Now what other special situations do you need to know about for the Boards, in terms of sarcomas? Well, desmoids which are mostly low grade, are mostly locally invasive. As I told you before, they are associated with the APC gene, Gardener’s syndrome. The treatment of choice is usually a wide excision with negative margins, if possible. But if you can’t achieve negative margins because there are vital structures in the area, then you ought to at least obtain the best margins you can and then the patient can either receive radiation therapy or, if the patient has a recurrence, they can then be re-resected and have radiation therapy. Then those who have further growth of tumor, you can treat them – there’s data for use with NSAID’s, with tamoxifen, low dose Velban with methotrexate or Adriamycin/DTIC. A recent article in your handout, for those patients who get into problems with neurotoxicity with Velban, you can substitute vinorelbine, Navelbine. That’s a recent article that came out in the American Journal of Clinical Oncology that’s in your handout.
Leiomyosarcomas, GI stromal, have an extremely poor response to chemo. I don’t know what to do for those. You need, if possible, to get them to a surgeon who does a lot of these the first time so that the tumor can be fully resected. Because if the tumor is not fully resected and the patient has positive margins, they are going to do extremely poorly. Those patients with uterine or extremity leiomyosarcomas, you may consider using Adriamycin/ifosfamide or specifically for the uterine sarcomas, continuous infusion DTIC at a dose of about 300 mg per meter squared over approximately seven days. Myxoid liposarcomas; you need to be aware that these have a very high incidence of extrapulmonary metastases and that for this specific sarcoma, soft tissue sarcoma, in addition to getting chest CT’s you need to get abdominal CT’s because they can have occurrences in the retroperitoneal area and other areas.

Clear cell sarcoma behaves more like a melanoma and is HB-45 positive, so they should be treated like a melanoma. Breast sarcomas; the treatment of choice is doing a total mastectomy, no axillary node dissection, because the incidence of lymph node metastasis is extremely rare.

So to review here, for soft tissue sarcomas, incisional or core biopsy. If you are at an institution that can do these, I would recommend core. To do a resection, wide if possible with at least 1-2 cm margins at minimum. Postoperative radiation therapy most of the time if the margins are not good, but there may be some patients now who don’t require postoperative radiation. That will depend on the location, grade, size, and margin status. Consider preoperative radiation for a very large tumor mass or possibly neoadjuvant chemo. Metastasectomy is beneficial in a select group of patients. In terms of chemotherapy, either the Adriamycin/ifosfamide regimen or MAID plus growth factors. There is an increased response rate but no survival advantage. If you are just palliating and it’s an elderly patient, just Adriamycin alone or Adriamycin plus DTIC, probably given as a continuous infusion where you are going to reduce cardiotoxicity and probably nausea and vomiting. Adjuvant therapy is unproven for soft tissue, except maybe for extremity, for large, deep, high grade extremity lesions. And neoadjuvant induction chemotherapy is now being looked at but certainly isn’t standard. We do it at our institution, but I would probably refer a patient into a center for that, if it’s a very large lesion that needs to be down-staged prior to surgery.