Soft tissue sarcomas

Dr.Yikunoamlak M.(MD./G.surgeon)
2016E.C,YHMC
 INTRODUCTION
• Sarcomas are a heterogeneous group of neoplasms that arise predominantly
from cells of the embryonic mesoderm that constitute connective tissues of
the body
• Sarcomas make up <1% of all cancers and occur in 2–4 people per 100,000
population

• Sarcomas can be:

 Soft tissue sarcomas
 Bone sarcomas
 Both

• Sites:
 Extremity (50–60%) ,the trunk (19%), retroperitoneum (15%), and head and
neck (9%)
 Soft-tissue sarcomas

• Predominate over bone sarcoma with a 4:1 incidence ratio

• 1.4:1, male-to-female ratio
• The median age of diagnosis is 59 years
• Bimodal distribution of STS peaking in the 5th and 8th decades.
• Includes more than 70 histologic subtypes
 Pathophysiology
• Most sarcomas are of unknown cause
• Most do not seem to result from progression or dedifferentiation of a
benign soft tissue tumor

 Molecular pathogenesis
• Translocations, gene amplification, defining oncogenic mutations, and
complex genomic rearrangements
 Radiation exposure
 Occupational chemical exposure
 Chronic lymphedema
 Infections
 Clinical presentation …STSs

• Determined by anatomic location, histologic subtype and grade of

aggressiveness, and size

 Asymptomatic mass
Increasing in size, >5 cm, deep seated, and/or painful

 Compressive symptoms

 Metastatic symptoms
• Dominant pattern is hematogenous, primarily to the lungs ,bone,brain,liver
• Lymph node metastasis is rare (affecting <5%)
STS
 Diagnosis
• Imaging
 Diagnostic and metastatic
 X-ray, ultrasound , CT-scan, MRI ,PET-scan
• Tissue diagnosis
 Fine-Needle Aspiration
 Core Needle Biopsy
 Incisional Biopsy
 Excisional Biopsy
 Metastatic work-up
 Lung metastasis -CT scan for tumors larger than 5 cm or high grade
-X-ray for smaller or low-grade lesions
 Abdominal/pelvic CT should be performed in patients
-with myxoid round cell liposarcomas, leiomyosarcomas, epithelioid
sarcomas, or angiosarcomas
 Whole Spine MRI has been advocated for myxoid round cell
liposarcoma.
 MRI of the brain should be considered for patients with
alveolar STS and angiosarcoma
 STSs Staging
• There are different staging systems depending on where the
cancer is in the body
 Head and neck
 Trunk and extremities
 Abdomen and thoracic (chest) visceral organs
 Retroperitoneum

• American Joint Committee on Cancer (AJCC) staging is based on

4 key pieces of information :
TNM + the Grade
• This system does not apply to GIST, fibromatosis
(desmoid tumor), Kaposi’s sarcoma, or infantile fibrosarcoma
 STS Treatment
 Surgery
• Wide local excision with 1- to 2-cm margins
• Amputation: for neurovascular or bone involvement

 Radiation therapy
• Large (T2), intermediate- or high-grade tumors

 Chemotherapy
• High-grade sarcomas or distant metastases based on the histologic
sensitivity
 Retroperitoneal sarcomas

 Are rare tumors accounting for only 1%–2% of all solid malignancies.
 10%–20% of sarcomas and one 3rd of retroperitoneal tumors
 Overall incidence is 0.3%–0.4%
 The peak incidence is in the 5th decade but can occur in any age group
 Approximately two-thirds of retroperitoneal sarcomas are high grade
 The most common types are liposarcomas and leiomyosarcomas

• Differential diagnosis
 Primary germ cell tumors, lymphoma, metastatic testicular cancer,
neuogenic tumors
 Clinical approach

• Typically do not produce symptoms until they grow large enough to

compress or invade contiguous structures

 Pain, early satiety, and obstructive gastrointestinal symptoms

 Mass
 History to exclude other differentials
• Metastatic :lung, liver, bone, peritoneal cavity (sarcomatosis)

• Physical examination: specially LN basins and testis(men)

 RPS cont…
Laboratory assessment
• Baseline tests
• LDH, β-HCG , α-fetoprotein
Imaging
• U/S , Contrast enhanced CT,MRI, Angiography
• Chest CT-for metastatic workup

• CT-guided core needle biopsy

– Unresectable tumour
– Doubtful diagnosis
– Neoadjuvent chemotherapy considered
 Staging

Histological grade (G)

G1 Well differentiated
G2 Moderately well differentiated
G3 Poorly or very poorly differentiated
Primary site (T)
T1 Tumor less than 5 cm in diameter
T1a Superficial tumor
T1b Deep tumor
T2 Tumor 5 cm or more in diameter
T2a Superficial tumor
T2b Deep tumor
N.B. Retroperitoneal and pelvic sarcomas are classified as deep
tumors
Nodal involvement (N)
N0 No histologically verified metastases to lymph nodes

N1 Histologically verified regional lymph nodes

Distant metastasis (M)

M0 No distant metastases
M1 Distant metastases present
 Staging summary

• Stage I- low grade

• Sage II- Intermediate grade
• Stage III- high grade or LN metastasis
• Stage IV- bone , vessele ,nerve inv’t or distant metastasis
 RPS Treatment

Surgery (TOC)
 Complete surgical resection+/- En block
 Debulking /palliative ??

Adjuvant Therapy
• Radiation therapy -preoperative
-For high risk patients defined as : large, high-grade tumors or recurrent low-
grade tumors
• Adjuvant chemotherapy
- based on the histology but overall response rate is low
• Prognosis for retroperitoneal sarcomas
 5 year survival after complete resection is 54-65%
 Drops to 10-36% if incompletely resected
 Recurrence occurs in 46-59% of completely resected tumors
 GASTROINTESTINAL STROMAL TUMORS
(GISTs)
• Most common visceral STS
• Share phenotypic similarities with the intestinal pacemaker cells known as
the interstitial cells of cajal
• Expression of the c-kit gene protein product, CD117, has emerged as an
important defining feature of GIST

• Can occur anywhere along the GIT , omentum, mesentery, peritoneum

• Common sites are stomach (60%) and small intestine (30%)
 Diagnosis…GIST
• Incidental
• Early satiety, mass,abdominal pain, or gastrointestinal bleeding

 Oral and IV contrast enhanced spiral CT

 FDG-PET
--Equivocal finding on CT or Metabolic response to therapy

 Endoscopy
 Treatment ..GIST
• Localized Disease
• Complete surgical resection with negative margins

• Locally Advanced or Metastatic Disease

• High response rates to Imatinib – tyrosine kinase inhibitor
 Breast Sarcoma
• Less than 1% of all breast malignancies and less than 5% of all STSs
• Different histology : angiosarcoma, stromal sarcoma,fibrosarcoma, and
MFH

Angiosarcoma
• 50% of all sarcomas of the breast
• Increasingly been associated with radiation therapy for treatment of
primary breast cancer (3-20 years)
• Expanding erythematous patch/bluish-black lesions, or bruise-like
discoloration overlying an area of induration
Breast angiosarcoma
 Breast sarcoma…cont

• Diagnosis requires punch or incisional biopsy.

Treatment
• Complete excision with negative margins is the primary
therapy
• LN dissection is not routinely indicated
• Neoadjuvant chemotherapy or radiation therapy may be
considered for patients with large, high-risk tumors
 DESMOIDS
• Arise from fibroblasts
• Most are sporadic, but may occur in association with familial cases
• Are most common in people between the ages of 15 and 60 years.
• They are more common in females than males

• Often appear as infiltrative, usually well-differentiated, firm overgrowths

of fibrous tissue,
• Are locally aggressive although they do not metastasize
 Desmoids cont…
• Originates from the musculoaponeurotic structures throughout the body
• Approximately half of these tumors arise in the extremities; the remaining
lesions are located on the trunk or in the retroperitoneum
• Pregnancy …
• Hard lump
 Treatment desmoids

• Surgery
 Function-sparing operations should be the goal, even if a positive margin is left on a
critical structures
• Radiation therapy
 Unresectable tumors or as adjuvant for recurrent diseases
• Systemic treatment
Another option when surgery is not indicated
 Tamoxifen
 Nonsteroidal anti-inflammatory drugs

• Chemotherapy
• Usually reserved for patients with tumor-associated symptoms who have not
responded to other interventions.
• Combinations of methotrexate and vinblastine
 PEDIATRIC SARCOMAS
• Relatively rare, accounting for 7% -8% of all pediatric cancers
• Totaling approximately 600 new cases per year

• Traditionally been divided into two groups:

 Rhabdomyosarcoma and
 Nonrhabdomyosarcoma
 Rhabdomyosarcoma
• Is a small round cell tumor that demonstrates muscle differentiation
• Most common soft tissue tumors among children younger than 15 years

 GUT-24%
 Extremities- 20%
 Head and neck- 20%
 Parameningeal region-16%
 Other sites- 22%

Categorized by histopathology into distinct subtypes:

 Embryonal, Alveolar, Pleomorphic, And Sclerosing/Spindle Cell Pathology
 RMS cont…
Clinically
 Painless / painful enlarging mass
 Metastasis
All patients are assumed to have micrometastatic disease at presentation
Distant metastasis at presentation( 15% to 20% ), most commonly
to the lungs, followed by bone marrow and bone
 Unlike other STSS, rhabdomyosarcomas have a high propensity for LN metastasis

Diagnosis
• Tissue –
small round cell tumor that demonstrates muscle differentiation upon light
microscopy and immunohistochemical analysis.
 RMS Treatment

• Complete surgical resection

 Treatment of choice when function and cosmesis can be preserved

• Chemotherapy
 Is recommended for all patients
 Combination regimens including
Vincristine,dactinomycin, and cyclophosphamide
Doxorubicin, ifosfamide, cisplatin, and etoposide

• Radiation therapy
 microscopic residual disease (group II) after resection.
THANK YOU!!!