Orthopaedic Tumor Matrix

Metastatic Carcinoma
Age	>40
Clinical Presentation	hx cancer, bone pain
X-Ray	lytic = lung, renal; mixed = breast, thyroid; blastic = prostate; anterior column spine, prox bones
CT	path fx
MRI	path fx, edema
Histology	glandular islands of epithelial cells in fibrous background, cytokeratin +
Rx	IM Rod; Mirel's; bisphosphonates; preop emboliz renal
Syndromes	25% mets present with pathologic fx

Renal Cell Carcinoma Metastasis
Age	>40
Clinical Presentation	hx cancer, bone pain
X-Ray	lytic = lung, renal; mixed = breast, thyroid; blastic = prostate; anterior column spine, prox bones
CT	path fx
MRI	path fx, edema
Histology	glandular islands of epithelial cells in fibrous background, cytokeratin +
Rx	IM Rod; Mirel's; bisphosphonates; preop emboliz renal
Syndromes	25% mets present with pathologic fx

Myeloma
Age	50-80
Clinical Presentation	bone pain (esp. spine/ribs); path fx; fatigue (anemia); renal disease; hypercalcemia
X-Ray	punched out lytic lesions; osteopenia
CT	lytic lesions
MRI	extent of disease
Histology	sheets of plasma cells
Rx	prednisone/alk agent; bisphosphonates; RTX; BMT; stabilize fx
Syndromes	SPEP - double protein spike ("Goal post" sign); M (whole IgG>IgA>>IgM) &/or Bence-Jone (light chain only) proteins in >99% pts; 50% die of renal failure; 18-24 month survival

Lymphoma
Age	>40
Clinical Presentation	bone pain; path fx; fatigue
X-Ray	long lesion; mottled; reactive and destructive; cortex thickened
CT	path fx
MRI	large soft tissue masses
Histology	most are large B cell, mixed cell infiltrate
Rx	chemo and RTX (multiple sites), RTX (solitary), surgery to stabilize fx
Syndromes

Chondrosarcoma
Age	30-70
Clinical Presentation	pain and/or mass in shoulder, pelvis, knee, spine
X-Ray	primary metaphyseal radiolucent with calcification; secondary osteochondroma with large >2cm soft tissue mass with calcification; axial skeleton; 85% cortical changes
CT	calcification in radiolucent lesion
MRI	soft tissue mass
Histology	malignant cartilage formation; 75% low grade
Rx	wide resection; RTX/chemo resistant
Syndromes

MFH
Age	20-60
Clinical Presentation	pain or mass; distal femur; prox tib/femur/hum, ilium
X-Ray	extensive lytic/blastic creates mottled bone
CT
MRI	extent of soft tissue
Histology	giant cells, storiform, "cartwheels", chronic inflammatory cells
Rx	wide resection and chemo; pulm mets in 30-40% = RTX
Syndromes	#1 sarcoma to complicate a preexisting condition (paget's, bone infarct, radiation osteitis, etc.)

Fibrosarcoma
Age	20-60
Clinical Presentation	pain or mass
X-Ray	"moth eaten"
CT
MRI	extent of soft tissue
Histology	spindle cells, collagen, ""herringbone pattern"" (fascicles cut transversely and sagittaly)
Rx	wide resection and chemo; pulm mets in 30-40% = RTX
Syndromes

Hyperparathyroidism
Age	20+
Clinical Presentation	stones, moans, groans, abdomenal overtones (PUD, pancreat), pth, Ca++
X-Ray	bone resorption, lytic, phalanges
CT
MRI
Histology	tunneling resorption b/c osteoclasts cannot penetrate unmineralized osteoid on cortex
Rx	gland resection, bisphosphonates
Syndromes

Osteonecrosis (AVN)
Age	any
Clinical Presentation	joint pain, effusion, weight bearing pain
X-Ray	periarticular, Ficat, crescent sign, collapse, OA
CT	look for reactive bone, collapse
MRI	MRI detects before pain (ficat), cartilage changes
Histology	empty osteocyte lacunae, +/- marrow necrosis
Rx	observation, core decompression, arthroplasty
Syndromes

Bone Infarct
Age	any
Clinical Presentation	incidental
X-Ray	metaphysical radiodensity
CT	calcification
MRI	necrosis, calcification
Histology	empty osteocyte lacunae, +/- marrow necrosis
Rx	observation
Syndromes

Paget's Disease
Age	>40
Clinical Presentation	pain; axial > appendicular
X-Ray	hot phase (flame shaped lucency meta to diaph); intermed phase ("cotton wool" pattern), cold (coarse trabeculae and bony enlargement")
CT	thickened cortex
MRI	no soft tissue involvement
Histology	coarsened trabeculae (blastic appearance); cement lines, osteoclastic, fibrous vascular tissue between trabeculae; measles inclusion bodies (electron microscopy)
Rx	bisphosphonates, calcitonin, methotrexate
Syndromes

Enostosis (Bone Island)
Age	>40
Clinical Presentation	no pain
X-Ray	radiodensity in medullary canal
CT	pseudopodia
MRI	no soft tissue involvement
Histology	cortical bone surrounded by medullary bone
Rx	observation
Syndromes

Osteosarcoma
Age	10->20
Clinical Presentation	pain, swelling, fracture, mass
X-Ray	knee metaphysis, lucent/blastic with cortical destruction, stage IIB at presentation, codman's, sunburst
CT	pathologic fracture +/-
MRI	usually extracompartmental
Histology	spindle shaped tumor cells that produce osteoid; lace-like osteoid
Rx	preop chemo 12 weeks, restage, wide resection, 6-9 months adjuvant; 60% survival
Syndromes

Ewing's Sarcoma
Age	5->30
Clinical Presentation	pain; +/- fever; increased ESR/WBC; anemia
X-Ray	pelvis, distal femur, prox tibia/humerus; femoral diaphysis, large destructive metaphy/diaphy lesion, radiolucent with reactive new bone, onion skin
CT	onion skin/periostel rxn
MRI	soft tissue component
Histology	small round blue cell tumor; t(11,22); fusion protein EWS-FLI 1
Rx	chemo, RTX, surgical resection; 60-70% survival
Syndromes	poor px: spine and pelvic tumors, tumors greater than 100 cm3, poor response to chemo (<90% necrosis), elevated LDH

Osteomyelitis
Age	any
Clinical Presentation	pain, fever, constitutional sx, redness, swelling, chronic wound drainage, bone exposed in wound
X-Ray	early periosteal rxn, late lucent or rxn thickened traebeculae
CT	cortical breech
MRI	target lesion, edema
Histology	inflammatory cells, necrosis
Rx	irrigation, debridement, antibiotic beads or spacers
Syndromes	cultures

Eosinophilic Granuloma (HistioX)
Age	5->10
Clinical Presentation	pain, great imitator
X-Ray	well circumscribed, radiolucent, reactive rim of bone, vertebra plana spine
CT	evaluate multiple locations… Hand Schuller Christian Disease (all over viscera, lytic skull lesions, exophthalmus)
MRI	no soft tissue extension
Histology	langerhan's cell histiocyte with coffee bean shape nuclei, multinucleated giant cells, eosinophils
Rx	methylprednisolone acetate; systemic form Rx with chemo; brace spine
Syndromes

Osteoid Osteoma
Age	0-20
Clinical Presentation	painful joint, relieved with salicylates, night pain, insidious
X-Ray	intensely reactive bone, radiolucent nidus <1.5cm, well circumscribed, cortical
CT	same as x-ray
MRI
Histology	distinction between nidus and reactive bone; nidus = osteoid trabeculae with greatest mineralization in center
Rx	observation, CT guided RF ablation, or excision
Syndromes

Osteoblastoma
Age	0-30
Clinical Presentation	(same as osteoid osteoma) painful joint, relieved with salicylates, night pain, insidious
X-Ray	radiolucent nidus >1.5cm, well defined, fluffy mineralization, bone destruction without characteristic bone formation as in osteoid osteoma
CT	same as bone
MRI	not as much bone formation or edema as osteoid osteoma
Histology
Rx	osteoblastic rimming of trabeculae
Syndromes

Chondroblastoma
Age	0-20
Clinical Presentation	joint pain, swelling; distal femur, prox tibia/humerus, triradiate cartilage
X-Ray	open physes, epiphysis, eccentric, apophysis, radiolucency with rim of reactive bone
CT	same as x-ray, evaluate for fx/intraarticular
MRI	no soft tissue extension of mass; soft tissue edema if fracture, joint effusion if intraarticular
Histology	chondroblasts, cobblestoning, chicken wire calcification, giant cells
Rx	currettage, phenol, sterile water, bone graft, hydrocet (90% success)
Syndromes	2% benign chondroblastomas metastasize to lung; chromosomes 5 and 8 may have genetic abnormalities

Chondromyxoid Fibroma
Age	10->20
Clinical Presentation	pain (months to years)
X-Ray	long bones (esp tibia); lytic; eccentric; no matrix mineralization
CT
MRI
Histology	chondroid, fibromatoid, myxoid
Rx	resection
Syndromes	chromosome 6 at q13 position

Osteochondroma
Age	>10
Clinical Presentation	painless mass, after trauma
X-Ray	surface lesion, cortex of lesion continuous with bone cortex, medullary canal flows into it, pedunculated, sessile
CT	same as x-ray
MRI	no edema, fx or soft tissue changes
Histology	bone covered by cartilage cap
Rx	observation; <1% chance of conversion to sarcoma
Syndromes	malignant transformation signs: mineralization of soft tissue mass, subchondral bone destruction, inhomogenous appearance… usually chondrosarcoma

Enchondroma
Age	20-50
Clinical Presentation	painless, incidental x-ray after trauma
X-Ray	lucent area in medullary canal; calcifications inside
CT
MRI
Histology	cartialge in bone
Rx	observation
Syndromes	Ollier's - multiple enchondromas and skeletal dysplasia

NOF
Age	0-20
Clinical Presentation	painless, incidental x-ray after trauma
X-Ray	metaphyseal eccentric fibrous cortical defect scalloped long bones
CT
MRI
Histology	cellular, giant cells, lipophages, storiform spindle cells
Rx	observation (currettage/graft if >75% cortex or painful)
Syndromes	Jaffe-Campanacci Syndrome - multiple NOF, café au lait, endocrine abn

Fibrous Dysplasia
Age	0-30
Clinical Presentation	monostotic or polystotic; café au lait spots
X-Ray	any bone; ground glass; lucency; rim of reactive bone
CT
MRI
Histology	fibroblasts; osteoid and bone within fibrous stroma; no osteoblastic rimming
Rx	observation; surgical stabilization for impending fx; currettage and graft if symptomatic
Syndromes	McCune Albright - polyostotic FD, café au lait, endocrine

UBC
Age	0-20
Clinical Presentation	pain from path fx from minor trauma prox humerus most common/prox femur
X-Ray	central lucency; symmetric / thinning of cortex;"fallen leaf"
CT	bone expanded but not usually greater than physis; +/- path fx
MRI	fluid
Histology	thin fibrous lining; lining has fibrous tissue, giant cells, hemosiderin, infl cells
Rx	aspirate to confirm; methyl prednisolone acetate injection; currettage/graft if recalcitrant
Syndromes	? etiology; possibly from disturbing physis

Aneurysmal Bone Cyst
Age	0-30
Clinical Presentation	pain/swelling months to years
X-Ray	eccentric, lytic, expansile, metaphysis, +/- path fx
CT	thin rim of periosteal new bone; +/- path fx
MRI	fluid/fluid
Histology	cavernous blood filled spaces without endothelial lining; giant cells; thin strands of bone in fibrous tissue
Rx	curettage and bone grafting; local recurrence common w/ open physes
Syndromes	in association with GCT, CB, CMF, FD

Giant Cell Tumor
Age	20-50
Clinical Presentation	pain referred to joint
X-Ray	radiolucent eccentric; metaphyseal/epiphyseal; knee (50%); vertebrae/sacrum (10%); uncommon w/ open physes
CT	benign bone tumor with mets to lung
MRI	reactive rim of bone; secondary necrosis or ABC
Histology	Giant Cells; secondary ABC/necrosis/reactive new bone
Rx	currettage, phenol, sterile water, bone graft, hydrocet (90% success)
Syndromes	9% of GCT metastasize to lungs

Chordoma
Age	>50
Clinical Presentation	pelvic or LBP, hip pain, GI symptoms; 50% found on rectal exam
X-Ray	sacrum and spheno-occipital region; 10% in vertebral bodies
CT	midline bone destruction, sacrum expanded, distinct lobules, vertebral mixed pattern
MRI	soft tissue mass irregular mineralization
Histology	notochordal tissue; physaliferous cells
Rx	wide surgical excision, RTX if not wide surgical excision
Syndromes	30-50% cases metastasize; mortality due to local extension

Adamantinoma
Age	30-50
Clinical Presentation	months to years of pain
X-Ray	tibia most common; multiple, sharply circumscribed lucent defects w/ sclerotic bone interspersed between; pathologic fx
CT	path fx
MRI	path fx
Histology	epithelial-like islands in background of FD; palisading or glandular
Rx	wide surgical excision
Syndromes	may metastasize either early or after multiple failed attempts at local control

Osteofibrous Dysplasia
Age	<10
Clinical Presentation	pain
X-Ray	ground glass; diaphysis; eccentric; tibia confined to cortex, bowing, +/- path fx
CT	radiolucency
MRI	no soft tissue extension; edema if fx
Histology	bx not needed; fibrous tissue in background of bone trabeculae with osteoblastic rimming
Rx	nonop until skeletal maturity
Syndromes

Neuroblastoma
Age	<5
Clinical Presentation	abd/bone pain, constitu sx; urine catachol
X-Ray	multiple lytic lesions
CT
MRI	adrenal mass
Histology	Homer-Wright pseudorosettes, neuropil
Rx
Syndromes	hepatomegaly; blanching subcutaneous nodules; or a large, irregular, firm abdominal mass; Homer-Wright pseudorosettes clusters neuroblasts surrounding areas of eosinophilic neuropil (neuritic process)

Soft Tissue - Vascular

Angiosarcoma
Age	20-70 bone; >50 skin
Clinical Presentation	mass, pain, skin changes/wound
X-Ray	soap bubble lesions; lytic mass irregular
CT
MRI	decr T1; incr T2; soft tissue extension
Histology	endothelium of blood vessels
Rx	amputation, pulm mets common
Syndromes	soft tissue and bone forms

Hemangioma
Age	any
Clinical Presentation	pain or path fx
X-Ray	phleboliths; radiolucent; coarsened honey-comb; vertebral bodies
CT	irregular blood vessels within lesion
MRI	cavernous (most common), capillary, or mixed
Histology	blood channels
Rx	alcohol injection; stockings; wide resection (recurrence common)
Syndromes

Soft Tissue - Skeletal Muscle

Rhabdomyoma
Age	<20
Clinical Presentation	mass
X-Ray	neg
CT	neg
MRI	mass in muscle
Histology	muscle without pleomorphism or atypia
Rx	resection
Syndromes

Rhabdomyosarcoma
Age	<20
Clinical Presentation	mass, pain
X-Ray	neg
CT	neg
MRI	heterogeneous mass in muscle
Histology	spindle cells in parallel bundles; giant cells; racquet shaped cells; rhabdomyoblasts (cross striations within the tumor cells)
Rx	preop chemo, wide resection, RTX
Syndromes

Soft Tissue - Smooth Muscle

Leiomyoma
Age	20-60
Clinical Presentation	nodule, pain, arise from pilar erector muscles; extensor surfaces
X-Ray	neg
CT	neg
MRI	mass in skin, sharp demarcation
Histology	spindle cells and cigar shaped nuclei
Rx	excision; recurrence after excision 50%
Syndromes

Leiomyosarcoma
Age	20-60
Clinical Presentation	small or large extremity mass
X-Ray	neg
CT	neg
MRI	may or may not be associated with vessels
Histology	leoimyoma w/ pleomorphism, atypia
Rx	preop RTX; resection; postop RTX
Syndromes

Soft Tissue - Fibrohistiocytic

Benign Fibrous Histiocytoma
Age	20-50
Clinical Presentation	small slow growing painless mass; dermatofibroma
X-Ray	neg
CT	neg
MRI	skin mass superficial
Histology	fibroblasts, histiocytes, storiform
Rx	excision, recurrence is uncommon
Syndromes

Dermatofibrosarcoma Protuberans
Age	20-50
Clinical Presentation	slow growing skin mass through skin, horizontal spread
X-Ray	soft tissue mass
CT	soft tissue mass
MRI	determines depth and invasion of tumor
Histology	fibroblasts, storiform, infiltrative, central portion with uniform plump fibroblasts, CD34
Rx	chemo, resection, RTX if margins positive
Syndromes

MFH
Age	10-90
Clinical Presentation	mass
X-Ray	soft tissue mass
CT	soft tissue mass
MRI	heterogeneous intramuscular mass; calcification, necrosis, hemorrhage
Histology	fibroblasts, histiocytes, anaplastic, pleomorphism
Rx	wide or radical excision
Syndromes

Soft Tissue - Adipose

Lipoma
Age	any
Clinical Presentation	long standing mass, no pain
X-Ray	soft tissue mass
CT	soft tissue mass
MRI	sub-Q; IM or intermusc mass; well demarcated lesion with same signal as mature fat
Histology
Rx	observation or excision
Syndromes

Liposarcoma
Age	>40
Clinical Presentation	painful mass
X-Ray	soft tissue mass
CT	soft tissue mass
MRI	lipoblasts (signet ring-type cells); low to high grade
Histology	preop RTX; excision; postop RTX
Rx	well differen liposarc <10% mets; 10-30% intermed grade;>50% high grade
Syndromes

Soft Tissue - Fibrous

Nodular Fascitis
Age	30-40
Clinical Presentation	fast growing lesion in subQ fascia or deep fascia; forearm common; numb if near nerve
X-Ray	neg
CT	soft tissue mass
MRI	mass
Histology	"reactive pseudosarcoma"; nodular nonencapsulated mases, high mitosis
Rx	excision, few recurrences
Syndromes

Deep and Superficial Fibromatosis
Age	juvenile vs. adult
Clinical Presentation	mass, flexion contracture of figures, dupuytren
X-Ray	neg
CT	soft tissue mass
MRI	mass
Histology	fibroblasts, myofibroblasts, collagen
Rx	observation, surgical excision of the abnormal fibrous tissue; RTX/chemo to reduce recurrence rate
Syndromes	Lederhosen's disease (sole of foot) and Peyronie's Disease (penis)

Fibrosarcoma
Age	35-55
Clinical Presentation	enlarging painless mass extrem/trunk
X-Ray	neg
CT	soft tissue mass
MRI	mass
Histology	vimentin, spindle cells, herringbone
Rx
Syndromes	infantile fibrosarcoma - for less than one year old, most common sarcoma

Soft Tissue - Synovial

Synovial Chondromatosis
Age	30-50
Clinical Presentation	knee pain, swelling
X-Ray	radiodensities (cartilage nodules)
CT	loose bodies in knee
MRI	cartilage lesions growing out of synovium
Histology	hyaline nodules may undergo ossification and become loose bodies growing out of synovium
Rx	open or arthroscopic total synovectomy and removal loose bodies
Syndromes

PVNS
Age	20-40
Clinical Presentation	knee involvement 80% in a 20-40 year old
X-Ray	lytic lesion on one or both sides of joint
CT	lytic lesion on one or both sides of joint
MRI	numerous foci of signal dropout "dark" on T2
Histology	mononuclear cells, hemosiderophages, giant cells, foam cells deep to synovial lining (dark brown synovitis)
Rx	open or arthroscopic total synovectomy recurrence 5-10%
Syndromes	rheumatoid arthritis of a single joint; hemosiderin staining

Ganglia
Age	20-60
Clinical Presentation	mass assoc w/joint, transilluminate
X-Ray	DJD of affected joint
CT	same as x-ray
MRI	fluid filled cyst assoc with joint
Histology	synovium; hyaluronic acid in cyst
Rx	aspirate and steroid injection, high recurrence rate; surgery w/ excision better
Syndromes

Synovial Sarcoma
Age	20-40
Clinical Presentation	mass near knee (most common), most common sarcoma of foot
X-Ray	calcification; biphasic; epithelial with fibrous/spindle component
CT	may detect secondary bone involvement
MRI	low T1, high T2 soft tissue mass, homogenous unless calcification present
Histology	S100, vimentin, CD99; cell origin unknown; (most are monophasic (only epithelial or spindle cells) and mistaken for fibrosarc
Rx	preop chemo; wide or radical resection; postop RTX
Syndromes	t(X,18); fused SSX-SYT gene; <10% intraarticular

Soft Tissue - Peripheral Nerves

NF
Age	any
Clinical Presentation	most superficial, slow growing, painless (if pain think sarcoma)
X-Ray	neg
CT	neg
MRI	PNST
Histology	interlacing elongated cells with wavy dark nuclei; wire-like collagen strands
Rx	marginal excision
Syndromes	neurofibromatosis - café au lait spots, variable skeletal abnorm (NOF, scoliosis, bowing); 5-30% malignant change

Schwannoma
Age	20-50
Clinical Presentation	asymptomatic mass
X-Ray	neg
CT	neg
MRI	eccentric mass from peripheral nerve; high on T2/low T1
Histology	Antoni A/B; Verocay bodies
Rx	excise mass while leaving nerve intact
Syndromes

Malignant PNST (malginant schwannoma, neurofibrosarcoma)
Age	20-40
Clinical Presentation	painful mass; neuro symptoms
X-Ray	neg
CT	neg
MRI	>5cm diameter (most)
Histology	spindle cells resembling schwann; hyaline nodules, cartilage/bone islands, mitotic figures
Rx	excise mass +/- RTX/chemo if >5cm or aggressive
Syndromes

Glomus Tumor
Age	any, more common adults
Clinical Presentation	subungual mass; nail ridging; cold intolerance; pain; exquisite TTP
X-Ray	50% erosions in distal phalanx
CT	same as x-ray
MRI	well circum nodules, fibrous surrounding; vascular; glomus cells
Histology	perivascular proliferation
Rx	marginal excision
Syndromes	the glomus cells are monomorphous round or polygonal cells with plump nuclei and scant eosinophilic cytoplasm

Miscellaneous

Clear Cell Sarcoma / Amelanotic Melanoma
Age	15-35
Clinical Presentation	slow grow, foot and ankle mass
X-Ray	neg
CT	neg
MRI	mass assoc w/tendons, aponeuroses
Histology	nests polygonal round clear cells fibrous septae divide; giant cells, S100
Rx	wide resection, postop RTX
Syndromes	t(12,22)

Alveolar Soft Parts Sarcoma
Age	15-35
Clinical Presentation	slow growing painless mass anterior thigh
X-Ray	neg
CT	neg
MRI	soft tissue mass anterior thigh common
Histology	dense fibrous divides tumor into nests; large round cells; vascular
Rx	wide resection, postop RTX
Syndromes