MSK neoplasms Flashcards
neoplasms that usually localizes in the facial bones and nasal sinuses
Osteoma
complications of an osteoma
sinus obstruction
congestion
pressure headaches
Difference between osteoid osteoma and osteoblastoma
osteoid osteoma
- < 2cm
- diaphysis of long bones
- Appendicular skeleton
Osteoblastoma
- >2cm
- axial skeleton
Describe the morphology of an osteoid osteoma
well-circumscribed round/ oval mass in the cortex of long bones
hemorrhagic, tan/ gritty tissue with haphazard interconnections of woven trabeculae rimmed by osteoblasts
Intertrabecular spaces are filled by vascularized loose connective tissue
Clinical presentation of osteoid osteoma
severe nocturnal pain that is relieved with ASA
Osteoblastoma clinical presentation
dull pain that is unresponsive to ASA
Most common primary malignant tumor of the bone
Osteosarcoma
Associations of Osteosarcoma
RB
TP53
INK4a
MDM2
CDK4
Paget Disease
Bone Infarcts
Prior radiation
Diagnostics of osteosarcoma
appears at the metaphyseal region of long bones
tumor frequently breaks through the cortex and lifts the periosteum, forming a sunburst appearance and Codman’s triangle
Morphology of osteosarcoma
gritty, grey-white areas of hemorrhage and or cystic degeneration
fine lacelike pattern of neoplastic bone replaced by anaplastic malignant tumor cells
broad sheets or primitive trabeculae
Clinical presentation of osteosarcoma
painfully aggressive enlarging masses in long bones
usually around the knee
2nd most common group of bone sarcomas in children
Ewing Sarcoma
Pathology of Ewing Sarcoma
t(11q24; 22q12) causes the fusion of EWS gene on chromosome 22 to the FLI1 gene
Diagnostics of Ewing sarcoma
appears at the diaphysis of femur, tibia and flat bones of the pelvis
Destructive lytic tumor with permeative margins that extend into the surrounding soft tissues
Periosteal reaction- layers of reactive bone
Morphology of Ewing Sarcoma
soft, tan/white areas of hemorrhage and necrosis
sheets of small uniform, round cells with scant cytoplasm
cytoplasm appears clear due to abundance of glycogen
Homer wright rosettes
Ewing Sarcoma Clinical Presentation
painful enlarging mass with tenderness warmth and swelling
Multiple Enchondromas
Ollier Disease
Maffucci Syndrome
Associations of Chondromas
Diagnostics of Chondromas
found in the metaphysis of tubular bones in hands and feet
Morphology of chondromas
well-circumscribed nodules of hyaline cartilage containing cytomorphological benign chondrocytes
sclerotic rim with an intact cortex
usually < 3cm
Grey-blue and translucent
Late adolescence- early adulthood
multiple hereditary exostosis syndrome
chondrosarcoma
Osteochondroma associations
Most common primary benign bone tumor
Osteochondroma
Pathology of Osteochondromas
inactivation of the EXT gene
Diagnostics of Osteochondromas
appears at the metaphysis of the bone near the knee
benign cartilage capped tumor that is attached to the underlying skeleton by a bony stalk
Morphology of Osteochondromas
Cap is composed of benign hyaline cartilage varying in thickness and has the appearance of a disorganized growth plate
Pedunculated lesion with a bone stalk that is continuous with the cortex and medullary cavity of the underlying bone
underlying stalk contains cancellous
Osteochondroma clinical presentation
painful and tender near the ends of long bones
2nd most common malignant matrix producing tumor of bone
Chondrosarcoma
Predisposing factor of chondrosarcoma
Osteochondroma
Diagnostics of Chondrosarcoma
Large bulky tumors of the Axial skeleton
nodules of glistening gray-white translucent cartilage
Chondrosarcoma morphology
matrix is often gelatinous or myxoid
cartilage infiltrates marrow space and surrounds pre-existing bone trabeculae
Nuclei often show up and open chromatin pattern with atypia
Clinical presentation of chondrosarcoma
painful progressive enlarging masses in the pelvis and axial skeleton
usually metastasize to lungs
Most common soft tissue tumor of adulthood
Lipoma
Features of lipomas
well-encapsulated mass of mature adipocytes
usually soft, mobile and painless
arises in SubQ of proximal extremities and trunk
Pathology of Liposarcoma
t(12;16) causes amplification of 12q13-15
causes mutation in MDM2, disinhibiting p53
Pathology of Nodular Fasciitis
t(17;22) produces a MYH9-UPS6 fusion gene
Diagnostics of Nodular Fasciitis
usually arises in the deep dermis, SubQ or muscle
Morphology of Nodular Fasciitis
usually < 5cm
circumscribed or slightly infiltrative
Richly cellular in immature appearing fibroblasts, myofibroblasts, lymphocytes and extravasated RBCs
Cells vary in shape and size
Cells have conspicuous nucleoli
Gradient of maturation from cellular, loose and myxoid to organized fibers
Which fibromatosis?
Males> females
Dupuytren contracture
Peyronie disease
superficial fibromatosis
Which fibromatosis?
women> men
Gardner Syndrome
FAP and APC mutations
arise in musculoskeletal aponeurotic structures, limb girdles or mesentery
Deep Fibromatosis
“Desmoid Tumor”
due to mutations in APC or Beta Catenin causing increased Wnt signaling
Morphology of fibromatoses
firm, grey-white lesions
poorly demarcated masses 1-15cm
Tough and rubbery, marked with infiltration of surrounding muscle, nerve and fat
cytologically bland fibroblasts are arranged in broad sweeping fascicles amid dese collagen
resembles a scar
osteoclastoma
tensynovial giant cell tumor
Pathology of tensynovial giant cell tumors
neoplastic mononuclear cells produce RANKL to induce osteoclast formation
Diagnostics of osteoclastoma
soap bubble lytic lesion surrounded by a thin bony shell, usually around the knee
Adults: usually appears at epiphysis or metaphysis
Children: usually appears at the metaphysis
XR: multicystic osteolytic lesions
Morphology of Tensynovial Gian Cell Tumor
red-brown mass with cystic degeneration
composed of reactive giant cells and neoplastic mononuclear cells
Clinical presentation of tensynovial giant cell tumors
local pain and swelling
pathologic fractures
Associations of fibrous dysplasia
mutations in GNAS1 gene
Increased cAMP
McCune-Albright Syndrome
Mazabraud Syndrome
Morphology of Fibrous dysplasia
Gritty, tan-white, well-circumscribed intramedullay mass
Curvilinear trabeculae of woven bone surrounded by a moderately cellular fibroblastic proliferation
No osteoblastic rim
Malignant bone tumor in metaphysis of long bones
Osteosarcoma
Malignant cartilage tumor in metaphysis of Axial Skeleton
Chondrosarcoma
Malignant cartilage tumor in diaphysis
Chondrosarcoma
Malignant bone tumor in the diaphysis of femur, tibia or pelvic bones
Ewing Sarcoma
Benign bone tumor in appendicular skeleton
Osteoid Osteoma
or
Fibrous Dysplasia
Benign bone tumor of the metaphysis or epiphysis
Giant Cell Tumor
Benign bone tumor in metaphysis of facial bones
osteoma
Benign bone tumor in metaphysis of axial skeleton
osteoblastoma
Benign cartilage tumor in metaphysis near the knee
osteochondroma
Benign cartilage tumor in the metaphysis of tubular bones
Chondroma
Benign tumor found in the metaphysis of long bones or posterior vertebral bodies
Aneurysmal Bone Cyst
