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Sarcoma

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Soft-Tissue Sarcoma
Ewing's Sarcoma
Osteosarcoma
Malignant Fibrous Histiocytoma (MFH)
Chondrosarcoma

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Doxorubicin + Dacarbazine (AD)

DOXORUBICIN 60 mg/m2/day C.I. DAYS 1-5
DACARBAZINE 1,000 mg/m2/day C.I. DAYS 1-4
Repeat cycle Q21 days

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CyVADIC (Soft Tissue Sarcoma)

Cyclophosphamide 500 mg/m2 IVB day 1
Vincristine 1 mg/m2 IVB* days 1 & 5
Doxorubicin 50 mg/m2 IVB day 1
Dacarbazine 250 mg/m2 IV/1h days 1-5

Repeat cycle every 21 days.

*Maximum vincristine dose is 2 mg.

Ref: Yap B, et al: Cancer Treat Rep 64:93 (1980).

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MAID

Mesna 2500 mg/m2 IV days 1-4
Doxorubicin 20 mg/m2 IV days 1-3
Ifosfamide 2500 mg/m2 IV days 1-3
Dacarbazine 300 mg/m2 IV days 1-3

Note: All drugs are administered by continuous infusion, doxorubicin is mixed with dacarbazine, and ifosfamide is mixed with mesna. Repeat treatment every 21 days.

Ref: Elias A, Ryan L, Sulkes A, et al; J Clin Oncol 7:1208-1216, 1989.

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A-DIC

Note: Where a dose range is given, the flowsheet
uses the lowest value.

Doxorubicin 45 - 60 mg/m2 IVB day 1
Dacarbazine 200-250 mg/m2 IV/1h days 1-5

Repeat cycle every 3 weeks.

Ref: Baker LH, et al: J Clin Oncol 5:851 (1987).
Borden EC, et al: J Clin Oncol 5:840 (1987).

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Ewing's Sarcoma
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CyVADIC - 2

Cyclophosphamide 600 mg/m2 IVB day 1
Vincristine 1.4 mg/m2 IVB* weekly X 6
then 1.4 mg/m2 IVB* day 1 each cycle

Doxorubicin 15 mg/m2 CIV days 1-4
Dacarbazine 250 mg/m2 CIV days 1-4
OR
Doxorubicin 22.5 mg/m2 CIV days 1-4
Dacarbazine 225 mg/m2 CIV days 1-4


Repeat cycle every 21 to 28 days for 18 months.

*Maximum vincristine dose is 2 mg.

At total doxorubicin dose (450 mg/m2), substitute dactinomycin 2 mg/m2 on day 1 of each cycle.

Ref: Benjamin RS: In RT Skeel (ed):Handbook of Chemotherapy, Boston, Little Brown, p 203 (1987).

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NCI Web Site for Adult Soft-tissue Sarcoma

NCI Web site for Ewing's Sarcoma

NCI web site for Osteosarcoma

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How to Distinguish PNET & Ewing’s from other small round cell tumors?

The monoclonal antibody, HBA71, recognizes a cell-surface glycoprotein (p30/32MIC2) in human Ewing’s sarcoma & PNET. The strong immunoreactivity of HBA71 in Ewing’s sarcoma & PNET distinguishes these tumors from other small round cell tumors of childhood & adolescence.
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PROGNOSTIC FACTORS:

Patients with retroperitoneal, head and neck, and visceral sarcomas have a worse overall prognosis than do patients with extremity tumors.

High-risk patient with extremity soft-tissue sarcoma: an individual with a large ( >5 cm), high-grade, deep lesion.

Unlike other solid tumors, the adverse prognostic factors for local recurrence of a soft-tissue sarcoma differ from those that predict distant metastasis & tumor-related mortality.
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Notes on treatment:

1. TNF-based isolated limb perfusion (ILP) appears to be an effective option for limb salvage in patients with locally advanced extremity soft-tissue sarcoma.
REF= Eggermont AMM et al: Proc Am Soc Clin Oncol 18:535a [abstract], 1999.

2. XRT for inoperable tumors: Radiation doses should be at least 65-70 Gy. 5-year survival rates of 25%-40% with local control rates of 30% have been reported.

3. ADJUVANT XRT. Radiation therapy is always combined with surgical resection in the management of extremity soft-tissue sarcomas. No advantage for pre-op XRT. In fact, more wound complications noted! Limb-sparing surgery + postoperative XRT produces comparable local control rates to those achieved with amputation. 5-yr local control rates of 70%-90%, survival rates of 70%, & limb preservation rates of 85% can be expected.

Therefore, Adjuvant XRT should be employed for virtually all high-grade extremity sarcomas & larger (>5 cm) low-grade lesions. If small (T1) lesions can be resected with clear margins, XRT can be omitted

Pre-operative chemotherapy should be considered for fit, high-risk patients.

Thoracotomy & metastectomy should be considered in patients with limited pulmonary metastases & no extrapulmonary diseases. 3-yr survival following thoracotomy for pulmonary metastectomy ranges between 23% & 42%.

Misc
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. Translocation t(11;22) EWS-FLII genes seen in 90% of patients with Ewing's Sarcomas & PNET