Soft Tissue Sarcomas
Group Members:
Agu, Hilary Hortelano, Yvonne
Castañeda, Marie Claire Pagalilauan, Rogeniv
Cometa, Fio...
OVERVIEW
• Sarcomas
– heterogeneous group of tumors that arise
predominantly from the embryonic
mesoderm, but also can ori...
iNCIDENCE
• Rare tumor
• <1% - in adults (estimated 10,000 cases per
year in the US)
• 7% - in children
Most common histologic
types in adults (excluding
Kaposi’s sarcoma)
Most common
in childhood
• Approximately two thirds originate in the
extremities (50 to 60%)
• Remaining one third:
• retroperitoneum
• Trunk
• Abd...
• Overall 5-year survival rate for patients with
all stages: 50 to 60%.
• Of the patients who die of sarcoma, most will
su...
EPIDEMIOLOGY
• The dominant pattern of metastasis is
hematogenous, primarily to the lungs.
• Lymph node metastases are rar...
RISK FACTORS
1. Radiation exposure
• External radiation therapy
• 8 – 50 fold increase in incidence: txt in
breast, cervix...
RISK FACTORS
3. Trauma
• no causal relationship has been established
4. Chronic lymphedema
• Lymphangiosarcoma : has been ...
GENETICS
Classification into two main groups:
1. with defined diagnostic
molecular events
• found to be younger with a
def...
Oncogene Activation
• Oncogenes are genes that can induce malignant
transformation and tend to drive cells toward
prolifer...
Tumor Suppressor Genes
• play a critical role in growth inhibition and can
suppress growth in cancer cells.
• Inactivation...
Two genes that are most relevant to soft tissue tumors:
1. retinoblastoma (Rb) tumor suppressor gene
• Mutations or deleti...
INITIAL ASSESSMENT
Clinical Presentation
• asymptomatic mass – most common presentation
• Less common presentation of an e...
Clinical Presentation
• often grow in a centrifugal fashion and compress surrounding normal
structures.
• Infrequently, th...
Differential diagnosis
I. Benign lesions :
1. lipomas (100 times more common than sarcoma)
2. lymphangiomas
3. Leiomyomas
...
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1 soft tissue sarcomas

  1. 1. Soft Tissue Sarcomas Group Members: Agu, Hilary Hortelano, Yvonne Castañeda, Marie Claire Pagalilauan, Rogeniv Cometa, Fiona Rose Saguigit, Joshua Eder, Richton Torida, Chinkee Ganga, Swathy Tulauan, Kelvin
  2. 2. OVERVIEW • Sarcomas – heterogeneous group of tumors that arise predominantly from the embryonic mesoderm, but also can originate from the ectoderm (eg. peripheral nervous system) – can occur throughout the body and encompass more than 50 subtypes with distinct histologic lines of differentiation
  3. 3. iNCIDENCE • Rare tumor • <1% - in adults (estimated 10,000 cases per year in the US) • 7% - in children
  4. 4. Most common histologic types in adults (excluding Kaposi’s sarcoma) Most common in childhood
  5. 5. • Approximately two thirds originate in the extremities (50 to 60%) • Remaining one third: • retroperitoneum • Trunk • Abdomen • Head & neck
  6. 6. • Overall 5-year survival rate for patients with all stages: 50 to 60%. • Of the patients who die of sarcoma, most will succumb to metastatic disease • 80% of the time occurs within 2 to 3 years of the initial diagnosis.
  7. 7. EPIDEMIOLOGY • The dominant pattern of metastasis is hematogenous, primarily to the lungs. • Lymph node metastases are rare (<5%) except for a few histologic subtypes such as: epithelioid sarcoma, rhabdomyosarcoma, clear-cell sarcoma, synovial sarcoma, MFH, angiosarcoma
  8. 8. RISK FACTORS 1. Radiation exposure • External radiation therapy • 8 – 50 fold increase in incidence: txt in breast, cervix, ovary, testes and lymphatic system 2. Occupational chemicals • Herbicides (phenoxyacetic acid), wood preservatives cont’g. chlorophenols • Thorium oxide, vinyl chloride, arsenic - assoc. with hepatic angiosarcomas
  9. 9. RISK FACTORS 3. Trauma • no causal relationship has been established 4. Chronic lymphedema • Lymphangiosarcoma : has been reported as occurring after filarial infections and in the lower extremities of patients with congenital or heritable lymphedema.
  10. 10. GENETICS Classification into two main groups: 1. with defined diagnostic molecular events • found to be younger with a defined histology, suggesting a clear line of differentiation. • The defined molecular events include: – point mutations, – a translocation causing overexpression of an autocrine growth factor ,or – oncogenic fusion transcription factor. 2. with variable histologic and genetic changes. • without currently identifiable genetic changes or expression profile signatures tend to occur in older patients exhibiting pleomorphic cytology and p53 dysfunction.
  11. 11. Oncogene Activation • Oncogenes are genes that can induce malignant transformation and tend to drive cells toward proliferation. • Several oncogenes have been identified in association with soft tissue sarcomas • MDM2 • N-myc • c-erbB-2 • Members of the ras family
  12. 12. Tumor Suppressor Genes • play a critical role in growth inhibition and can suppress growth in cancer cells. • Inactivation of tumor suppressor genes (also known as anti-oncogenes) can occur through hereditary or sporadic mechanisms.
  13. 13. Two genes that are most relevant to soft tissue tumors: 1. retinoblastoma (Rb) tumor suppressor gene • Mutations or deletions in Rb can lead to development of retinoblastoma or sarcomas of soft tissue and bone. 2. p53 tumor suppressor gene • Mutations in the p53 tumor suppressor gene are the most common mutations in human solid tumors • have been reported in 30 to 60% of soft tissue sarcomas
  14. 14. INITIAL ASSESSMENT Clinical Presentation • asymptomatic mass – most common presentation • Less common presentation of an extremity sarcoma may be a deep venous thrombosis, particularly in patients without significant risk factors for thrombosis. • Tumor size at presentation usually is associated with the location of the tumor. – Smaller tumors generally are located in the distal extremities – tumors in the proximal extremities and retroperitoneum can grow quite large before becoming apparent.
  15. 15. Clinical Presentation • often grow in a centrifugal fashion and compress surrounding normal structures. • Infrequently, their impingement on bone or neurovascular bundles produces • pain, edema, and swelling • Retroperitoneal soft tissue sarcomas almost always present as large asymptomatic masses. • Less frequently, patients present with obstructive GI symptoms or neurologic symptoms related to compression of lumbar or pelvic nerves.
  16. 16. Differential diagnosis I. Benign lesions : 1. lipomas (100 times more common than sarcoma) 2. lymphangiomas 3. Leiomyomas 4. Neuromas II. Other malignant lesions 1. Primary or metastatic carcinomas 2. Melanomas 3. Lymphomas • Small lesions that have not changed for several years by clinical history may be closely observed. • All other tumors should be considered for biopsy to establish a definitive diagnosis.

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