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)