms

▪ 35% of osteosarcomas have Rb mutations

o p53

▪ mutated in 50% of all tumors

▪ dominant suppressor

▪ arrests cell cycle when DNA damage is noted

• G1 phase

▪ occurs in osteosarcoma

- Oncogenes

o Genes involved in growth of the cells

Less than 1 in 10,000 cells capable of metastasis

Bone Producing Lesions

- Osteoid Osteoma

o Young pt

o 50% long bones of LE

o PAIN from PG

▪ Inc w/ time

▪ Night pain

▪ Relieved by NSAIDs

o Nidus w/ reactive bone

o Can produce growth disturbances

o Nidus < 1.5 cm

o Always HOT on bone scan

o May spontaneously resolve in 5-7 yrs

o Need CT imaging in spine to identify

o MRI w/ extensive edema – like marrow-placing neoplasm

o Histo

▪ Sharp demarcation b/w nidus and reactive bone

▪ Variable degree of mineralization in nidus

▪ Woven bone and Osteoblastic rimming

o Tx: radiofrequency ablation

▪ 2nd tx – remove nidus

▪ if failed – then open curettage

- Osteoblastoma

o Rare, in young pt

o Pain, but not relieved w/ ASA

o Mixed lytic/blastic lesion

▪ Blastic in extremities

▪ Lytic in spine (40% occur in spine) (posterior elements)

o calcified lesion in post elements

o Larger than 2.0 cm

o HOT on bone scan

o Looks like osteoid osteoma

o Tx: marginal resection vs. extended intralesional curettage w/ adjuvant agent (5% phenol, liquid nitrogen, argon beam laser, cement)

o Recurrence 10-20%

o Histo: immature osteoid, mineralized matrix, fibrovascular stroma

- Osteosarcoma

o MC primary sarcoma of bone

o Adolescents and young adults

o Associated w/ Paget’s

o High grade intramedullary

▪ MC form

▪ 85%

▪ most present as IIB lesions

▪ 10-20% present w/ mets

• lung mets 90%

• bone mets 20%

▪ 50% occur around knee

▪ mixed lytic/blastic lesion

▪ skip lesions – need to look for

• poor prognosis (same as if w/ met)

▪ mandatory bone scan and chest CT evaluation

▪ on bone scan, hot around and photopenic in center, either is

• lesion w/ necrosis in center

• or cyst

▪ pain MC symptom

▪ Histo

• Lacy osteoid and stromal cells malignant looking

• May be giant cells, small cells

▪ Tx

• Multiagent chemo, surgery, chemo

• Necrosis of > 98% good prognostic sign

▪ Local recurrence is poor prognosis

▪ In pathologic fracture – amputation vs. limb salvage is equal survival advantage

▪ Expression of multidrug resistance gene – poor prognosis

• 25% primary lesions, 50% in metastatic lesions

o Telangectatic

▪ IM lesion

▪ Looks similar to ABC

▪ Lytic, destructive, expansile lesion

▪ Histo

• Lakes of blood

• Malignant cells

▪ Ulnar-based distal radius lesion is usu telangectactic OS (not ABC)

o Parosteal Osteosarcoma

▪ Female predominance

▪ Low grade surface lesion on metaphysis of long bones

• Distal femur 80%

▪ Formation of painless mass, firm/fixed

▪ Heavily ossified XX appearance, stuck-on, lobulated appearance

▪ Histo

• Look like fibrous dysplasia

• Bone trabeculae

• Bland spindle cells around bone

- Myositis Ossificans

o Juxtaposed to bone (not stuck on bone)

o Mineralized appearance on XX

o Mineralizes from periphery in (osteosarcoma mineralizes from in to periphery)

- Osteochondral Exostosis

o Shares the cortex (not stuck-on)

- Periosteal Osteosarcoma

o Female > male

o Diaphysis of tibia or femur

o XX w/ sunburst lesion resting in saucerized cortical depression

o Histo w/ chondroblastic appearance, intermediate to high grade

o Need chemo, resection , chemo

o Prognosis worse then parosteal, better than standard OSA

- Enchondromas

o Benign in medullary cavity, metaphysic

o Rarely symptomatic

o 60% hands and feet, 20% femur, 10% prox humerus

o pathologic hand fx common

o XX: stippled calcification, rings/arcs, popcorn calcification

▪ Rarely purely lytic

▪ If calcified lesion w/ lytic focus, then could be malignant degeneration

o Histo: small chondroid cells in lacunar spaces, myxoid, blue balls of cartilage

o Infarct is in differential – but more “smoke up the chimney”

o Observation w/ serial radiographs

o If changing, must biopsy and remove entire lesion

o Ollier’s disease – multiple lesions

▪ Risk of sarcomatous degeneration 30%

▪ Often unilateral

o Maffucci sx – multiple lesions and soft tissue angiomas

▪ High risk of malignancy (visceral malignancy)

▪ High risk of CHSA

o Histo: blue balls of cartilage

o Warm on bone scan

- Periosteal chondromas

o Rare, develops on surface of bone under periosteum

o 50% of lesions on proximal humerus

o pain common (tendon insertions irritated)

o histo similar to enchondromas

▪ looks like low-grade CHSA

o treatment: marginal excision

- Osteochondromas

o MC tumorlike lesion

o Arise secondary to aberrant cartilage on bone surface

o Painless mass

▪ May have mechanical sx

o XX: shares the cortex, IM contents flow into lesion

o 35% around knee, points away from joint

o bright on MRI T1: gad, fat, proteinaceous fluid, melanin, methemoglobin

o bright on T1 and T2

o Histo: cartilage cap 2-3 mm thick

▪ Looks like nl physis; primary trabeculae & active chondrocytes

▪ If growing in adult – then low grade OSA

▪ Beware cartilage cap > 2 cm (malign degeneration)

o Tx: observation

o Multiple hereditary osteochondromas – higher rate of malignant degeneration (into CHSA)

- Chondroblastomas

o Epiphyseal lesion of young pt (can cross physis)

o 50% in skeletally immature pt

o painful lesion

o 30% around knee, also prox humerus, prox femur

o Xx thin sclerotic rim, possible mineralization

o Histo: chicken wire calcification, cobblestone appearance

o Tx: meticulous curettage and bone grafting

▪ Recurrence 30% in some series

▪ Local adjuvant tx: phenol, cryotherapy

o 2-5% lesions metastasize to lung

- Chondromyxoid fibroma

o Rare chondroid lesion, 2nd, 3rd decade of life, males > females

o 30% knee, followed by feet, pelvis

o cortical thinning but no periosteal rx

o Pain common sx – swelling

o XX: lytic, eccentric, demarcated from bone

o Histo: stellate appearing cells, fibrous spindle cells

o Tx: curettage and grafting

▪ Recurrence up to 25% in some series

- Chondrosarcoma

o 2nd MC primary bone sarcoma

o pelvis 25%, ribs, femur, prox humerus

o pt older than 50

o Pain presenting sx

o Soft tissue component can mineralize

o XX: destructive, reactive cortical changes, mineralization

o MRI T2 – high signal, gad – high signal

o Histo: increased cellularity, multinucleated cells, enlarged cartilage cells

o Most lesions low to intermediate grade

o Lesions of axial and proximal skeleton more aggressive

o Tx: wide surgical excision

▪ Mostly chemo/radiation resistant

o Histo grade correlates w/ presence of mets

▪ Most important determinant of survival

o Dedifferentiated type

▪ 80% metastatic rate

▪ XX: superimposed widely destructive region

▪ 50% w/ pathologic fx

▪ pain is presenting feature

▪ Histo: chondroid and spindle areas (high-grade spindle component)

▪ 30% femur, 20% pelvis

▪ Tx: chemo, resection, chemo

▪ 13% survival @ 5 yrs

▪ MDR gene identified in nl and neoplastic cartilage

• Resistance to chemo

• P-glycoprotein on cell membrane

- NOF

o Found in up to 30% of skeletally immature pt

o most resolve spontaneously

o distal femur, prox tibia, dist tibia

o well-demarcated, eccentric, lobular

o bright on T2

o not hot on bone scan

o histo: spindle cells in whorled bundles, giant cells, hemosiderin

o Tx: observation if ASx, curettage/BG if Sx

o Jaffe-Campanacci Sx: multiple NOF w/ café au lait, retinal prob

- Desmoplastic fibroma

o Painful rare neoplasm in young

o Bony counterpart to desmoid

o Femur, tibia, humerus

o XX: lytic, expansile, well-defined margin

o Histo: spindle cells, swirling fibrous cells

o Low grade malignant

o Tx: aggressive curettage

- Fibrosarcoma of Bone

o Pt over 50

o 50% around knee, pelvis, prox humerus

o XX: lytic lesion w/ destructive features

o Histo: spindle cells, herringbone pattern

o Tx: wide surgical resection

▪ High grade chemo, but difficult in elderly

o High grade lesions prognosis 30% at 5 yrs

o Tx: like OSA

- Benign Fibrous Histiocytoma

o Rare

o Ilium, ribs MC, but also in tibia, femur

o XX: lytic lesion w/ sclerotic border

o More centrally located than NOF

o Pain is presenting Sx (unlike NOF)

o Histo: foamy macrophages, spindle cells, storiform pattern

o Tx: curettage and BG

- MFH

o Pt over 50

o 30% around knee, pelvis, too

o 30% felt to arise from chronic condition (infx, implant)

o w/ soft tissue component

o XX: lytic, destructive

o Histo: large, pleomorphic nuclei, storiform pattern

o Tx: chemo, resection, chemo

o Prognosis similar to OSA, 60% at 5 yrs

- Chordoma

o Malignant lesion from notochordal tissue

o Occurs at ends of spine

▪ 85% spheno-occipital and sacrum

o insidious onset of pain

o need to do rectal exam

o soft tissue mass

o CT/MRI mandatory to delineate lesion

o Histo: lobules of myxoid tissue, physaliferous cells (pink, bubbly, abundant cytoplasm w/ nuclei)

o Need to save S1, 2, 3 unilaterally or S1, 2 bilateral for nl bladder/bowel function

o Tx: wide resection, radiation

▪ Recurrence very common

o Metastatic dz in 30-50%

o 5 yr survival 60%

- Hemangioma

o 20% in spine

o often asx, may have pain

o multifocal lesions poss

o XX: lytic lesion, mild cortical expansion, trabecular striations

▪ Jail-bar vertebra

• Tumor destroys cross-trabeculae only

▪ Honeycomb appearance

o Warm to hot on bone scan

o Histo: dilated thin-walled blood vessels

o Tx: observe if asx, curettage and BG if symptomatic

▪ Low-dose radiation for inaccessible lesions

- Hemangioendothelioma/Angiosarcoma of Bone

o Malignant, rare

o Multifocal

o Pt present w/ pain

o “crawling up the bone” – consider vascular lesion

o Histo: varies

o Tx: wide resection, radiation

- Lymphoma

o Non-hodgkin’s LA

o Metastatic focus to bone

o 50% in pt over 40

o pelvis, femur, humerus, vert bodies

o pain

o soft tissue mass frequently present

o round blue cell lesion

o XX: mottled appearance, reactive bone, cortical destruction, may be blastic

▪ Involves bone diffusely (esp in pelvis)

o LCA positive (binds to CD45)

o Very hot on bone scan

o Histo: mixed round cell infiltrate

o Tx: radiation and chemo (surg rarely required)

o Stain for leukocyte common antigen, or B or T cell markers

o Fx after Bx / XRT

- Myeloma

o Plasma cell malignancy over 50 yo

o Most frequent neoplasm presenting as skeletal lesions

o Axial and proximal appendicular skeleton

o Pain, anemia, infx, renal failure

▪ 50% w/ elevated creatinine

▪ 30% w/ hypercalcemia

▪ 15% w/ amyloidosis

o XX: punched out lesions in bone, expansile appearance

o 1/3 ‘cold’ on bone scan

o M spike of serum electropherogram

▪ IgG 50%, IgA 25%

o More than 10% plasma cells on marrow aspirate

o Urine show Bence Jones protein

o Tx: chemo, radiation (surg for stabilization of bones)

o Surgery

▪ Conservative route (choose ORIF vs. hemi)

o Prog poor

- Solitary plasmacytoma

o 25% have + M-protein

o No diffuse involvement

o May go on to develop MM

o Tx: w/ radiation

o Osteosclerotic form

▪ Ass w/ polyneuropathy

▪ Lytic/sclerotic lesion

▪ POEMS sx: polyneuropathy, organomegaly, endocrinopathy, M protein, Skin changes

o Tx: radiation: neuro changes don’t improve

- Giant Cell Tumor

o Aggressive benign lesions age 20-40

o Young pt get metaphyseal, older epiphyseal

o 60% around knee, distal radius, sacrum (neuro def common)

o r/o hyperPTH (may be multicentric)

o XX: lytic, eccentric, subchondral lesion

o Histo: giant cells, mononuclear cells, reactive bone

o Bone scan – can be doughnut (bone in middle)

▪ Hot on bone scan

o Tx: aggressive exteriorization, extended curettage/burring

▪ Adjuvant tx may prevent recurrence

o PMMA reduced recurrence to 3% w/ cementation

o May metastasize to lungs – 2%

o No correlation b/w histo and clinical aggressiveness

o Radiation for inoperable lesions

- Epiphyseal lesion of bone (differential)

o GCT

o Chondroblastoma

o Clear cell chondrosarcoma

- Ewing’s sarcoma

o Pt 5-25 yo

▪ Under age 5, consider leukemia, metastatic NB

▪ Over age 30, met or LA

o 3rd MC primary sarcoma of bone

o pelvis, femur most

o in ribs, called Askin’s tumor

o XX: moth-eaten, permeative of diaphysis, metaphysic, onion-skin, sunburst appearance

o Hot on bone scan

o Soft tissue mass

o Diffuse, extension up IM canal

o 90% w/ t(11:22)

o pain, fever, leukocytosis, anemia, elevated ESR

o Histo: monotonous, smudge blue cells, pseudo-rosettes (true rosettes in NB)

▪ circles of round cells surrounding pink ground subst

o stains for intracell glycogen are +

o Mets to lung MC

o Pelvic lesions w/ poor prog, extremity lesions better

o Tx: chemo, resection, chemo (may be radiation)

o Prognosis: 60% survival

▪ 40% in pelvic lesions

▪ 15% if present w/ mets

o workup

▪ MRI, CT chest, bone scan, Bone marrow biopsy

- Adamantinoma

o Rare lesion of tibia

o 50% cases synchronously involve tibia and fibula

o pain, bowing deformity of tibia

o XX: demarcated mixed lytic/sclerotic lesion, bowing of anterior cortex

o Histo: epitheliod cells in stroma, fibrous tissue

▪ can also be columnar cells in palisading fashion

o May be continuum of osteofibrous dysplasia (Campanacci dz)

o Soft tissue mass

o Tx: wide surgical resection

o Lesion is low grade malignancy

o Lung mets in 25% cases

- ABC

o Under 20 yo

o Can arise in preexisting GCT, CBMA, CMF, Fib Dys

o Vertebra, long bones

o XX: lytic, eccentric, expansile lesion

o MRI: gad around outside (rim enhancement), dark on T1, bright T2, see fluid-fluid lines

o Hot on bone scan

o Histo: lakes of blood w/o endothelial lining

o Tx: aggressive curettage and BG

▪ Recurrence about 25%

▪ Adjuvant tx

- SBC

o Pt 3-14, cystic lesion of metaphysis

o 80% in prox humerus, prox femur in young pt

▪ older pt in ilium, talus, calcaneus

o 50% present with fracture

o caused by physeal disturbance, resorptive erosion from pressure

o Xx: central lucency, thinning of cortex

o Thinner bone than in ABC

o Active cyst abuts physis, latent cyst nl intervening bone

o Histo: thin fibrous lining, giant cells, hemosiderin

o Tx: obs/aspiration, injx/curettage and grafting

o Fallen leaf sign

o Histo: bone, fibrous lining, fluid

o 2% invade across physis

o injection w/ methylprednisolone, marrow, or bone substitute

- Histiocytosis X

o Hand-Schuller-Christian Dz

▪ Bone, visceral involvement

▪ Classic triad < 25% pt: diabetes, exopthalmus, lytic bone skull lesions

o Leterer-Siwe dz fatal in young children

- EG

o Single, or multiple bones

o Pain, swelling MC presentation

o XX: well-demarcated, lytic lesion, destroy cortex, “punched-out”

o Hot on bone scan

o Vertebra plana

▪ Vertebra will reconstitute with time (not nl appearing)

o Histo: Langerhans cell (histiocyte w/ grooved nucleus), “coffee-bean nuclei”, eosinophils, mitotic figures

o Tx: variable, self-limiting, low dose radiation or curettage, BG

o EM: racquet shaped Birbeck granules

- Fibrous Dysplasia

o Polyostotic form: LE and homolateral pelvis

▪ 20% multifocal

▪ Endocrine abn common

▪ Albright’s precocious puberty, café au lait

▪ Hyperthyroidism, Cushing’s associated

o Vert level is rare

o Xx: ground glass, lytic, well-defined sclerotic rim, expansile, angular in appearance

o Histo: no osteoblastic rimming, fibroblasts w/ dense collagen matrix, Chinese characters, woven bone

▪ osteob rimming is reactive, while no rimming is tumor

o Tx: w/ cortical bone (if cancellous bone, then fibrous dysplasia heals w/ fibrous dysplasia)

▪ Observation in most pt

▪ Graft w/ cortical allograft to prevent resorption

o Can look like anything

o MRI: bright on T2 (if cystic), bright on gad

o Not hot on bone scan

- Osteofibrous dysplasia

o Rare lesion of tibia in young pt

o Tibial bowing/prominence

o XX: anterior cortex, multiloculated

o MRI: T1 “crawling lesion”

o Histo: rimming osteoblasts, fibroblast-like spindle cells

o Tx: observation until maturity

- Paget’s

o 5th decade (3-4% population)

o often asx

o onset insidious

o 4 hallmarks

▪ coarse, purposeful trabeculae

▪ thickening of cortex

▪ enlargement of bone

▪ mixed lytic / blastic pattern

o early stage – lytic bone, late is blastic

o etiology: paramyxovirus (inclusion body in osteoclast)

o scribbled bone

o Histo: marrow fibrosis, prominent vascularity, mosaic pattern

o Pre-treat to decrease bleeding problems

o Tx: bisphosphonates, calcitonin

o Paget’s sarcoma 1-15% transformation rate

▪ < 20% 5 yr survival

▪ tx like bone sarcoma

- Metastatic Bone Dz

o over age 40

o path fx in 8-30% pt w/ mets

o breast, lung, prostate, kidney, thyroid

o lung – cookie bite out of cortex

o mets distal to elbow/knee usu lung or renal

o Histo: glandular pattern

o Renal is more destructive than lung

▪ They bleed (also myeloma and thyroid)

o Thyroid and renal cell may be cold on bone scan

o Tx: to maintain skeletal integrity

▪ In hip fx, go aggressive (choose hemi vs. ORIF)

o Use bisphosphonates, PMMA, radiation as adjuncts

o Batson’s plexus

▪ Valveless system

▪ Retrograde embolism from breast, prostate, lung, kidney, thyroid

o stain positive for cytokeratins

- Osteomyelitis

o Minimal osteolysis w/ surrounding sclerosis

o Sequestrum – dead bone

o Involucrum – reactive bone around it

o Histo: granulation tissue, mixed cell pop of inflam cells, poly, lymphocytes

o Chronic infx may present w/ SCC

o Fungal osteomyelitis – Langhans cell (multinucleated cell ass w/ spores)

o Ass w/ squamous cell CA (chronic osteomyelitis)

Sarcomas

- radiation carries 20% risk of major wound compl

- Calcifying aponeurotic fibroma

o Young children

o Benign

o Hands and feet

o Tx: local excision (recurrence common)

- Fibromatosis

o Firm nodules fibroblasts

o Nodules are painful early, contractures late (Dupuytren’s)

o Lederhosen’s in feet

o Peyronies in penis

- Extra-abdominal Desmoid Tumor

o Most locally invasive of all soft tissue tumors

o Cold on bone scan

o Recurrence common

o XRT effective adjuvant tx

o high rate of recurrence

o Prognosis good

o Histo: dense fibrous tissue, spindle cells, looks like scar

- MFH

o Pt 30-80 in age

o Enlarging, painless mass

▪ Large enough > 10 cm, then sx

o MRI: inhomogeneous mass

o Histo: storiform spindle cells

▪ Fibrosarcoma – herringbone pattern

o Tx: wide resection, radiation (pre and post)

o Prognosis poor

▪ Survival 5 yr 50%

▪ Mets present in 30%

- Dermatofibrosarcoma protuberans

o Nodular cutaneous tumor – early adult life

o Intermediate grade

o Grows slowly but progressively

o 40% upper or lower ext

o Histo: uniform fibroblasts, storiform pattern

o Tx: wide resection

▪ Margin > 3 cm w/ lower recurrence

▪ Adjuvant XRT consider

- Lipomas

o Slowly growing, painless

o MRI shows fat

o Tx: observation, resection if symptomatic

o atypical lipoma

▪ lobules of fat on MRI

▪ different b/c in liposarcoma, lobules not fatty

▪ 10% risk of transformation to liposarcoma

- Lipoblastomas

o In kids

o Immature fat – does not follow fat MRI signal

- Liposarcomas

o Range from low grade to high

o Imaging shows ‘stranding’ through mass

o Higher grade lesions metastasize to retroperitoneum

o Histo: more fibrous stromal background, has some fat in it, large/clear cytoplasm

o Not fat density or fat signal

o Tx: wide excision, radiation

o Prognosis – 5 yr survival rates

- Neurilemoma/Schwannoma

o Benign nerve sheath lesion of adults 20-50

o Asx x for presence of mass on flexor surfaces

o Lesion may wax and wane in size

o MRI eccentric mass to nerve

▪ On gad – mixed signal

o Histo: antoni A – compact spindle cells, nuclear palisading, verocay body

▪ Antoni B – haphazard matrix w/ delicate collagen, irr vessels

o Tx: removal of mass, leave nerve intact

o Prognosis excellent

- Neurofibroma

o Superficial or deep, painless

o Histo: wavy, crinkled elongated cells, neural elements

o Xx: scalloping from without (from within is fibrous dysplasia)

o Tx: marginal excision

▪ Cannot remove lesion if it sacrifices nerve

o NF

▪ AD

▪ Peripheral type 1, central type II

▪ Malignant transformation in 5%

- Neurofibrosarcoma

o Rare malignant nerve lesion

o Usu from NF pt

o Enlarging mass

o Histo similar to fibrosarcoma

o Histo: cigar-shaped nuclei, spindle cells

o Tx: resection, radiation (check frozen section on nerve to clear margin)

o Prognosis poor (worse in NF pt)

- Leiomyosarcoma

o Small, nodular, low grade

o Prognosis poor

o Tx: aggressive resection, radiation

o Histo: spindle cells, cigar-shaped nuclei, smooth muscle

- Rhabdomyosarcoma

o MC pediatric soft tissue sarcoma in children < 10

o Highly malignant lesion

o Very responsive to chemotherapy

o Alveolar subtype MC in extremities

o Histo: spindle cells, racquet shaped cells

o Embryonal rhabdo – look like round cell appearance, loose cytoplasm

▪ Most are (65%)

▪ Most in kids

▪ Cross-striations

o Tx: wide resection, chemo

o Pleomorphic rhabdo – malignant appearing histo

- Hemangioma

o Common, can be anywhere

o Dull pain, wax/wane in size

o MRI: fatty infiltration, heterogeneous lesion

o Mineralizes when it grows close to bone

o very high rate of recurrence

o Tx: observation, NSAIDs

▪ Recurrence is high w/ surgery

▪ Percutaneous sclerosing w/ alcohol popular (but painful)

- Angiosarcoma

o Rare, ass w/ endothelium of blood vessels

o Tx: amputation (no radiation or chemo)

- PVNS

o 2nd-4th decades of life

o Nodular or diffuse

o Knee MC, hip, shoulder

o Joint w/ bloody effusion

o XX: erosive changes on both sides of jt

o Histo: plump synovial cells, giant cells, inflam cells, stains w/ hemosiderin

o Tx: complete excision, synovectemy

o Recurrence is a problem

o Leads to arthroplasty

o Histo: hemosiderin

- GCT of tendon sheath

o Looks just like PVNS

- Synovial chondromatosis

o Benign metaplasia of synovium

o Nodules of cartilage

o Young adults – pain, stiffness

o XX: mineralized nodules

o MRI: jt full of nodular lesions

o Tx: excision of nodules, but lesions may burn out over time

o Histo: chondroid tissue w/ synovial tissue around it

- Synovial sarcoma

o Arises near but rarely from jt ................
................

In order to avoid copyright disputes, this page is only a partial summary.

Google Online Preview   Download