Describe Bone lesions by patient age:
Types of tumor bone interactions:
Distributions of varius lesions in patients less than 30
Distribution of varius bone lesions in patients older than 30
Classification of Primary Tumors of Bone and Bone Matrix:
Tumors by Location:
Principles of Biopsy:
Immunohistochemistry and molecular testing for bone and Soft tissue Tumors:
Common Chomosomal translocations in Bone and Soft Tissue Tumors:
Musculoskeletal Syndromes, Genes, and Neoplasms:
Histologic grading of soft tissue tumors:
Staging System; Enneking System
American Joint Committee on Cancer Staging System for primary malignant tumors of bone for those tumors diagnosed on or after Jan 1 2010:
American Joint Committee on Cancer staging system for primary malignant tumors of soft tissue for those tumors diagnosed on or after Jan 1,2010:
Treatment regimens for malignant bone tumors:
Chemotherapy for bone sarcomas:
Radiation Therapy for soft tissue sarcoma:
Most Common MSK tumors:
Key points about soft tissue sarcomas:
A mass that is more than 5cm, growing and deep to the fascia should be presumed to be a soft tissue sarcoma.--image with MRI.
MRI is the best imaging modality
CT of the chest is performed to evaluate for mets
ESARC--STS with mets to lymph nodes---5% of STS
Epitheloid, Synovial, Angiosarcoma, Rhabdomyosarcoma, Clear cell
why do I have to learn about Ollier and Mafucci Syndrome?
Ollier disease/Maffucci syndrome
When there are many lesions, the involved bones are dysplastic, and the lesions tend toward unilaterality, the diagnosis is multiple enchondromatosis, or Ollier disease.
Inheritance pattern is sporadic.
If soft tissue angiomas are also present, the diagnosis is Maffucci syndrome.
Patients with multiple enchondromatosis are at increased risk of malignancy (in Ollier disease, 30%; in Maffucci syndrome, 100%).
Patients with Maffucci syndrome also have a markedly increased risk of visceral malignancies, such as astrocytomas and gastrointestinal malignancies.
why do I have to learn about MHE or multiple Heridetary Exostoses?
Multiple hereditary exostoses
The osteochondromas in MHE are often sessile and large. This is an autosomal dominant condition with mutations in the EXT1 and EXT2 gene loci. In approximately 10% of patients with multiple exostoses, a secondary chondrosarcoma develops. The EXT1 mutation is associated with a greater burden of disease and higher risk of malignancy.
Describe a chondroblastoma:
Centered in the epiphysis in young patients, usually with open physes; it may also occur in an apophysis
Pain referable to the involved joint
The most common locations are the distal femur, proximal tibia, and proximal humerus.
Imaging Shows a central region of bone destruction that is usually sharply demarcated from the normal medullary cavity by a thin rim of sclerotic bone
The basic proliferating cells are thought to be chondroblasts.
Scattered multinucleated giant cells are found throughout the lesion.Zones of chondroid are present.Mitotic figures may be found.
Treatment: Intralesional resection with curettage and reconstruction
Differential diagnosis: Brodie’s abscess and giant cell tumor of bone
Less than 5% mets to the lungs
Describe a chondromyxoid fibroma
Describe a chondrosarcoma
Pain or a mass in adults over 50 years old
Most common locations include flat bones (e.g., the scapula), pelvis, and spine.
Radiographs usually yield diagnostic findings, with bone destruction, thickening of the cortex, and mineralization consistent with cartilage within the lesion (see Fig. 9.16).
Prominent cortical changes (endosteal scalloping and erosion) are present in 85% of affected patients.
Histology-Differentiating malignant cartilage may be extremely difficult on the basis of histologic features alone.
The clinical, radiographic, and histologic features must be considered in combination for an accurate diagnosis. Criteria for the diagnosis of malignancy include the following:
Many binucleate cells with plump nuclei
Particularly large cartilage cells with large single or multiple nuclei containing clumps of chromatin-Infiltration of the bone trabeculae
Chondromas of the hand (enchondromas)—the lesions in patients with Ollier disease and Maffucci syndrome—and periosteal chondromas may have atypical histopathologic features.
Treatment: Wide surgical resection
Chemotherapy has not been shown to improve survival.
Dedifferentiated chondrosarcoma: The most malignant cartilage tumor
Pain and decreased motion.
Most common locations include the distal and proximal femur and the proximal humerus.
Imaging: Biomorphic appearance with that of a typical chondrosarcoma with a superimposed, highly destructive area
Histology: Low-grade cartilage component that is intimately associated with a high-grade spindle cell sarcoma (osteosarcoma, fibrosarcoma, MFH)
Treatment: Wide-margin surgical resection and multiagent chemotherapy
Other features: Prognosis is poor, and rate of long-term survival is less than 10%.
Key considerations for lymphoma of bone
Pain in patients of all ages.
The most common locations include the distal femur, proximal tibia, pelvis, proximal femur, vertebra, and shoulder girdle.
Images often show a lesion that involves a large portion of the bone
•Bone destruction is common and often has a mottled appearance.
A large soft tissue mass out of proportion to the amount of bone destruction is characteristic of lymphoma of bone.
Histology: A mixed cellular infiltrate is usually present. Most lymphomas of bone are diffuse, large B-cell lymphomas.
Immunohistochemistry: CD45 and leukocyte common antigen (LCA) positive.
Treatment: Multiagent chemotherapy ( [CHOP]) is curative.
Surgery is used only to stabilize fractures.
Plasma cell dyscrasias represent a wide range of conditions from monoclonal gammopathy of undetermined significance (MGUS; Kyle disease) to multiple myeloma.
Three plasma cell dyscrasias pertain to orthopaedic surgery: multiple myeloma, solitary plasmacytoma of bone, and osteosclerotic myeloma.
Malignant plasma cell disorder that commonly occurs in patients between 50 and 80 years of age
Presentation: Manifests with bone pain, usually in the spine and ribs, or as a pathologic fracture
Fatigue is a common complaint secondary to the associated anemia.
Symptoms may be related to complications such as renal insufficiency, hypercalcemia, and the deposition of amyloid.
Serum creatinine values are elevated in about 50% of affected patients.
Hypercalcemia is present in about 33% of affected patients.
Imaging: Radiographic appearance is of punched-out, lytic lesions which may show expansion and a “ballooned” appearance.
Sheets of plasma cells that appear monoclonal with immunostaining
Well-differentiated plasma cells have eccentric nuclei that have peripherally clumped, chromatic “clock faces.”
Systemic therapy and bisphosphonates
Surgical stabilization with irradiation is used for pathologic fractures.
Radiotherapy is also used for palliation of pain and treatment of neurologic symptoms.
The prognosis is related to the stage of disease; the overall median survival time is 18–24 months.
Solitary Plasmocytoma of bone
It is important to differentiate solitary myeloma from multiple myeloma because of the more favorable prognosis in patients with the solitary form. Diagnostic criteria include the following:
A solitary lesion on skeletal survey
Histologic confirmation of plasmacytoma
Bone marrow plasmacyte count of 10% or less
Patients with serum protein abnormalities and Bence Jones proteinuria (protein levels <1 g/24 hr) at presentation are not excluded if they meet the aforementioned criteria.
External beam irradiation of the lesion (4500–5000 cGy)
When necessary, prophylactic internal fixation
In approximately 50%–75% of affected patients, solitary myeloma progresses to multiple myeloma.