PubMed · 11094321
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V K Sondak. 2000. Difficult situations in sarcoma. Issue 2.. https://doi.org/10.1016/s0960-7404(00)00033-5
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BACKGROUND: In the current study, the authors aim to evaluate clinical features and treatment results observed in patients from the German and Italian studies who had nonmetastatic abdominal rhabdomyosarcomas (RMS). METHODS: One hundred sixty-one patients were observed; 78 registered in the German studies between October 1980 and August 1995, and 83 registered in the Italian studies between April 1975 and December 1995. The age range of the patients was 0-18 years (median, 4 yrs). The distribution of tumor sites was as follows: 32 intraperitoneal, 42 retroperitoneal, 75 pelvic, and 12 not otherwise specified (NOS). Most patients had a large and invasive primary mass (26 T1b, 114 T2b). The breakdown in histology was as follows: 116 embryonal, 34 alveolar, and 11 other (leiomyomatous, pleomorphic, and NOS); all cases were staged according to the Intergroup Rhabdomyosarcoma Studies (IRS) system. Nine Group I patients were treated after surgery with chemotherapy (CT) (radiotherapy [RT] was delivered to treat alveolar RMS in the 1991 German and 1988 Italian studies); 19 Group II patients received CT + RT (40-44 Gy); 133 Group III patients underwent neoadjuvant CT +/- surgery and/or RT (54 Gy) + CT. Different CT regimens (based primarily on the administration of vincristine, dactinomycin, doxorubicin, and cyclophosphamide or ifosfamide) were adopted. RT was not recommended for patients age < 3 years. RESULTS: The 10-year overall survival (OS) and progression-free survival (PFS) were 47.2% and 43.9%, respectively. The OS was related significantly to the following variables: histology (alveolar, 29.4% vs. nonalveolar, 52.1% [P = 0.0156]), tumor size (> 5 cm, 42.1% vs. < 5 cm, 81% [P = 0.005]), age (< 10 yrs, 51.4% vs. >or= 10 yrs, 27.8% [P = 0.02]), complete surgery at diagnosis or after CT (+/-RT) (70.4% vs. 34.4% without it [P = 0.0015]). Most patients who achieved the delayed local control had responded well to neoadjuvant CT. CONCLUSIONS: Tumor size, histology, age, and initial or delayed achievement of local control were important prognostic factors. Most relapsed patients had unfavorable outcomes.
PURPOSE: To evaluate the outcome and patterns of failure in women with uterine clear-cell carcinoma and discuss implications for adjuvant radiation therapy (RT). METHODS: Between 1980 and 2000, 686 endometrial carcinoma patients underwent primary surgery at our institution. Thirty-eight women (5.5%) had clear-cell tumors (18 clear-cell only, 8 clear-cell + adenocarcinoma, and 12 clear-cell + other unfavorable histologies [10 papillary serous, 1 uterine sarcoma, 1 both]). All underwent surgery and assessment of peritoneal cytology. None received preoperative RT. Pelvic and para-aortic node samplings were performed in 26 and 17 patients, respectively. FIGO stages were as follows: 3 IA, 4 IB, 5 IC, 4 IIA, 6 IIB, 8 IIIA, 2 IIIB, 3 IIIC, and 6 IV. Adjuvant therapies included the following: 5 none, 22 RT (13 pelvic RT, 2 vaginal brachytherapy, 7 both), 11 chemotherapy (8 alone, 3 after pelvic RT), and 3 hormones. No patient received whole-abdominal RT or para-aortic RT. Median follow-up was 36.5 months. RESULTS: The 5-year actuarial disease-free survival of the entire group was 38.5%. No correlation was seen between relapse and stage, myometrial invasion, cytology, cervical extension, or involvement of extrauterine sites. Patients with clear +/- adenocarcinoma histology had a similar 5-year disease-free survival (38.8% vs. 38.7%, p = 0.95) compared with those with clear-cell + other unfavorable histologies. Sixteen patients relapsed (42%). Eight failed in the pelvis (5 vagina, 3 lateral pelvis). There were no pelvic failures in the group of 22 patients who received adjuvant RT, whereas in the group of 16 women who did not, 8 (50%) relapsed in the pelvis (p < 0.0001). Corresponding pelvic failure rates in the Stage IA-IIB patients with and without RT were 0/16 (0%) and 5/6 (83%) (p < 0.0001). Six patients (16%) failed in the para-aortic nodes and 2 (5%) in the abdomen. Only 1 (2%) patient developed an isolated abdominal failure (This patient had a mixed clear-cell/papillary serous tumor). Of the 26 women with clear-cell +/- adenocarcinoma histology, only 1 (3.8%) relapsed in the abdomen. Nine patients (24%) relapsed in distant sites, primarily the lungs and bone. CONCLUSION: Clear-cell carcinoma comprises a small percentage of endometrial cancers, frequently presents as a mixed histology, and has a poor overall outcome. Unlike papillary serous tumors, clear-cell carcinoma does not seem to have a high propensity for abdominal failure. Our results thus do not support the routine use of whole-abdominal RT in these patients. Future protocols should focus instead on combinations of locoregional RT and chemotherapy to reduce the risk of local and systemic recurrence.
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