Bone and soft tissue tumors Flashcards
Most common primary tumor of bone
Multiple myeloma
Benign chondrogenic bone tumors
Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Malignant chondrogenic tumor
Chondrosarcoma
Benign osteogenic tumors
Osteoid osteoma
Osteoblastoma
Malignant osteogenic tumor
Osteosarcoma
Benign bone tumor of unknown origin
Giant cell tumor
Malignant bone tumor of unknown origin
Ewing tumor
Malignant notochord bone tumor
Chordoma
Tumors of epiphysis
Chondroblastoma
Giant cell tumor
Tumors of diaphysis
Enchondroma
Fibrous dysplasia
Ewing sarcoma
Chondrosarcoma
Small bosselated benign tumor of the facial bones and skull that histologically resembles normal bone
Osteoma
Gardener’s syndrome
Multiple osteomas
Bone tumor characterized by pain during the night relieved by NSAIDs and is typically found on the metaphysis of the femur or tibia
Osteoid osteoma
Cause of pain at night in osteoid osteoma
Prostaglandin E2 produced by proliferating osteoblast
Composed of haphazardly interconnecting trabeculae of woven bone that are rimmed by prominent osteoblasts with the intertrabecular spaces filled by vascularized loose CT
Osteoid osteoma
Radiograph shows round radiolucency with central mineralization surrounded by abundant reactive bone that has a massively thickened cortex
Osteoid osteoma
Differences between osteoblastoma and osteoid osteoma
Osteoblastoma is larger, with duller pain not relieved by NSAID, and it involves the spine
Treatment for both osteoid osteoma and osteoblastoma
Surgery
Bone tumor associated with RB mutation and retinoblastoma
Osteosarcoma
Malignant mesenchymal tumor where the neoplastic cells produce bone matrix, predominantly in those <20 yo
Osteosarcoma
Secondary causes of osteosarcoma in elderly
Paget disease
Bone infarct
Prior radiotion
Big, bulky, gritty, gray-white tumors of the metaphysis of long bones with areas of hemorrhage and cystic degeneration
Osteosarcoma
Pleomorphic tumor cells with larger hyperchromatic nuclei, bizarre giant cells, and mitotic figure with fine and lacelike neoplasm of bone
Osteosarcoma
Periosteal reactions in osteosarcoma
Sunburst appearance
Codman’s triangle
Inheritance of multiple hereditary exostosis syndrome
Autosomal dominant
Benign tumor that develops only in bone of endochondral origin
Osteochondroma/exostosis
Attached to the skeleton by a bone stalk capped by benign hyaline cartilage covered by perichondrium on the metaphysis near the growth plate
Osteochondroma
Histologic appearance of cartilage in osteochondroma
Disorganized growth plate that undergoes endochondral ossification
Benign tumor of hyaline cartilage that occurs in bones of endochondral origin
Chondroma
Mutation associated with chondroma
IDH mutation
Location of enchondromas
In medullary cavity
Location of juxtacortical chondromas
On bone surface
Ollier syndrome
Nonhereditary condition associated with multiple enchondromas in the hands and feet. Pt c/o pain and fracture.
Maffucci syndrome
Multiple enchondromas with soft tissue hemangiomas
X-ray shows circumscribed lucency with central irregular calcifications, a sclerotic rim, and an intact cortex
Chondroma
Composed of nodule of hyaline cartilage encased by a thin layer of reactive bone
Chondroma
Malignant cartilage producing tumors, commonly in axial skeleton
Chondrosarcoma
Large bulky tumors made of nodules of glistening gray-white translucent cavity that destroy bone that presents as a painful, progressively enlarging mass
Chondrosarcoma
Characterized by the presence of numerous multinucleated osteoclast-type giant cells that is benign, but can be locally aggressive
Giant cell tumor or osteoclastoma
Bone tumor of large, red-brown mass that may have cystic degeneration that occurs in the epiphysis, but may extend into the metaphysis
Giant cell tumor
X-ray shows predominantly lytic and expansile with destruction of cortex
Soap bubble appearance of giant cell tumor
Pathogenesis of giant cell tumor
Neoplastic cells are primitive osteoblast precursors expressing increased RANKL which promotes proliferation of osteoclasts
Rare metastasis of giant cell tumor
Lung
Treatment with good response for giant cell tumor
Denosumab –> RANKL inhibitor
Malignant bone tumor characterized by primitive round cells without obvious differentiation
Ewing’s sarcoma
Pathogenesis of Ewing’s sarcoma
t(11;22) creating an EWSR1/FLI1 fusion gene
Soft, tan-white bone tumor mainly in medullary cavity with areas of hemorrhage and necrosis
Ewing’s sarcoma
Rounded cell clusters with a central fibrillary core and sheets of uniform, small, round cells with scant clear cytoplasm
Ewing’s sarocma
Homer-Wright rosette
Rounded cell clusters with a central fibrillary core that indicate neuroectodermal differentiation
Bone tumor that presents as painful, enlarging mass that is warm to touch and tender with fever
Ewing’s sarcoma
X-ray shows destructive lytic tumor permeating into soft tissue with layers of reactive bone deposition
Ewing’s sarcoma
Cancers that can cause osteolytic metastasis to bone
Carcinomas of kidney, lung, and GIT
Cancer that can cause osteoblastic metastasis to bone
Prostate CA
Benign tumor where all the components of normal bone are present, but do not differentiate into mature structures
Fibrous dysplasia
Mutation associated with fibrous dysplasia
Gain of function mutation in GNS1 that codes for alpha subunit of Gs
Tan-white, well-circumscribed, expansive lesion with an intramedullary location
Fibrous dysplasia
X-ray shows well-defined, radiolucent areas with thin-cortices and ground-glass appearance in the proximal midshaft
Fibrous dysplasia
Curvilinear trabeculae of woven bone surrounded by a fibroblastic proliferation without prominent osteoblastic rimming
Fibrous dysplasia
Mazabraud syndrome
Polyostotic fibrous dysplasia with soft tissue myxomas
McCune-Albright syndrome
Polyostotic fibrous disease with Cafe-au-lait macules and precocious puberty
Well-encapsulated, soft, yellow lesions often indistinguishable from normal adipose
Lipoma
Malignant tumors of adipose tissue
Liposarcoma
Characteristic cell of liposarcoma with central, scalloped nuclear and numerous fat vesicles
Lipoblast
Benign neoplasm of skeletal muscle
Rhabdomyoma
Associated with cardiac rhabdomyoma
Tuberous sclerosis
Malignant mesenchymal tumor with skeletal muscle differentiation
Rhabdomyosarcoma
Four types of rhabdomyosarcoma
Alveolar
Embryonal
Pleomorphic
Spindle cell/sclerosing
Rhabdomyoblasts
Differentiation strap cells with clearly visible cross-striations in embryonal rhabdomyosarcoma
Sarcoma botryoides
Variant of rhabdomyosarcoma that develops in the walls of hollow, mucosa-lined structures
Skeletal muscle specific markers commonly expressed by rhabdomyosarcoma
Myogenin
MyoD1
