Follow-up Therapy for Metastatic Endometrial Stromal Sarcoma?
A 23-year-old woman diagnosed with endometrial stromal sarcoma underwent laparotomy; debulking was not complete. Pelvic radiotherapy and chemotherapy with doxorubicin/ifosfamide was recommended. Before beginning therapy, she complained of severe pelvic pain and urinary discomfort; continued symptoms of lower-limb weakness, neuropathic pain, and urinary retention indicated spinal-cord compression. MRI revealed metastatic disease of T9, T11, L1, and L2 vertebral bodies, with massive spinal channel occupation. Six cycles of 25 mg/m doxorubicin every 3 days and 2000 mg/m ifosfamide every 5 days were given over a 21-day interval. Pain control was achieved by the second cycle. Repeat MRI at the end of the third cycle showed no spinal channel infiltration. Urinary sphincter-control recovery was complete by the fifth cycle. Pelvic CT at the end of chemotherapy was normal and MRI showed only residual bone commitment at known vertebral sites. No other sites were found with metastatic disease. What would you recommend at this point?
The case describes a patient with endometrial stromal sarcoma treated with suboptimal debulking surgery followed by adjuvant chemotherapy with doxorubicin and ifosfamide for 6 cycles with a complete response by symptoms and at least partial response by radiographic studies. If the residual bone commitment describes persistent disease, then one could consider treatment with radiation therapy.
The use of radiation therapy has been reported to reduce local relapse in patients with uterine sarcoma. In a recent report by Soumarova and colleagues in which 22% of the patients included endometrial stromal sarcoma, an improvement in disease-free interval was observed in patients who received adjuvant radiation therapy. Although this study also suggests an improvement in overall survival, others demonstrate an improvement in local control with adjuvant radiation therapy but no improvement in overall survival. In this patient with residual bone commitment, directed radiation therapy may be considered.
By FIGO staging criteria, the patient described would be considered stage IV because of bony metastases. Unfortunately, this carries a grave prognosis. A pathologic study of the tumor may be helpful in directing adjuvant therapy. Endometrial stromal sarcomas rich in estrogen and progesterone receptors have shown objective response rates to hormonal treatments such as megestrol acetate, and, more recently, to the use of aromatase inhibitors. Because of the excellent side-effect profile of these agents, one may consider adjuvant treatment with either an aromatase inhibitor and/or progestin therapy in this setting.
A 23-year-old woman diagnosed with endometrial stromal sarcoma underwent laparotomy; debulking was not complete. Pelvic radiotherapy and chemotherapy with doxorubicin/ifosfamide was recommended. Before beginning therapy, she complained of severe pelvic pain and urinary discomfort; continued symptoms of lower-limb weakness, neuropathic pain, and urinary retention indicated spinal-cord compression. MRI revealed metastatic disease of T9, T11, L1, and L2 vertebral bodies, with massive spinal channel occupation. Six cycles of 25 mg/m doxorubicin every 3 days and 2000 mg/m ifosfamide every 5 days were given over a 21-day interval. Pain control was achieved by the second cycle. Repeat MRI at the end of the third cycle showed no spinal channel infiltration. Urinary sphincter-control recovery was complete by the fifth cycle. Pelvic CT at the end of chemotherapy was normal and MRI showed only residual bone commitment at known vertebral sites. No other sites were found with metastatic disease. What would you recommend at this point?
The case describes a patient with endometrial stromal sarcoma treated with suboptimal debulking surgery followed by adjuvant chemotherapy with doxorubicin and ifosfamide for 6 cycles with a complete response by symptoms and at least partial response by radiographic studies. If the residual bone commitment describes persistent disease, then one could consider treatment with radiation therapy.
The use of radiation therapy has been reported to reduce local relapse in patients with uterine sarcoma. In a recent report by Soumarova and colleagues in which 22% of the patients included endometrial stromal sarcoma, an improvement in disease-free interval was observed in patients who received adjuvant radiation therapy. Although this study also suggests an improvement in overall survival, others demonstrate an improvement in local control with adjuvant radiation therapy but no improvement in overall survival. In this patient with residual bone commitment, directed radiation therapy may be considered.
By FIGO staging criteria, the patient described would be considered stage IV because of bony metastases. Unfortunately, this carries a grave prognosis. A pathologic study of the tumor may be helpful in directing adjuvant therapy. Endometrial stromal sarcomas rich in estrogen and progesterone receptors have shown objective response rates to hormonal treatments such as megestrol acetate, and, more recently, to the use of aromatase inhibitors. Because of the excellent side-effect profile of these agents, one may consider adjuvant treatment with either an aromatase inhibitor and/or progestin therapy in this setting.
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