NO NEED TO DOMSK tumors and conditions Flashcards
Exostosis (osteochondroma)
tumor or area of aberrant growth of normal tissue, Arises near the growth plat at edge of epiphysis, arises in bones which grow by endochondral ossification (have growth plate), lesion has a cartilage cap which grows thru endochondral ossification
Exostoses stop growing at end of standard long growth of long bones
Usually solitary
Multiple= autosomal Dominant syndrome : multiple hereditary exostoses
Sessile (flat) or pedunculated (stalked lesion)
Bone from metaphysis grows into lesion base, if symptomatic resect lesion through the stalk, COMMON lesion
Rarely malignant, multiple lesions have a greater chance of malignancy, ,alignancy arises from cartilage cap, if an adult lesion begins to grow, then suspect malignancy
endochondroma aka chondroma
Benign tumor, occurs in shafts of tubular bones, bones that are preformed in cartilage
May fill completely fill the canal and begin to expand cortex,
Shouldnt break thru cortex and form a soft tissue if benign
Symptomatic lesion treated with currettage, and bone grafting
Histologically: cellular cartilage matrix w/o cytologic atypia
Rarely malignant( if they are low to medium grade chondrosarcoma)
Mulitple bone involvement= ollier disease
Multiple bone + associated with cutaneous hemangiomas= Maffuccis syndrome
Chondrosarcoma
occurs in adults (30s to 60s)
Consists of malignant cartilage cells, higher incidence of occurrence in flat bones: pelvis and scapula
Can occur in end of long bones and small bones of hands and feet
Aggressive lesions with cortical destruction and soft tissue masses, areas of calcification within the mass are seen, 2nd lesion to old benign enchondromas and exostoses
Histology: cell lesions with pleomorphism, frequent binucleation of cells and cytologic atypia, Myxoid changes in matrix,
osteoid osteoma
benign bone forming tumor, tumor can occur anywhere in the skeleton, peak incidence in 0-25
Presents with deep aching pain (pain is worse at night), pain is relieved with NSAIDS
Lesion produces PGs, poroduces severe muscle spasm, secondary scoliosis can occur in spinal lesions
Radiofraphically: small lucency is surrounded by marked amount of benign appearing reactive zone of bone, could be brodies Abcess, stress fractures, osteoblastoma
Histology: benign nidus of reactive fiber bone surrounding by dense cortical bone, both areas appear bengin
Treatment varies: treat the symptoms , en bloc resection, radiofrequency ablation by MSK radiology or interventional radiology
Active but bnign osteoblasts
Osteoblastoma
destructive bone forming benign bone tumor
occurs in younger age group from below 10-40s
vertebral lesions (posterior element), flat bones (scapular and pelvic) and long bones
May be aggressive appearing malignant appearance
Histology: irregular osteoid formation surrounded by vascularity and osteoblastic rimming, may have numerous mitotic figures but no atypical mitoses, histology resembles the nidus of an osteoid osteoma
Treatment: surgical resection
osteosarcoma
Several types:
Conventional OS: Age (bimodal ) , secondary OS pagets disease
Bone location: metaphysis
Bones: distal femur, proximal tibia, proximal humerus, pelvis, smaller bones
Radiographs: destruction of cortical and metaphyseal bone, periosteal new bone (codmans triangles) with poorly defined borders. Soft tissue masses, intra and extra osseous new bone formation
Histology: hypercellular with pleomorphic and hyperchromatic spindle cells, abnormal mitoses, areas of necrosis, osteoid formation (must have it but could have chondroid or fibrous)
Metastases: occur frequently mainly pulmonary
Treatment: neoadjuvant chemo, wide resection
Prognosis 70% 5 year
Ewings Sarcoma
malignant tumor whose cell of origin may be undifferentiated mesenchymal cells that may be neural crest in origin
Small round blue cell tumor, onion skin
Radiography: arises in diaphysis of long bones, short tubular bones, pelvis or scapula, destructive/premeative lesions, large soft tissue masses, non-mineralized matrix
Histology: lots of blue cells, delicate chromatin pattern, not much matrix, mitotic figures arent common, CD99 positive, Periodic acid schiff positive
Gross: slimy, discohesive, pus like material, no matrix or structure
Treatment: chemo, surg, radiation
giant cell tumor of bone
Aggressive benign bone tumor
Occurs in adults after physeal plate has closed
Benign lesion can met to lung
Radiography: old epiphyseal regions near bone ends, extends to subchondral bone of joint, destructive radiolucent bone lesion, soft tissue mass, vascular appearing, may have secondary Aneurysmal Bone cyst formation
Path: grossly firable red brown tissue, necrotic and cyst formation possible
Histology: mononuclear cells with scattered to abundant numbers of giant cells, giant cell nuclei resemble the nuclei of the mononuclear cells, No matrix production, mitotic figures are variable, varying vascularity
Treatment: surgical (extendd curettage of tumor with pulse lavage, PMMA cement, lungs to make sure no mets
Non op: Denosumab: RANKL inhibitor–> inhibits osteoclasts (aka OSTEOCLASTOMA)
nonossifying fibroma
Fibroma of bone, metaphyseal fibrous defect, most common bone tumor of childhood
Usually asymptomatic are discovered incidentally
Radiology: lacks aggressive features, excellent rim of reactive bone, multiloculated, no evidence of bony matrix, rarely fractures may occur
Path: Gross (thinner than normal cortex, brownish yellow lesion, non invasive into bone, easily curetted, non vascular). Microscopic (benign spindle cells, lipid laden histiocytes, a few giant cells no signs of malignancy atypia)
Treatment: if asymptomatic and incidental (periodic x rays), if symptomatic (curettage of lesion, bone graft with allograft of autogenous iliac crets), treat fractures by closed means of ORIF, if fracture occurs then fracture followed by treatment of lesion
fibrous dysplasia of bone
benign condition, mostly asymptomatic, present with pain deformity or pathologic fracture. Monostotic (single bone), poluostotic (multiple bones). Can involve long bones, pelvis, ribs, skull
Albrights: polostotic FD, cutaneous pigmentation, precocious puberty
Radiography: Metaphyseal or diaphyseal long bone lesions, expansion of cortices, ground glass appearance of matrix, involves flat bones (skull and ribs), may involve only one side of body, proximal femurs can deve curvature (shepherds crook deformity(
Pathology: gorss (dense fibrous tissue with gritty tissue), secondary cysts may form
Histology: collagenous matrix with benign fibroblasts and immature bony trabeculi scattered throughout the matrix, minimal vascularoty, no worrisome feature
Treatment: observation if asymptomatic, curettage, use of cortical allografts if critical areas of bone, open reduction and internal fixation
Starry night
Unicameral bone cyst
simple cyst, most asymptomatic, may present with swelling of the region, may present with sudden onset of pain or pathologic fracture, common affect proximal humerus or proximal femur
Radiography: metaphyseal frequently in upper humerus or proximal femur. Also found in calcaneus, fingers. Lesions about the epiphyseal plate, expansion of lesion does not exceed the width of the bone. MAy have a fallen leaf sign a piece of metaphyseal bony cortex that has fallen inside of the lesion. Pathologic fracture can occur
Path: Gross (cyst contains thin yellow fluid, red/pink due to trauma to cyst, cyst wall is thin and white), microscopy: thin lining of cyst with benign appearing cells, no atypia, amorphous debris within cyst
Treatment: irrigation of cyst with injection with cort, prodense, bone grafting
aneurysmal bone cyst
Swelling or ballooning of the affected extremity in which the tumor is located, pain in the affected bone with deformity or swelling of the extremity in which the tumor lies.
Radiography:expansion of the affected bone, thin to sclerotic rim of bone surrounding the cyst, no identifiable matrix formation, on CT and MRI fluid are seen, DD includes unicameral simple bone cyst, giant cell tumor of bone, sarcoma (telangiectatic osteosarcoma, osteoblastoma of vertebrae)
Path: Gross (hemorrhagic appearance with both a fleshy portion and areas of clotted and unclotted blood, eggshell thn area of periosteal reactive bone formation covering the lesion
Microscopy: cavernous spaces lacking endothelial lining with blood or clot, walls of spaces contain both fibroblastic spindle cells and giant cells
Pagets disease of bone (osteitis deromans)
Abnormal funciton of osteoclasts and osteoblasts (uncoordinated efforts) and lack of appropriate remodeling. Often an incidental radiographic finding or found on autopsy. Predilection for adults of european dissent, Cause is unclear Most common bones: skull, pelvis, femur, tibia, humerus, and vertebrae. bones have both sclerotic and radiolucent areas and enlarge overtime. Haphazard remodeling caused by dysfunction of the osteoclasts, secondary sarcomas (osteosarcoma) can form in adults with Pagets Radiography: skull radiolucent defects with sharp margins (osteoporosis circumscripta) Late (bone thickens and enlargement with increased density) Spine (increased width of vertebral bodies with coarsened trabeculae) Long bones (lucent wedge)
Lipoma
Painless lump occurs superficially or deep in extremities or trunk, consists of mature fat without cytologic atypia, growth occurs despite dietary changes, may become symptomatic because of size or pressure on an adjacent NV structure
Imaging (use an MRI)–uniform fat throuout lesion and be similar in signal to surrounding normal fatty tissues. Abnormalities may indicate an atypical lipoma or higher grade sarcoma. Use of contrast helps define both the vascularity of the tumor and surrounding structures
CT and ultrasound also used
Histology: mature fat with normal appearance of lipocytes, no atypia or malignant features
Desmoid tumor
Aggressive fibromatosis, locally aggressive neoplams originating in muscular facial planes, sheaths, joint capsules and even bone. Like sarcomas (infiltrate and are not encapsulated), Occurs in adults and older children. Common areas (proximal regions such as shoulder and gluteal areas, thigh popliteal space, upper arm, forearm, common in men. Can occur in abdominal wall in women following pregnancy (usually solitary)
Gardner syndrome: desmoids, APC
Imaging:MRI behaves like a sarcoma
History: malignant lipocytic pattern which resembles normal fat in low grade lesion, becomes more myxoid, then round cell pleomorphic with more cellularity and atypical metastases as the grade of tumor increases
Treatment: surgery, radiation ets
