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

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

Distribution 0f Sarcoma

 Overall, the three most common

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

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. The pattern of metastasis
of most sarcomas is hematogenous.
Lymph node metastases are uncommon,
except for selected cell types usually
associated with childhood sarcoma. 28
Devita, 2008

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

Etiology

Genetic
Radiation
Lymphedema
Chemical agents exposure

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

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

Imaging
Plain photo
CT Scan
MRI

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

 If the lesion under investigation is

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

Ennekings classification

AJCC

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

Surgical Oncology, 2003

Algorythm of Treatment

Surgical Oncology, 2003

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

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

Surgical Oncology, 2003

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

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

Benign Bone Tumor

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

Clinical Finding
progressive pain at rest and at
night

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

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

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.

Chondrosarcoma

AJCC for Bone Sarcoma

Campbell's, 2007

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

Campbell's, 2007

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

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

Surgery
now allow limb salvage to be a
reasonable option for most patients with
bone or soft-tissue sarcomas.
Specifically, 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.