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Journal of Clinical Oncology, Vol 18, Issue 11 (June), 2000: 2343-2344
© 2000 American Society for Clinical Oncology


DIAGNOSIS IN ONCOLOGY

Two Patients With Sarcoma

Arthur Skarin, MDConsultant Editor

Gerard C. Blobe, Michelle Mantel, Milos Janicek, George D. Demetri

Dana-Farber Cancer Institute, Brigham and Women’s Hospital, and Harvard Medical School, Boston, MA

CASE 2. UTERINE SARCOMA

A 54-year old woman presented with a 6-month prodrome of fatigue, sweats, and irregular menstrual bleeding as well as intermittent fevers to 104°F. After developing acute left lower quadrant pain, she underwent emergent exploratory laparotomy, which revealed diverticulosis with multiple mural abscesses, a tubo-ovarian abscess, and a malignancy of the uterus. A total abdominal hysterectomy and bilateral salpingo-oophorectomy were carried out along with resection of rectosigmoid colon. Pathology showed a high-grade stromal sarcoma of the uterus with full-thickness penetration of the myometrium and into, but not through, the serosa. Lymphovascular invasion was present (Fig 1, arrow). Under high-power view, the large spindle and polygonal neoplastic cells showed a high nuclear-cytoplasmic ratio with numerous mitoses, up to 30 per 10 high power fields (Fig 2). The patient had an uneventful postoperative course until 2 weeks after the operation, when she was involved in a motor vehicle accident resulting in progressively worsening lower back pain. Physical therapy and nonsteroidal anti-inflammatory drugs were tried without relief. Magnetic resonance imaging was performed, which revealed multiple lytic lesions in the thoracic and lumbar spine (Fig 3, arrow). Further staging revealed widespread metastases in the lung, liver, and pelvis (Fig 4, arrows). The patient was referred to our institution and was subsequently treated with ifosfamide, carboplatin, and etoposide. Unfortunately, she did not respond to treatment and died 3 months after presentation.



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Uterine sarcomas make up 10% of all uterine cancers and include leiomyosarcomas, endometrial stromal sarcomas (ESS), and mixed mullerian tumors that contain both carcinoma and sarcoma. ESS make up 5.7% of all uterine sarcomas and usually result in postmenopausal or abnormal uterine bleeding (92%).1 ESS tend to have a more favorable prognosis than other uterine sarcomas, with an overall median survival of 3 years. Extent of disease, size of primary tumor, and grade are significant prognostic features.1,2 Uterine sarcomas have a propensity for extensive local growth resulting in pelvic fullness, pressure, pain, or bowel obstruction. Hematogenous spread is also common, with pulmonary metastasis being most frequent, followed by liver, bone, and brain. Treatment involves surgical resection when possible. Postoperative radiation therapy may decrease local recurrence but does not change overall survival. Although there is no proven role for adjuvant chemotherapy, anthracyclines and ifosfamide are effective for advanced leiomyosarcomas, ifosfamide and cisplatin have been effective for mixed mullerian tumors, and recent evidence supports the use of ifosfamide, cisplatin, doxorubicin, and etoposide for ESS.3-5

REFERENCES

1. DeFusco PA, Gaffey TA, Malkasian GD Jr, et al: Endometrial stromal sarcoma: Review of Mayo Clinic experience, 1945-1980. Gynecol Oncol 35:8-14, 1989[Medline]

2. Nordal RR, Thoresen SO: Uterine sarcomas in Norway 1956-1992: Incidence, survival and mortality. Eur J Cancer 33:907-911, 1997

3. Lin YC, Kudelka AP, Tresukosol D, et al: Prolonged stabilization of progressive endometrial stromal sarcoma with prolonged oral etoposide therapy. Gynecol Oncol 58:262-265, 1995 [Medline]

4. Sutton G, Blessing JA, Park R, et al: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: A study of the Gynecologic Oncology Group. Obstet Gynecol 87:747-750, 1996[Abstract]

5. Yamawaki T, Shimizu Y, Hasumi K: Treatment of stage IV "high-grade" endometrial stromal sarcoma with ifosfamide, adriamycin, and cisplatin. Gynecol Oncol 64:265-269, 1997[Medline]





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