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Tumor muskuloskeletal

Sarcoma
• A type of cancer that begins in bone
or in the soft tissues of the body,
including cartilage, fat, muscle, blood
vessels, fibrous tissue, or other
connective or supportive tissue.
• Mesenchym

Soft Tissue Tumor










Fibrous tumor
Fibrohistiocytic tumor
Lipomatous tumor
Smooth muscle tumor
Skeletal muscle tumor
Tumors of blood and lymphatic vessels
Perivascular tumor
Synovial tumor
Neural tumor
Paraganglionic tumor
Pluripoten mesenchymal tumor

Incidence
• It represents 1% of adult (age >16
years) malignancies and 15% of
pediatric cancers. This malignancy
can occur in any part of the body,
though approximately 50% of all
soft-tissue sarcomas present in an
extremity

Surgical Oncology, 2003

• Among extremity soft tissue sarcomas.Incidence • Overall. and leiomyosarcoma. malignant fibrous histiocytoma(MFH). the three most common subtypes of soft tissue sarcoma are liposarcoma.liposarcoma and MFH predominate. 2003 . Surgical Oncology.

Distribution 0f Sarcoma .

liposarcoma. MFH. • Histopathologic type is anatomic site dependent: The common subtypes in the extremities are liposarcoma. 2008 . synovial sarcoma. and leiomyosarcoma. and fibrosarcoma. the three most common histopathologic subtypes are MFH. Devita.• Overall.

Lymph node metastases are uncommon. 2008 . The pattern of metastasis of most sarcomas is hematogenous.Overview • Sarcoma histologic type is generally an important determinant of prognosis and also an important predictor of distinctive patterns of behavior • Sarcomas are characterized by local invasiveness. 28 Devita. except for selected cell types usually associated with childhood sarcoma.

and number of mitoses. . • Grading needs to be complemented with radiologic and molecular parameters.Grading • The pathologic features that define grade include cellularity. histologic type and subtype and/or differentiation. necrosis. pleomorphism.

Etiology • • • • Genetic Radiation Lymphedema Chemical agents exposure .

melanoma. as well as primary or metastatic carcinoma. and lymphoma. .Differential Diagnosis • benign lesions.

(Washington. • There is frequently a perceived antecedent trauma.Clinical presentation • a mass is usually large. Pain is usually a late symptom. is often painless. The majority present at a size larger than 5 cm. Lesions may be misdiagnosed as hematomas or strained muscle. and may be associated by the patient with an episode of injury. 2007) .

.Primary bone tumor on pediatric • Primary bone tumors in children are rare conditions • Th e most common benign lesion of bone is undoubtedly the solitary osteocartilaginous exostosis ( osteochondroma).

Sign & Symptoms • Pain that is constant. • Swelling • Pathologic fracture . unrelated to activity. and is worse at night.

Imaging • Plain photo • CT Scan • MRI .

FNA usually cannot give the grade . 2007) Incision should parallel to the long axis of the extremity. (Washington. • Core needle biopsy • Excisional biopsy • FNAB : cytopathologic evaluation only.Biopsy • Incisional biopsy : gold standard.

.• If the lesion under investigation is small (<5cm). an excisional biopsy should be performed. • If the lesion is large. an incisional) biopsy is the procedure of choice. a Tru-Cut (or if nondiagnostic. serving as both a diagnostic and therapeutic procedure.

Enneking’s classification .

AJCC .

2003 . • Current treatment of choice is thoracotomy with wedge resection of all pulmonary nodules. Surgical Oncology.Metastases • Soft tissue sarcoma of the extremity metastasizes almost exclusively to the lung.

2003 .Algorythm of Treatment Surgical Oncology.

Goals of treatment for resectable disease: • Complete removal with negative margins • Maximal preservation of function • Remove prior biopsy scar en bloc • Postoperative irradiation reduces recurrence .

Unresectable lesions or tumors: • Associated with widely disseminated disease: treat with primary irradiation 6500cGy .

Resection • Smaller. • Larger tumors may benefit from a larger margin or radiation to prevent recurrence.Surgical Treatment A. Washington. in general. grade I tumors can be excised with a minimum 1-cm margin. 2007 . usually without adjuvant radiation. Depending on the size and grade of tumor. require radiation therapy in addition to excision to avoid more-radical surgery. compartment resection may be indicated. • Grade II and III tumors.

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2003 .Surgical Oncology.

Washington.196:305).B. 2007 . The resection field should be marked with clips to guide radiation therapy. Limb-sparing resection • Limb-sparing resection combined with radiation therapy offers survival equivalent to that achieved with amputation (Ann Surg 1982.

Recurency • Soft tissue sarcoma of the extremity recurs in approximately onethird of all patients.and low-grade tumors of all sizes. with a median disease-free interval of 18 months. including those with high. .

Non-ossifying fibroma. 1995 .Giant cell tumour • Bone tumor : Osteoid osteoma. Fibrous dysplasia. Osteoblastoma Key Topic in Orthopedic.Benign Bone Tumor • Cartilage tumor : enchondroma. • Fibrous tissue : solitary bone cyst. chondroblastoma and chondromyxoid fibroma. Aneurysmal bone cyst.

Clinical Finding • progressive pain at rest and at night .

Location • epiphyseal (adult) a giant cell tumor. lymphoma. myelomas. and adamantinoma (especially in the tibia). Campbell's. or hemangiomas. whereas an epiphyseal lesion in children is likely to be a chondroblastoma. In younger patients with a vertebral body lesion. chordoma is at the top of the list of differential diagnoses.2007 . fibrous dysplasia. • Diaphyseal lesions includes Ewing sarcoma. • Most vertebral lesions in adult patients are metastases. the most likely diagnosis is histiocytosis. histiocytosis. In the sacrum.

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• Prostate cancer and breast cancer are the two most common primary sources for bone metastases. painful bone lesion. however. multiple myeloma and metastatic carcinoma are the most likely diagnoses even if the patient has no known history of carcinoma.Metastases of Unknown Origin • In a patient older than age 40 with a new. the most likely sources are lung cancer and renal cell carcinoma . • If a patient has no known primary tumor.

.Imaging • cortical destruction usually is a sign of malignancy. Periosteal reactive new bone formation results when the tumor destroys cortex and may take the form of Codman's triangle. “onionskinning.” or a “sunburst” pattern.

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Chondrosarcoma .

AJCC for Bone Sarcoma Campbell's. 2007 .

chemotherapy. Campbell's. 2007 . • The goal of treatment of a patient with metastatic carcinoma to bone is to minimize pain and to preserve function.Treatment • The goal of treatment in a patient with a primary malignancy of the musculoskeletal system is to make the patient disease free. and surgery. • The optimal treatment of the tumor often requires a combination of radiation therapy.

radiation has a limited role because local control is achieved better with surgery. . also frequently are sensitive to radiation treatment. with the exception of renal cell carcinoma.Radiation • Most primary bone malignancies are relatively radioresistant. and Ewing sarcoma. which are each exquisitely sensitive. • Carcinomas metastatic to bone. Exceptions are the marrow cell tumors. lymphoma. including multiple myeloma. • For most other bone tumors.

such as rhabdomyosarcoma. • The role of chemotherapy is less well defined for adult soft-tissue malignancies. .Chemotherapy • chemotherapy has a well-defined role in the treatment of other high-grade malignancies of bone. and high-grade soft-tissue malignancies of childhood. such as malignant fibrous histiocytoma. chemotherapy is not useful for cartilaginous lesions and most other low-grade malignancies. • In general.

preoperative radiation therapy for soft-tissue sarcomas and neoadjuvant chemotherapy for bone sarcomas have helped surgeons to resect successfully some tumors that in the past would have been deemed unresectable. . • Specifically.Surgery • now allow limb salvage to be a reasonable option for most patients with bone or soft-tissue sarcomas.