Principles Of Management Of Soft

Tissue Sarcoma
Dr Bashiru Aminu
MODERATOR DR MAI

Outline
Introduction
Epidemiology
Etiology
Classification
Principles of management
Rehabilitation
Complication

Introduction
Soft tissue sarcomas are solid malignancies

mostly of mesodermal origin

their rarity and variety
present formidable challenges in characterization and
treatment.
These malignant tumours require early diagnosis and prompt
treatment like any other tumour, in order to achieve optimum
care and a possible cure
management is multimodal and multidisciplinary

Epidemiology
1% of all malignancies in adults and 15% of malignancies

in children.
Age range is 3-85 years mean of 31 yrs
Twice as common in males as in females
Lower limb is the most favoured site foot and leg in Zaria,

thigh in Jos in mfu mandible is commoner than other

sites on face
Rhabdomyosarcoma is the commonest variety here
followed by fibrosarcoma, liposarcoma,
dermatofibrosarcoma, angiosarcoma

Epidemiology
Embryonal/alveolar rhabdomyosarcomas are the

most common soft tissue sarcomas of

childhood
whereas pleomorphic rhabdomyosarcoma occurs
predominantly in adults,

Etiology
Radiation exposure

-External radiation therapy is a rare but wellestablished risk factor for soft tissue sarcoma that
may be associated with radiationinduced mutations
of the p53 gene.
- The incidence of sarcoma among patients who are
often treated with radiation for cancer
of the breast, cervix, ovary, testes, or lymphatic
system is 8-50 times the general population

Etiology
Occupational chemical exposure

-Exposure to herbicides such as phenoxyacetic acids

and to wood preservatives containing chlorophenols
has been linked to an increased risk of soft tissue
sarcoma.
- Several chemical carcinogens, including thorium
oxide (Thorotrast), vinyl chloride, and arsenic, have
been associated with hepatic angiosarcomas.

Etiology
Trauma

-likely brings the tumour to focus not an etiology

-repeated trauma may however be a risk factor

Etiology
Chronic lymphedema
-Stewart and Treves first described the association
between chronic lymphedema after axillary
dissection and subsequent Lymphangiosarcoma
-estimated to occur in 0.07% of patients who undergo
axillary node dissection
- It also has been reported to occur after filarial
infections and in the lower extremities of patients
with congenital lymphedema

Classification
Molecular pathogenesis
Specific translocations,(alveolar rhabdo, synovial)
gene amplification(dedifferetiated liposarcoma)
defining oncogenic mutations(GIST)
complex genomic rearrangements(spindle cell, pleomorphic).
In general, sarcomas resulting from identifiable molecular events tend to

occur
in younger patients with histology suggesting a clear line of deferetiation
In contrast, sarcomas without identifiable genetic changes or
expression profile signatures tend to occur in older patients and
exhibit pleomorphic cytology and p53 dysfunction

Based on histology

Management
Diagnosis

History
an asymptomatic mass
as a deep venous thrombosis, particularly in patients without significant risk factors
for thrombosis
Tumors in the distal extremities are generally smaller, whereas tumors in the proximal
extremities and retroperitoneum can grow quite large before becoming apparent
Tumors often grow centrifugally and can compress surrounding
normal structures
Infrequently, tumor impingement on bone or neurovascular bundles produces pain,
edema, and swelling.
Less frequently, tumors cause obstructive gastrointestinal symptoms
or neurologic symptoms related to compression of lumbar or
pelvic nerves

Management
Examination

-deformity
-abnormal gait
-seizures or neurological symptoms
-cardiopulmonary compromise
-abdominal distension with splinted diaphragm or
visceral compression
-tumor may be tender, differential warmth, massive or
small, hard, involving skin or muscle depending on
depth

Management
Biopsy
open or image guided, Trucut is standard, Histology, grade possible
prevent sampling of nondiagnostic necrotic or cystic areas of the tumor
thus increase the positive yield rate.
Sonography/CT guidance also permits biopsy of tumors in otherwise

inaccessible locations and tumors located near vital structures.

incisional biopsy when core needle biopsy cannot produce adequate tissue for
diagnosis or when findings on core needle biopsy are nondiagnostic.
The disadvantages of incisional biopsy include the need

to schedule the procedure, the need for general anesthesia, and high costs.
In addition, an inappropriately placed incisions can necessitate more extensive
definitive resection to encompass the biopsy site

Management
The biopsy incision should be oriented longitudinally along

the extremity to allow a subsequent wide local excision that

encompasses the biopsy site, scar, and tumor en bloc.
A poorly oriented biopsy incision often necessitates an
excessively large surgical defect for a wide local excision,
which in turn can result in a larger postoperative radiation
therapy field to encompass all tissues
at risk.
Adequate hemostasis must be achieved at the time of
biopsy to prevent dissemination of tumor cells into adjacent
tissue planes by hematoma

Management
Excisional biopsy

-can be performed
for easily accessible (superficial) extremity or truncal
lesions
smaller than 3 cm.
However, excisional biopsy rarely provides
benefits over other biopsy techniques.
Excisional biopsies should not be performed for lesions
involving the hands and feet because such biopsies may
complicate definitive re-excision

Management
The features that define

grade are cellularity, differentiation (good, moderate,

or poor/anaplastic), pleomorphism, necrosis
(absent, <50%, or 50%),
and number of mitoses per high-power field (<10,
1019, or
20

Management
Extent of disease

-plain radiograph when diagnosis not known to rule out bone involvement
-MRI is gold standard for extremity, CT scan for trunk and abdomen
-When histologic assessment
of an extremity sarcoma reveals a myxoid liposarcoma, CT of
the abdomen and pelvis should be done because this subtype is
known to metastasize to the abdomen.
-MRI is the most useful imaging modality for extremity sarcomas because of its superior soft
tissue contrast resolution and multiplanar capabilities.
MRI accurately delineates muscle groups and distinguishes among
bone, vascular structures, and tumor.
Sagittal and coronal views
allow evaluation of anatomic compartments in three dimensions
Soft tissue sarcomas of the extremities usually present on MRI as a heterogeneous mass.

Management
- Their signal intensity tend to be equal to or slightly higher than that of
adjacent skeletal muscle on T1-weighted images and heterogeneous and high
on T2-weighted images.
-Hemorrhagic, cystic, or necrotic changes
may also be observed in the tumor.
If adjacent vascular structures must be delineated, special MRI techniques
may be performed, including magnetic resonance angiography.
MRI may also be an important adjunct to cytologic analysis in distinguishing
benign lesions such as lipomas, hemangiomas, schwannomas,
neurofibromas, and intramuscular myxomas from their malignant
counterparts.
In patients undergoing preoperative chemotherapy, contrast-enhanced T1weighted MRI can be useful in evaluating
intratumoral necrosis.

management
PET SCAN-

-FDG is used, whole body scan

-tumour grading
-response to chemotherapy
Other investigations to prepare for treatment
-E/U/CR, FBS, FBC, urinalysis
Informed consent
Selection of treatment
Multimodal, multidisciplinary

Management
treatment options

Local
Surgery
Radiotherapy
Isolated regional perfusion
Systemic

-chemotherapy
-targetted therapy

Management
surgery
The type of surgical resection is determined by several factors,
including tumor location, tumor size, depth of invasion, involvement
of nearby structures, need for skin grafting or autogenous tissue
reconstruction, and the patients performance status example
Wide local excision for the extremity and trunk
Tumour must be localised
Must be primary
<5cm wide
Must be superficial
Low grade, margins must be free, resectable without need for large
skin
Resection and anastomosis for bowel involvement

management
When margins are involved, grade is high, adjacent

structures involved, function affected

-modality can be combined
-amputation may be an alternative
Do not enucleate, if adjacent structures involved the
take adventitia, perineurium

Management
radiotherapy
External beam
Radiation therapy standard treatment for high grade extremity and trunk

wall soft tissue sarcomas either in the pre- or postoperative setting

High grade tumours, >5cm, involved margins
50gray in 25 fractions surgery in 4-8 wks
Brachytherapy
-shorter overall treatment time of 4 to 6 days, compared to the 4 to 6 weeks
generally required for preoperative or postoperative radiation therapy
regimens.
- A cost-analysis comparison of adjuvant brachytherapy versus adjuvant
external-beam irradiation for soft tissue sarcomas showed that costs were
lower with brachytherapy

management
Isolated regional perfusion is

a limb-sparing technique in which a soft tissue sarcoma

is perfused with high concentrations of tumor necrosis
factor- and
melphalan under hyperthermic conditions.
The technique is generally used for locally advanced,
multifocal, or locally recurrent
disease
it has also served as a palliative treatment to achieve
local control for patients with distant metastases

Management
chemotherapy
Results have been poor
Response determined by histologic subtype
Synovial sarcoma, myxoid/round cell liposarcoma,

and uterine leiomyosarcoma are sensitive

pleomorphic liposarcoma, myxofibrosarcoma, epithelioid sarcoma,
leiomyosarcoma, angiosarcoma, and desmoplastic round cell tumors
are intermediate
Resistant ones are clear cell sarcoma, endometrial stromal sarcoma,
alveolar soft part sarcoma, and extraskeletal myxoid chondrosarcoma
Doxorubicin and ifosfamide

targeted agents
being investigated for the treatment of soft tissue sarcomas.
Among these are tyrosine kinase inhibitors (e.g., imatinib,

sunitinib, sorafenib,
and dasatinib) for treatment of GIST
Antivascular endothelial growth factor antibodies eg
bevacizumab in metastatic or unresectable angiosarcoma,
solitary fibrous
tumor, and epithelioid hemangioendothelioma

Rehabilitation
Early physiotherapy
Prosthesis for post amputees
Ergonometry
Plastic reconstruction for defects

Complications
surgery

Early
heamorrhage
Heamatoma
Seroma
Flap necrosis
Wound infection
Late
Scar
Ventral hernia
recurrence

Complications
chemotherapy

Doxorubicin- cardiotoxicity
Ifosfamide-gastrointestinal hemorrhage
Radiotherapy wound necrosis, dehiscence,

ulceration

Recent advances
More effective and less toxic chemotherapy
Improved understanding of the molecular

pathogenesis of sarcomas has revealed several

potential targets against which investigators are
working to develop subtype-specific targeted therapy
Better molecular biologic profiling of tumours

Conclusion
Though a rarity, the relentless course of these group

of tumours in our resource scarce environment

necessitates a proper understating of the right mode
of treatment of the disease in order to obtain good
outcome and better prognosis

Thank you