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Dr. Joseph A. Di Como
Introduction
Group of anatomically and histologically diverse
tumors of extraskeletal mesenchymal origin.
Rare: Account ...
Location and Type
Etiology
History of radiation therapy increases risk, grade of
tumors and risk for metastasis.
Chemical exposure
 Thoro...
Classification
Soft tissue and bone
 viscera (gastrointestinal, genitourinary, and gynecologic organs)
 nonvisceral sof...
Biopsy
Most present as painless mass leading to delayed diagnosis
– Mistaken for lipoma, hematoma, muscle injury
Core ne...
Imaging
MRI
 For extremity masses
 Gives good delineation between muscle, tumor and blood
vessels
CT for abdominal and...
Staging
AJCC/UICC Staging System for Soft Tissue Sarcomas
 T1: <5cm
 T1a: superficial to muscular fascia
 T1b: Deep to...
Staging
Stage IA G1,2 T1a,b N0 M0
Stage IB G2,2 T2a,b N0 M0
Stage IIA G3,4 T1a,b N0 M0
Stage IIB G3,4 T2a N0 M0
Stage III ...
Survival by stage
Relative risk for recurrence and survival
Age >50 years 1.6
Local recurrence at presentation 2.0
Microscopically positi...
Surgery
Limb-sparing vs amputation
 Comparison study with post-op radiation in limb sparing
showed no difference in surv...
Resection
Arbitrary 2 cm margin if no plan for post-op
radiotherapy
Negative margins may be adequate for post-op
radiati...
Adjuvant radiotherapy
Small, low grade tumors resected with 2 cm margins
may not require radiation
Improves local contro...
Local recurrence with post-op brachytherapy
Pre-op or post-op radiation?
Some avoid pre-op use because of increased wound
complications (although this is debatable)
...
Pre-op vs post-op radiotherapy
Chemotherapy
Can improve local control, but not survival
Doxorubicin and ibosfamide have response rates of
20%
Use only...
Treatment of Recurrence
20-30% of STS patients will recur
More common in retroperitoneal and head & neck
high grade tumo...
Metastatic disease
Lung most common site of mets, but visceral often go
to liver
Median survival from development of met...
Case #1
64 y/o male with increasing abdominal girth
Retroperitoneal Sarcomas
15% of all sarcomas
Liposarcoma 42% and leiomyosarcoma 26%
CT scan can show cystic/solid/necro...
Liposarcoma
Survival after resection of primary
retroperitoneal sarcoma
Prognosis for retroperitoneal sarcomas
5 year survival after complete resection of 54-65%
 Drops to 10-36% if incomplete...
Radiation or chemotherapy for retroperitoneal
sarcomas
Radiation
GI and neurotoxicities limit delivery of sufficient dos...
Case #2
• 49 y/o female with GERD undergoing EGD
GIST
Separate subtype of sarcoma defined by expression of c-
Kit (CD117)
Surgery: complete resection without local or re...
GIST
GIST
Extremity sarcomas
MFH
Synovial sarcoma
Breast sarcomas
1% of all breast neoplasms
Wide excision with negative margins
No clear role for adjuvant radiotherapy
Sarcoma after mastectomy
Vascular sarcomas
Angiosarcoma, hemangiosarcoma,
lymphangiosarcoma, hemangiopericytoma
Key points:
 Hepatic angiosarcom...
References
www.Dartmouth-Hitchcock.org
The Johns Hopkins ABSITE Review Manual. By
Robert A. Meguid, Kyle Van Arendonk, P...
Soft tissue sarcomas, treatment (surgical, radiation, chemotherapy)
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Soft tissue sarcomas, treatment (surgical, radiation, chemotherapy)

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A PowerPoint presentation for medical professionals regarding soft tissue sarcomas, likely most helpful to surgical residents and medical students. Gist tumors, liposarcomas, retroperitoneal sarcomas extremity, breast sarcoma and vascular sarcomas

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Soft tissue sarcomas, treatment (surgical, radiation, chemotherapy)

  1. 1. Dr. Joseph A. Di Como
  2. 2. Introduction Group of anatomically and histologically diverse tumors of extraskeletal mesenchymal origin. Rare: Account for about 1% of adult malignancies and approximately 9,000 to 12,000 cases reported every year in the United States 3,000 to 4,000 deaths annually from STS in the United Stated
  3. 3. Location and Type
  4. 4. Etiology History of radiation therapy increases risk, grade of tumors and risk for metastasis. Chemical exposure  Thorotrast, vinyl chloride, arsenic for hepatic angiosarcoma Genetic syndromes  Neurofibromatosis – nerve sheath tumors  Li Fraumeni Syndrome (germline mutation in p53)  Hereditary retinoblastoma (germline mtuation in Rb)  Familial adenomatous polyposis/Gardner’s syndrome  Familial gastrointestinal stromal tumor syndrome – KIT mutation  Skin hyperpigmentation, uticaria, cutaneous mast cell dx
  5. 5. Classification Soft tissue and bone  viscera (gastrointestinal, genitourinary, and gynecologic organs)  nonvisceral soft tissues (muscle, tendon, adipose, pleura, and connective tissue) By differentiation (usually with IHC staining)  adipocytic tumors  fibroblastic/myofibroblastic tumors  fibrohistiocytic tumors  smooth muscle tumors  pericytic (perivascular) tumors  primitive neuroectodermal tumors (PNETs)  skeletal muscle tumors  vascular tumors  osseous tumors  tumors of uncertain differentiation
  6. 6. Biopsy Most present as painless mass leading to delayed diagnosis – Mistaken for lipoma, hematoma, muscle injury Core needle biopsy guided by palpation or by image guidance if not palpable  Few cases of tumor seeding with closed biopsy so some recommend tattooing site for later excision with specimen Excisional biopsy for superficial small lesions if needle biopsy non-diagnostic Incision biopsy  Longitudinal incision without tissue flaps with meticulous hemostasis to prevent tumor seeding in hematomas  Send biopsy fresh and orientated
  7. 7. Imaging MRI  For extremity masses  Gives good delineation between muscle, tumor and blood vessels CT for abdominal and retroperitoneal PET  May help determine high vs. low grade  May be helpful in recurrences
  8. 8. Staging AJCC/UICC Staging System for Soft Tissue Sarcomas  T1: <5cm  T1a: superficial to muscular fascia  T1b: Deep to muscular fascia  T2: >5cm  T2a: superficial to muscular fascia  T2b: Deep to muscular fascia  N1: Regional nodal involvement  Grading  G1: Well-differentiated  G2: Moderately differentiated  G3: Poorly differentiated  G4: Undifferentiated
  9. 9. Staging Stage IA G1,2 T1a,b N0 M0 Stage IB G2,2 T2a,b N0 M0 Stage IIA G3,4 T1a,b N0 M0 Stage IIB G3,4 T2a N0 M0 Stage III G3,4 T2b N0 M0 Stage IV Any G Any T N1 M1 **Does not take into account extremity vs. visceral Staging system predicts survival and risk of metastasis, but not local recurrence
  10. 10. Survival by stage
  11. 11. Relative risk for recurrence and survival Age >50 years 1.6 Local recurrence at presentation 2.0 Microscopically positive margin 1.8 Size 5.0–10.0 cm 1.9 Size > 10.0 cm 1.5 High-grade 4.3 Deep location 2.5 Local recurrence 1.5
  12. 12. Surgery Limb-sparing vs amputation  Comparison study with post-op radiation in limb sparing showed no difference in survival Amputation still may be indicated for neurovascular or bone involvement
  13. 13. Resection Arbitrary 2 cm margin if no plan for post-op radiotherapy Negative margins may be adequate for post-op radiation therapy  Presence of positive margins increases local recurrence by 10- 15% No need for lymph node dissection as only 2-3% have nodal metastasis
  14. 14. Adjuvant radiotherapy Small, low grade tumors resected with 2 cm margins may not require radiation Improves local control but not survival Whether improved local control leads to improved survival is controversial
  15. 15. Local recurrence with post-op brachytherapy
  16. 16. Pre-op or post-op radiation? Some avoid pre-op use because of increased wound complications (although this is debatable)  RCT looking at wound complication rate pre-op vs post-op radiation showed 35% vs 17%  Risk confined to lower extremity  Conclusions: pre-op may be better for upper extremity and head & neck because of equal wound complication risk and benefit of lower radiation doses to more vital tissues
  17. 17. Pre-op vs post-op radiotherapy
  18. 18. Chemotherapy Can improve local control, but not survival Doxorubicin and ibosfamide have response rates of 20% Use only in advanced disease Combination with radiation or neoadjuvant therapy are controversial Hypothermic isolated limb perfusion may be used for palliation
  19. 19. Treatment of Recurrence 20-30% of STS patients will recur More common in retroperitoneal and head & neck high grade tumors because hard to get clear margins  38% for retroperitoneal  42% for head and neck  5-25% for extremity After re-resection recurrence is 32% for extremity and much higher for visceral
  20. 20. Metastatic disease Lung most common site of mets, but visceral often go to liver Median survival from development of metastatic disease is 8-12 months Resection of pulmonary mets can give 5 year survival of 32% if all mets can be removed  >3 mets is poor prognosticator
  21. 21. Case #1 64 y/o male with increasing abdominal girth
  22. 22. Retroperitoneal Sarcomas 15% of all sarcomas Liposarcoma 42% and leiomyosarcoma 26% CT scan can show cystic/solid/necrotic components and relation to surroundings CXR to r/o mets, chest CT if CXR abnormal Biopsy not necessary unless suspect a lymphoma or germ cell tumor or plan preop chemo or radiation En bloc resection is standard treatment  bowel prep  assess bilateral kidney function  50-80% need organ resection  78% of primary lesions can be completely resected
  23. 23. Liposarcoma
  24. 24. Survival after resection of primary retroperitoneal sarcoma
  25. 25. Prognosis for retroperitoneal sarcomas 5 year survival after complete resection of 54-65%  Drops to 10-36% if incompletely resected Recurrence occurs in 46-59% of completely resected tumors
  26. 26. Radiation or chemotherapy for retroperitoneal sarcomas Radiation GI and neurotoxicities limit delivery of sufficient doses May improve local control Recommended for use only in clinical trials given lack of data either way Chemotherapy Use for recurrent, unresectable or metastatic disease
  27. 27. Case #2 • 49 y/o female with GERD undergoing EGD
  28. 28. GIST Separate subtype of sarcoma defined by expression of c- Kit (CD117) Surgery: complete resection without local or regional lymphadenectomy Very resistant to traditional chemotherapy Gleevec (imantinib mesylate)  c-Kit is constitutively active tyrosine kinase receptor  Drug is tyrosine kinase inhibitor used in CML  Initial studies showed 54% response rates  Two RCTs currently looking at adjuvant treatment
  29. 29. GIST
  30. 30. GIST
  31. 31. Extremity sarcomas MFH Synovial sarcoma
  32. 32. Breast sarcomas 1% of all breast neoplasms Wide excision with negative margins No clear role for adjuvant radiotherapy
  33. 33. Sarcoma after mastectomy
  34. 34. Vascular sarcomas Angiosarcoma, hemangiosarcoma, lymphangiosarcoma, hemangiopericytoma Key points:  Hepatic angiosarcoma – thorotrast, vinyl chloride, arsenic  Stewart Treve’s – lymphangiosarcoma in chronic lymphedema High risk for bleeding during excision No clear role for chemo or radiation
  35. 35. References www.Dartmouth-Hitchcock.org The Johns Hopkins ABSITE Review Manual. By Robert A. Meguid, Kyle Van Arendonk, Pamela A. Lipsett
  • DRAMNAHASSAN

    Jul. 24, 2021
  • MutaleMwenya1

    Feb. 28, 2021
  • AsiyaShaikh22

    Aug. 9, 2020
  • VishnuAnilKumar12

    Feb. 16, 2020

A PowerPoint presentation for medical professionals regarding soft tissue sarcomas, likely most helpful to surgical residents and medical students. Gist tumors, liposarcomas, retroperitoneal sarcomas extremity, breast sarcoma and vascular sarcomas

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