Tumor Chart

Benign bone lesions

	Site	Size	To Bone	Bone response	Matrix	Cortex	Soft Tissue	Age	Class	Pathology	DDx	Other	Rx
Osteoid Osteoma	?Long bones & post elements	Nidus < 1cm, if > 2cm = osteoblastoma	(subperiosteal = ±scalloping)	Cortical/medullary = intense sclerosis	± calcified central nidus	thickened	none	10 to 35	Benign active	Osteoid with fibrovascular tissue between trabeculae	Stress #, Brodie's abscess, osteoblastoma, bone island (cold bone scan)	Pain relieved by NSAIDs, scoliosis, hot bone scan	None, NSAIDs, curettage, ablation, en bloc
Osteoblastoma	?post elements, met/dia of long bones, skull	> 2 cm, progressive	Usually lytic, also sclerotic, resemble ABC if lytic	Geographic, sclerotic rim, ±neocorticalization	Poorly visible osteoblastic, bone if small	Aggressive can erode	Neocorticalization if present	10 to 35	Benign aggressive	Trabeculae in fibrovascular stroma, may coexist with GCT or ABC	OO, ABC (esp. spine), GCT, Brodie's, osteosarc, chondrosarc, chondroblastoma (epiphyseal)	Hot bone scan, difficult to differentiate from osteosarc	En bloc or curettage/bone graft/cement, (RR = 10 – 20%)
Osteoma	Bone surface, calvarium	Variable	Focal, dense, homogenous bone, geographic	None	Sclerotic	Smooth, merge	none	Dx in 5^th dec	Benign hamartomatous	Heavily ossified tissue	Parosteal osteosarc, osteochondroma, periostitis, met	Assoc. with Gardner's synd (auto dom, intestinal polyposis, ST fibromas, sebaceous cysts) ? fibrosarc, ampulla of Vater carcinoma, thyroid Ca	Excise if symptomatic
Osteochondroma ("exostosis")	Metaphyseal, knee, shoulder, hip	variable	Metaphysis may be expanded and remodeled	Remodelling	Enchondral ossification of cartilage cap, cartilaginous matrix prior to maturity	Contiguous marrow cavity, grows away from epiphysis	Non-ossified cartilage cap >1 or 2 cm in an adult suggests malignant transformation (chondrosarcoma)	10 to 35	Benign latent, parosteal or surface lesion	Cartilage cap matures into trabecular bone	Juxtacortical myositis ossificans, periosteal chondroma, surface or soft tissue osteosarcoma	Assoc. with familial osteochondromatosis, MRI shows thickness of cartilage cap, CT shows contiguous marrow, malignant transformation risk 1% for solitary, higher more proximal	Asymptomatic = no treatment, excise if painful or NV compression, recurrence rate if immature
Enchondroma	Metadiaphysis, femur, humerus, diaphyseal in hand (prox phal > MC > mid phal > dist phal	3 to 5 cm, smaller in hands and feet	Minor endosteal erosion, hand lesions may be quite aggressive but are still benign	Geographic, little endosteal response	Popcorn calcification in long bones, radiolucent in hand and foot	Cortical erosion usually only in hand and foot	None, rarely enchondroma protuberans in hand	15 to 40	Benign latent, except pathologic # in phalanges	Well-circumscribed lobules of cartilage	Medullary bone infarct, GCT in metacarpals, fibrous dysplasia, UBC, chondrosarcoma	Assoc. with Ollier's and Maffucci's, worrisome = endosteal scalloping, permeative lucency, rare malignant transformation	Biopsy if change with time, persistent pain, worrisome Xray features. Xray @ 3, 6 12 months. Curettage & BG
Chondromyxoid fibroma	Proximal tibia, distal femur, metaphyseal, eccentric	3 to 10 cm	radiolucent	Geographic, sharp sclerotic margin	Not usually seen	Cortical erosion or ballooning	None	10 to 30	Benign aggressive cartilage lesion	Firm, tan tissue with lobular pattern, myxoid or chondroid matrix	GCT, ABC, UBC, nonossifying fibroma, chondroblastoma, infection, osteoblastoma, Brown tumour, telangiectatic/chondroblastic osteosarcoma		Biopsy, curettage & BG or marginal excision, recurrence < 20%
Chondroblastoma	Epiphyseal/apophyseal, femur > humerus > tibia > pelvis > tarsal bones	Variable	Lytic lesion with geographic margin	Sclerotic rim of reactive bone	Lytic, possible internal cartilaginous calcifications	Expansion if metaphyseal	None	5 to 25	Benign aggressive giant cell and chondroid type lesion	Proliferating chondroblasts, chondroid matrix (pink not blue), numerous giant cells	Degenerative joint cyst, enchondroma, clear cell chondrosarcoma, GCT	GCT is usually epiphyseal-metaphyseal and has no calcifications, no ossifications, and no sclerotic margin	Thorough curettage & BG, recurrence 10 to 30%
Periosteal chondroma	Eccentric, subperiosteal, external to cortex, metaphyseal cortex of long bones, esp. prox humerus, tibia, femur, and small tubular bones of hands and feet	Variable	Periosteal, causes cortical erosion	Well defined, shallow, geographic, peripheral periosteal elevation and reaction	Radiolucent, scattered calcifications	Cortical erosion	always	2^nd to 4^th decades	Benign active, slow growing, rare	Hyaline cartilage, nodules on surface of the cortex	Periosteal chondrosarcoma or osteosarcoma, adjacent soft tissue neoplasm		En bloc resection, narrow margin
Synovial chondromatosis	Periarticular, intra-articular loose bodies, knee >> hip, shoulder, elbow, ankle	Multiple calcified bodies, mm to cms	Degenerative arthritis	Mild reactive sclerosis from bone erosion	Cartilage +/- calcification/ossification	N/A	Extension into bursae around distended joints	3^rd to 5^th decades	Benign cartilaginous lesion	Nodules of chondroid tissue both free and within synovium	Loose bodies: secondary osteochondromatosis, osteochondral #, OA, Charcot, osteochondritis dessicans		Remove loose bodies, total synovectomy
Fibrous cortical defect	Distal femur and tibia, eccentric, cortical, metaphyseal	< 4cm	Radiolucent, elliptical	Sharp demarcation, thin sclerotic margin	none	Entirely a cortical lesion	None	Age 4 to 8	Benign latent	Cortex replaced with fibrous tissue, spindle cell proliferation, storiform pattern, giant cells, ++iron (Prussian blue)	Infection, chondromyxoid fibroma, fibrous histiocytoma, nonossifying fibroma	Symptomatic almost exclusively in children, spontaneously resolves with cessation of growth	Observation, 3,6,12 months if lesion atypical
Nonossifying fibroma	Tibia and femur, expands into medullary cavity	> 4cm	Fracture risk as enlargens	Thin, sclerotic, lobulated margin	None	Expanded and thinned	None	<30	Benign latent or active	Identical to fibrous cortical defect	ABC, fibrous dysplasia, chondromyxoid fibroma	Arises from a fibrous cortical defect that enlarges into metaphysic, most lesions spontaneously heal	Curettage & BG +/- ORIF if: atypical location, questionable diagnosis, intractable pain, impending path #
Fibrous dysplasia - monostotic	Ribs > femur > tibia > maxilla > mandible > skull > humerus, usually spares epiphysis but can be throughout bone	Variable, can involve entire bone	Lytic to ground glass, endosteal expansion	Well-demarcated sclerotic border, limited periosteal response	Osseous matrix if present can produce ground glass appearance	Endosteal erosion	None	Late childhood into adulthood	Benign latent, active, or aggressive	Cellular fibrous tissue with immature woven bone "Chinese character" shapes	UBC, nonossifying fibroma, solitary EG, ABC, GCT, hemangioma, infection, central low-grade osteosarcoma, fibrosarcoma, Ewing's	Fibrous dysplasia is the third most common cause of secondary osteosarcoma arising in diseased bone after Paget's and radiation	Biopsy may be required for diagnosis, bracing, activity modification
Fibrous dysplasia - polyostotic	Femur > craniofacial bones > tibia > humerus > ribs > fibula	May progress in size and number until skeletal maturity	Geographic, more aggressive than monostotic	Endosteal expansion	Same as monostotic	Fusiform expansion of cortex	None	Younger than monostotic, esp. if endocrine disorder		Same as monostotic	Multiple enchondromatosis, Paget's, Brown tumour, metastasis, von Recklinghausen's (NF, café au lait), MM, EG	Earlier onset = more progressive, 25% polyostotic, assoc. with McCune-Albright syndrome	Bone scan useful for detecting multiple lesions, can usually be diagnoses on Xray
Ossifying fibroma = Osteofibrous dysplasia = Kempson-Campanacci	Usually proximal anterior tibia, eccentric, diaphyseal	Variable	Osteolytic	Blister-like areas of cortical lucency surrounded by dense sclerosis, geographic	radiolucent	Intact and expanded	None	<20	Benign active or aggressive	Woven bone trabeculae in fibrous stroma	Adamantinoma (age), fibrous dysplasia, nonossifying fibroma, osteoblastoma, ABC	Very rare, can progress to adamantinoma	Observe if asymptomatic, high recurrence if <10, excision & BG
Giant cell tumor	75% in epi/metaphysic of long bones, 50% around knee, distal radius, prox humerus, vertebrae, sacrum	Usually >50% bone diameter	Lytic, geographic, often extends to subchondral bone	Expansile, usually non-sclerotic	None	Neocorticalization if perforates cortex	Almost always occurs by eroding through cortex (as opposed to Ewing's), 20%	80% > 20 years old, <5% with open plates	Benign aggressive, rarely metastatic (5%)	Multinucleated giant cells, hemosiderin = low signal on both T1 and T2	ABC, Brown tumour (Serum Ca), telangiectatic osteosarcoma, fibrosarcoma, osteoblastoma	Campanacci stages: 1. early, bone has normal contour 2. active, erosion, expansion 3. destructive, poor margin, cortical break +/- path #	Expendable bone = wide en bloc, non-expendable = curettage, adjuvant phenol/Nitrogen, BG or cement, 10-25% recur
Aneurysmal Bone Cyst	Spine (esp. post), femur, tibia, metaphysic of long bones, usually eccentric, can be diaphyseal	Variable, usually > 5cm	Lytic and expansile	Marginal sclerotic rim, neocorticalization	None	Eroded	Generally contained lesion	<30, 80% <20	Benign aggressive, not metastaic	Hemorrhagic cystic tissue, lacks endothelial cell lining, walls are fibrous tissue and giant cells	UBC (generally central), GCT (may co-exist), osteoblastoma, telangiectatic osteosarcoma	ABC and osteoblastoma both appear in posterior spine, MRI = multicystic, fluid-fluid level, markedly expansile	Open biopsy, curettage & BG/cement, recurrence 10-20%, en bloc in expendable bone
Unicameral bone cyst	Central medullary lesion, thought to begin in metaphysic and migrate to diaphysis, 80% = prox humerus or prox femur, also ilium, calcaneus, talus	Can be quite large	Lytic, lobulated borders	Geographic, thin sclerotic margin	None	Often circumferential endosteal erosion	None	80-90% < 20, younger = more active	Benign latent or active lesion of bone	green-yellow fluid, wall is fibrous tissue	ABC, fibrous dysplasia, chondromyxoid fibroma, telangiectatic osteosarcoma, infection, nonossifying fibroma	Fallen leaf sign = cortical fragment falls into lesion in path #, MRI = fluid-fluid only if path #, minimal bony expansion	Plain films and aspiration usually diagnostic, open biopsy if no fluid or hemorrhagic
Eosinophilic Granuloma	Diaphyseal or metaphyseal, skull, ribs, vertebrae, pelvis, and long bones (mostly femur and humerus), almost never hands or feet	Variable	Lytic, can be patchy, long bone expansion less common, path # common in spine (vertebra plana)	Commonly geographic, minimal metaphyseal sclerosis, may be periosteal reaction or onion skinning	None, except hazy matrix at resolution stage	Thinned, scalloped, onion skinning	Rarely	5 to 10, rare after 30	Benign active or aggressive, can be multiple, tumour-like disorder, belongs to Langerhans' cell histiocytoses	Friable tan tissue, histiocytes, Langerhans' cells (S100+, Birbeck granules on EM), eosinophils, giant cells	UBC (usually metaphyseal, larger, less painful), osteomyelitis, Onion skinning = Ewing's, non-Hodgkin's lymphoma, osteomyelitis, osteosarcoma	Often heals spontaneously (months to years), Biopsy may trigger healing	Indications: 1.diagnosis, 2.persistent pain, 3.prevention or treatment of pathologic fracture. Diagnosed lesions may be observed
Hemangioma of bone	Spine > craniofacial > femur or humerus > hands and feet	Variable	Vertebrae = vertical striations (corduroy), honeycombing	Rarefaction, reactive ossification	None	"disappearing bone disease", ballooning	Rarely	40 to 60	Benign latent or active, often incidental	Hemorrhagic, vessels, dilated capillaries between bone trabeculae	Spine = Paget's (picture frame appearance, expansion), myeloma, metastasis	Gorham's disease (massive lysis that can cross joints)	Asymptomatic = no treatment, vertebra collapse treated if neuro deficit, chronic pain, pre-op embolization

Familial Osteochondromatosis

Autosomal dominant, variable penetrance
30% spontaneous
Short stature, unequal growth of radius and ulna - Madelung deformity (radial deviation of the hand due to ulnar plus), unequal growth tibia and fibula – valgus knees and ankles
Long bones, pelvis, ribs, scapula, distal ends of proximal and distal phalanges
Monitor for malignant change

Ollier's disease = multiple enchondromatosis

Inborn error of osseous development
non-hereditary
Often unilateral, metaphysic & diaphysis
Deformity, growth disturbance
25-30% risk of transformation to low- or intermediate grade chondrosarcoma

Maffucci's syndrome

Congenital, non-hereditary
Multiple enchondromas and multiple soft-tissue hemangiomas
Calcified phleboliths
Higher risk of malignant transformation than Ollier's

McCune Albright syndrome

Polyostotic fibrous dysplasia – tendency to be unilateral
Café au lait spots – midline neck, chest, shoulders, sacrum, rough borders unlike NF
Multiple myxomas
Endocrine disturbances – Cushing's, hyperthyroidism, acromegaly, various adenomas
Classically present with precocious puberty and short stature

Letterer-Siwe Disease

Associated with EG
Acute, fulminant disease affecting infants < 2
Hepatosplenomegaly, adenopathy, CNS involvement, fever, anemia, thrombocytopenia
Potentially fatal
Treated with corticosteroids and chemo

Hand-Schuller-Christian disease

Develops in <20% of patients with EG
Onset usually 5 to 10 years old, rarely sooner
Exophthalmos (retro-orbital invasion), diabetes insipidus (sphenoid invasion), skull defects (classic triad < 10% of patients)

Malignant bone lesions

			Site	Size	To Bone	Bone response	Matrix	Cortex	Soft Tissue	Age	Chemo/rad	Pathology	DDx	Other	Rx
Osteosarcoma	Central Osteosarcoma	Conventional (75%)	Metaphysis, may extend across physis, distal femur, proximal tibia, proximal humerus, flat bones	Large (>5 cm)	Permeative, patchy lytic/sclerotic	Sunburst, Codman's triangle, no marginating reaction	Osteoid with variable sclerosis	Erosion	May occur without cortical destruction, poorly organized bony matrix	2 to25; Secondary osteosarcomas (Paget's) >50	Methotrexate, adriamycin, cisplatin, ifosphamide	Osteoid in lace-like, or sheet-like pattern, cells spindle, epithelioid, or small and round	GCT, ABC, Ewing's, osteoblastoma, mets, lymphoma	Alk phos, LDH often elevated, local recurrence is almost always associated with metastases, removal of lung mets often prolongs survival, radiation ineffective	Biopsy with MSK oncologist, neoadjuvant chemotherapy prior to limb salvage, 5-yr survival 50-85%,
		Telangiectatic	Metaphysis femur, tibia, humerus		Lytic, expansile, rarely permeative	+/- periosteal reaction			Usually, with hemorrhage		Chemo-responsive		ABC, GCT, Brown tumour, osteoblastoma		Similar to conventional
		Low-Grade	Most metaphysis of long bones, less diaphysis	Large	Lytic with internal sclerosis/ trabeculations	Wide zone of transition			Not usually	Young adults, mean 30		Difficult to diff from fibrous dysplasia, minimal atypia	Fibrous dysplasia, fibrosarc, infection, lymphoma	Better prognosis than conventional, rare path #
		In Pagetoid Bone								46 to 91		Usually high grade		Also chondrosarc, fibrosarc, MFH	Prognosis worse: large, axial, older (no chemo)
		Small Cell	Metaphyseal distal femur, humerus		Sclerotic or mixed, permeative	Codman's			Usually	10 to 30		Sheets of small, round, blue cells	Ewing's		Rare, differs from Ewing's by making osteoid
	Surface	Parosteal	2/3 post distal femur, prox medial humerus, lat prox tibia	Large	"Applied" to bone (plasticine), may encircle			Juxtacortical sclerotic mass, lobulated large base			Chemo if high grade	Fibrous, osseous, cartilaginous elements. Often low-grade, well-formed trabeculae	Periosteal osteosarc or chondrosarc, myositis ossificans, exostosis	Highest osteosarcoma prognosis	Biopsy with MSK surgical oncologist, wide resection, chemo if high grade
		High-Grade Parosteal (Dedifferentiated)	2/3 post distal femur, prox medial humerus, lat prox tibia	Large					Maybe	"	Chemo			Rare	Similar to central
		Periosteal	Metaphyseal and diaphyseal femur and tibia		Destructive, periosteal reaction, Codman's	Periosteal new bone in spiculed pattern	Stippled calcification, denser at base	Scooped out			Chemo if high grade	Generally low grade	High-grade surface osteosarc, periosteal chondrosarc, Myositis oss.		Wide excision
Chondrosarcoma	Central		Prox/dist femur, prox humerus, scapula, pelvis	>5 cm, variable	Geographic, destruction proportional to grade	Endosteal expansion	Punctuate calcifications	Endosteal scalloping, maybe cortical thickening	More in high grade	30-70	Not effective primary therapy, rads can be used if wide resection not possible	Myxoid or chondroid, binucleate cartilage cells, grade determined by atypia	Enchondroma, osteosarc, fibrosarc, MFH, mets, chordoma if sacrum	More distal location is more likely benign, lung mets can be years after	Biopsy with MSK surgical oncologist, wide resection
	Periosteal		Surface					May invade canal					Periosteal chondrome, periosteal osteosarc
	Clear cell		Epiphysis or metaphysic of long bone, esp. prox femur and humerus		Lytix, geographic	Sharp sclerotic margins	uncommon	Intact but may be expanded		20-40			chondroblastoma	Rare, low grade, can metastasize	Wide resection
	Mesenchymal				Lytic, destructive, permeative					10-30	Sometimes chemo	Small, round, blue cells with chondroid		Rare, high grade, poor prognosis	Wide resection
	Dedifferentiated		Similar to central							Similar to central		Mix of chondrosarc with fibrosarc or MFH		Rare, high grade, usually mets by 2 yrs	Wide resection
Fibrosarcoma			Pelvis, dist femur, prox tib, prox humerus, central > eccentric	Usually > 5cm	lytic	Permeative, no sclerosis	radiolucent	Thinned, destroyed, maybe periosteal rxn	Common in high grade	20-60	No. rads for palliation	Herringbone pattern spindle cells, atypia	MM, solitary met, GCT, MFH, dediff chondrosarc, (Ewing's if < 25)		Biopsy with MSK surgical oncologist, wide resection
MFH			Metaphysic of long bones, 50% in femur	Usually > 5cm	Lytic, destructive	Permeative, but may have sclerotic margin in low grade	rarely	Erosion	Usually	Bimodal 50-60, and 20-30	Neoadjuvant and adjuvant chemo	Malignant spindle cells in storiform pattern, mix of giant cells, histiocytes		Unknown cell of origin	Biopsy with MSK surgical oncologist, wide resection, chemo