Soft Tissue Sarcoma Rapid Fire: Update Course 2015
Half of soft tissue sarcomas are rhabdomyosarcomas, and the other half are non-rhabdomyosarcomas.
epidemiologicalAndrea0:00 ↗
Prognosis in rhabdomyosarcoma varies depending on site, with abdominal, pelvic, and retroperitoneal tumors having the worst survival and orbital rhabdomyosarcomas having the best survival.
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Stage 1 rhabdomyosarcoma includes favorable sites (vaginal and paratesticular), while stages 2, 3, and 4 are unfavorable sites with various amounts of disease.
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Even a small tumor in the extremity or abdomen can only achieve stage 2 at best.
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Clinical grouping in rhabdomyosarcoma depends on surgical extent: group 1 is complete removal, group 2 is microscopic residual, and group 3 is biopsy only.
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Group 1 and 2 rhabdomyosarcoma patients have an excellent prognosis, while group 3 patients do more poorly.
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A large tumor that a surgeon cannot resect is a biologic determination, and attempting resection with high morbidity will not improve the patient's outcome.
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Five centimeters is the cutoff for low-risk versus high-risk patients in soft tissue sarcoma.
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Sentinel lymph node biopsies are now required for all rhabdomyosarcoma patients with trunk or extremity tumors.
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In rhabdomyosarcoma, completion node dissection is not performed after sentinel lymph node biopsy; patients receive radiation therapy instead.
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Alveolar rhabdomyosarcoma patients should receive chemotherapy after biopsy, which typically reduces the tumor to approximately 1 centimeter, making resection with negative margins easier.
clinicalAndrea3:53 ↗
Sentinel lymph node mapping should be performed at the time of re-excision in rhabdomyosarcoma.
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Forty to fifty percent of biopsied lymph nodes are positive in rhabdomyosarcoma.
epidemiologicalAndrea4:39 ↗
Clinically negative lymph nodes may be positive in rhabdomyosarcoma, which is why sentinel node biopsy is performed.
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Positive lymph nodes in rhabdomyosarcoma have a worse prognosis and require radiation therapy.
clinicalAndrea4:39 ↗
In non-rhabdomyosarcoma soft tissue sarcoma, histological grade is now critical for treatment planning.
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Large soft tissue sarcoma tumors should be biopsied first and treated with chemotherapy before resection.
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Soft tissue sarcomas have been identified as chemotherapy-sensitive or chemotherapy-insensitive, which changes treatment approach.
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Low-grade soft tissue sarcoma tumors require only resection and observation, with no need for radiation or chemotherapy.
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If a low-grade soft tissue sarcoma is resected with positive margins, the patient will receive adjuvant radiation therapy.
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Unresectable large soft tissue sarcomas should be treated with preoperative radiation therapy and preoperative chemotherapy (previously only chemotherapy was recommended).
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Small soft tissue sarcoma tumors less than 5 centimeters should be resected with attempts to achieve negative margins.
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Chemotherapy-sensitive soft tissue sarcomas include synovial sarcoma and undifferentiated sarcoma.
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Chemotherapy-insensitive soft tissue sarcomas include alveolar soft part sarcoma, malignant epithelioid sarcoma, and clear cell sarcoma, all of which are high-grade tumors.
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For chemotherapy-insensitive soft tissue sarcomas, the surgeon and radiation therapist are the primary treaters.
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High-risk soft tissue sarcoma patients receive preoperative radiation and chemotherapy.
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An Italian study showed that a 5 centimeter tumor in a child less than 3 years old is equivalent to a 3 centimeter tumor in larger patients, suggesting 3 centimeters as the cutoff for excision versus biopsy in toddlers.
Host summaryAndrea summarizing the discussion — not the host's own clinical position6:54 ↗
Sentinel lymph node biopsy should always be performed if lymph nodes are clinically negative by imaging.
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Positive lymph nodes receive radiation to the lymph node basin, and completion node dissection is not required.
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Non-rhabdomyosarcoma tumors with negative margins after resection do not need chemotherapy if they are low-grade.
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