CHAPTER 87 Soft Tissue and Bone Sarcomas and Bone Metastases

SARCOMAS
Soft Tissue Sarcomas <1% of all malignancies Bone Sarcoma
mesodermal in origin

FACTORS CLASSIFICATION
Scar Tissue- trauma/ previous injury Benign- enchondroma, osteochondroma, chondroblastoma,
Chemical carcinogens osteoid osteoma, osteoblastoma, fibroma, hemangioma
Previous radiation therapy Malignant- plasma cell tumors, osteosarcoma,
Infections, Therapeutic immunosuppression chondrosarcoma, Ewing’s, UPS

GENETIC Musculoskeletal Tumor Society Staging System

Li Fraumeni - Based on grade (roman numeral) and
Neurofibromatosis compartmental localization (letter)
Inherited Retinoblastoma I - low grade
t(x,18) (p11;q11) II - high grade
III- Tumor with LN or distant metastasis
60% in extremities A - Intracompartmental
30% in trunk B - Extracompartmental
10% in head & neck
Osteosarcoma
formation of osteoid or bone
DIAGNOSIS radiograph: moth-eaten, sunburst appearance, Codman’s
MC presentation- asymptomatic mass Core Needle Biopsy triangle
AJCC on Cancer Staging System of Sarcomas MC prognostic factor: response to chemotherapy
5% LN metastasis, MC- pulmonary parenchyma STD management: Preop chemotherapy followed
by limb-sparing surgery then post-op chemo
Radiograph, MRI- tumors of extremities, head & neck
CT- tumors of chest, abdomen, retroperitoneum
Chondrosarcoma
affects flat bones of shoulders & pelvic girdles
radiograph: lobular appearance with mottled/ annular
TREATMENT by STAGE: calcification
I- wide excision with negative margin incorporating management: surgical resection of primary/recurrent tumor
biopsy site (STANDARD)
II- considered for adjuvant radiation therapy Ewing’s Sarcoma
very aggressive
III- benefits from a chemotherapy involves diaphyseal region of long bones ad flat bones
IV- chemotherapy, with or without other modalities radiograph: onion peel periosteal reaction
management: systemic chemotherapy before surgery
 Primary tumor sites: Prostate, Breast, Lungs, Kidney, Bladder, Thyroid

Spread hematogenously or through local invasion

METASTATIC TUMORS TO BONE

(vertebrae, proximal femur, pelvis, ribs, sternum,
proximal humerus)

osteoclasts osteoblasts

osteolysis osteogenesis

Treatment depends on underlying malignancy

Pain: local radiation therapy
Denosumab, Pamidronate
CHAPTER 86 Primary and Metastatic Tumors of the Nervous System

BRAIN
TUMOR
PRESENTATION NEUROLOGIC TOXICITY OF THERAPY
Generalized/Nonspecific- impaired cognitive function, hemiparesis, headache Radiotherapy
Focal/Lateralizing- seizures, aphasia, visual field defects Acute- due to transient disruption of BBB
Early Delayed- due to focal demyelination
NEUROIMAGING Late Delayed- most serious, often irreversible
Cranial MRI with gadolinium contrast (preferred) Chemotherapy
Functional MRI myelosuppression
PET scan neurotoxicity- acute & chronic encephalopathy,
visual loss, cerebellar dysfunction, seizures,
TREATMENT myelopathy, peripheral neuropathy
Definitive: based on tumor type- Surgery, Radiotherapy & Chemotherapy
Symptomatic: Dexamethasone- glucocorticoid of choice for edema,
anti-epilepsy- for seizure control, prophylactic anticoagulants

PRIMARY BRAIN TUMORS METASTATIC TUMORS TO

Risk Factor: exposure to ionizing radiation, immunosuppression, genetic syndrome BRAIN
spread hematogenously
Intrinsic “Malignant” Tumors primary tumors from: lung, breast, melanoma,
Diffuse Gliomas prostate
Astrocytomas
Oligodendrogliomas
Ependymomas
Primary Central Nervous System Lymphoma
Medulloblastoma- MC malignant brain tumor of childhood, Homer-Wright rosettes
Pineal region Tumors

Extrinsic “Benign” Tumors

Meningiomas- now the most common primary brain tumor
Schwannomas
Pituitary Tumors
Craniopharyngiomas
Other Benign Tumors- Epidermoid Cysts, Dermoid cysts, Colloid cysts

Neurocutaneous Syndromes (Phakomatoses)

Neurofibromatosis Type 1, 2
Tuberous Sclerosis