Sarcomas -- Evaluation
Often is referred as “lipoma” or a “trauma” Size:
<5 , > 5, > 10 cm Location:
Extremity --> lung deep to fascia more often high grade
Head and neck --> lung GI or retroperitoneal --> liver
Sarcoma -- Nodal Metastases
Nodal metastases are rare Clinical assessment only Stage IV when present No special efforts for node basin resection
Sarcomas -- Pre-op imaging
CT scan or MRI Plan for post-op radiation bed (if needed) Allow 3-D visualization of tumor for resection
Extremity or head and neck: CXR (if low grade sarcoma on biopsy) CT chest (if high grade sarcoma on biopsy)
Sarcomas -- Biopsy
Tru-Cut Core biopsy CT guided if intra- or retro-peritoneal
Open biopsy Incisional only
Longitudinal Biopsy incision must be excised at final excision
FNA inadequate
Sarcomas -- Staging
Stage I Grade 1 (G1) -- Well differentiated
Stage IA -- < 5cm Stage IB -- > 5 cm
Stage II Grade 2 (G2) -- Moderately well differentiated
Stage IIA -- < 5cm Stage IIB -- > 5 cm
Sarcomas -- Staging
Stage III Poorly differentiated
Stage IIIA -- < 5cm Stage IIIB -- > 5 cm
Stage IV Nodal (IVA) or distant (IVB) metastases
Any grade and any size
Sarcomas -- Neoadjuvant chemo
More effective in children Ifosfamide and adriamycin (or epirubicin)
Chemo mainstay in some sarcomas Ewing’s sarcoma, embryonal rhabdomyosarcoma,
most osteogenic sarcomas
Sarcomas -- Neoadjuvant chemo
Controversial in many sarcomas
High grade sarcomas -- some sensitive MFH, liposarcoma, synovial cell sarcoma
High grade sarcomas -- some resistant GIST, leiomyosarcoma, epithelioid,
hemangiopericytoma, extraskeletal myxoid chrondrosarcomas
Sarcomas -- Neoadjuvant chemo
Allows chemosensitivity assessment pre-op Allows pathologic chemo-necrosis
assessment Micro-metastasis treatment earlier Surgical bulk reduction
Important in tumors > 10 cm
Sarcomas -- Radiation
Intermediate or high grade sarcomas Extremity Head and Neck
No difference in pre-op or post-op Wide field, 5000 - 5500 Gy Spare area of skin to prevent
circumferential stricture
Sarcomas -- Radiation
Brachytherapy -- iridium Catheters should be placed at time of resection
Sarcomas -- Surgery
2 to 3 cm margins -- do not violate tumor Some recommend 3 - 5 cm -- microsatellites One uninvolved fascial plane Include skin, SQ tissue, adjacent soft tissue, and
incision site, sometimes periosteum, bone ctx. Preserve neurovascular bundle if low grade
Excise adventitia of artery, vein, nerve if necessary Frozen section of margins as needed
Sarcomas -- Surgery
With post-op radiation, full compartment resection not necessary
Function and limb sparing achievable with post-op radiation
Post-op coverage Flaps Eliminate dead space, compression, drains
Retroperitoneal Sarcoma -- Surgery
45 degree positioning in OR Incision -- 11th rib extended to paramedian Rotate colon, duodenum (Kocher), or other
viscera Sacrifice kidney, involved organs as needed
Retroperitoneal sarcoma -- radiation
Vena Cava -- radiation helpful ?
Intra-abdominal Sarcomas -- Surgery
Total gastrectomy not necessary Adequate margins needed, however
Sarcomas -- Metastasis treatment
Metastases seen in 25% of sarcomas Lungs are site of metastases in 75%
Sarcomas -- Metastasis treatment
Lung -- improved outcome with resection Resect isolated/localized met (s) only Multiple met resection in experimental settings
only Liver -- few survivors
Do not resect Chemoembolization, cryoablation, RF ablation
Sarcomas -- Recurrence treatment
Recurrence in 1/3 of sarcoma patients Re-resect with appropriate margins 20 - 30% of extremities require amputation
Sarcomas -- Survival
Small low-grade tumors -- > 90% disease free survival
Large, high grade tumors -- 20 - 30% overall survival
Sarcomas -- Follow-up
Extremity and head and neck CXR and extremity MRI/CT every 6 months
for 5 years Retroperitoneal and intra-abdominal
CT every 6 months for 3 years then yearly
Sarcomas -- Screening
Neurofibromatosis (vonRecklinghausen) 3-10% lifetime risk
Familial adenomatous polyposis Li-Fraumeni (p53) -- mother at risk for breast
CA Retinoblastoma, HIV (Kaposi’s) Prior radiation (radium, thorium) Arsenic, dioxin (Agent Orange)