Diagnostic criteria for uterine smooth muscle tumors: leiomyoma variants associated with malignant behavior.

OBJECTIVE: To determine the prognostic accuracy of current diagnostic criteria for uterine smooth muscle tumors. STUDY DESIGN: Cases of uterine leiomyosarcoma (LMS) treated from 1976 to 1999 were analyzed retrospectively. Uterine LMS specimens were reevaluated using current criteria by a pathologist specializing in gynecologic diseases. Kaplan-Meier survival curves were evaluated. RESULTS: Specimens were available from 67 patients diagnosed with uterine LMS. On rereview, only 47 specimens were thought to represent uterine LMS. The 20 other patients were deemed to have leiomyomas or leiomyoma variants, including 13 cellular leiomyomas, 5 atypical leiomyomas and 2 leiomyomas. Median survival for patients with uterine LMS was 2.1 years. (Ninety-seven percent of disease-specific deaths occurred within 6 years after the diagnosis.) With leiomyoma variants, median survival was > 25 years. Among these 18 women were 3 disease-specific deaths (all > 6 years after diagnosis). CONCLUSION: Diagnostic criteria for uterine smooth muscle tumors require continued refinement. A small but significant number of patients diagnosed with leiomyoma variants will die of the disease. In contrast to the aggressive behavior of uterine LMS, disease-specific deaths attributed to leiomyoma variants occurred later. With this potential for delayed recurrence, these patients warrant close clinical surveillance.

Surgical treatment for local control of female urethral carcinoma.

We reviewed 53 patients (mean age 63 years) who underwent partial urethrectomy (n = 26) or radical extirpation (n = 27) for primary female urethral cancer from 1948 through 1999. Clinical stage, histology, high pathologic stage (3 or 4) and grade, tumor location, nodal status, surgery type, adjuvant therapy, and treatment decade were candidate outcome predictors. The predominant carcinomas were squamous cell (n = 21), transitional cell (TCC) (n = 15), and adenocarcinoma (n = 14). For adjuvant therapy, 20 patients had radiation (8 preoperatively), 2 had radiation + chemotherapy, and 1 had chemotherapy alone. During mean follow-up of 12.8 years, 27 patients had recurrence; 15 local only, 2 distant only and 10 local + distant. Of patients undergoing partial urethrectomy for pT1-3 tumors, 6/27 (22%) had urethral recurrence. Overall, there were no bladder recurrences. Recurrence-free survival +/- standard error (SE) at 10 years was 45 + 8%. Those who recurred had a cancer mortality rate of 71% at 5 years postrecurrence. The estimated 10-year cancer-specific survival (CSS) and crude survival (CS) rates were 60 +/- 8% and 42 +/- 7%, respectively. Pathologic stage was predictive for local recurrence (P = 0.02) and CSS (P = 0.01). Positive nodes on pathology were related to local and distant recurrence and CSS (P = 0.01). Upon review, partial urethrectomy resulted in a high urethral recurrence rate (22%) with no bladder recurrences. These patients may be better served with radical urethrectomy and creation of continent catheterizable stoma.

Outcome of surgical treatment for primary malignant melanoma of the female urethra.

PURPOSE: We evaluate tumor characteristics, recurrence and survival following surgical treatment for female urethral melanoma. MATERIALS AND METHODS: A review of the records of all female patients with primary localized urethral melanoma (11, mean age 68 years) who underwent partial urethrectomy or radical extirpation from 1950 to 1999 was performed to determine disease specific survival and/or tumor characteristics correlating with survival. Clinical and pathological stage, tumor location, nodal status, adjuvant therapy and tumor pathological components including depth, width, necrosis and vascular/lymphatic invasion, were evaluated. Overall disease recurrence, crude and disease specific survival rates were calculated using the Kaplan-Meier method. RESULTS: Malignant melanoma occurred in the distal urethra in all 11 cases with local extension into the vagina (T3) in 7. Mean depth of invasion was 6.1 mm and mean tumor width was 2.0 cm. No vascular/lymphatic invasion or tumor necrosis was seen pathologically. No patient had received adjuvant therapy at the time of initial surgery. There were 7 recurrences (6 of 7 within 1 year postoperatively). Of the 7 cases of partial urethrectomy, urethral recurrence (1 with concurrent lung metastasis) developed in 5 and none had bladder recurrence. Those who underwent radical surgery had recurrence in the pelvis and lungs and inguinal lymph nodes. Crude and disease specific survival +/- standard error at 3 years was 27 +/- 15% and 38 +/- 19%, respectively. CONCLUSIONS: Primary female urethral melanoma is associated with a rapid and high local recurrence rate (60% at 1 year). Overall and cancer specific survival at 3 years is 27% and 38%, respectively. Local failure may in part be due to inadequate resection.

Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature.

BACKGROUND: Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas. Involvement of the reproductive tract is unusual. CASE: A 35-year-old woman had a pelvic mass and omental cake. Frozen-section examination at laparotomy revealed small round cell tumor confirmed as pPNET. Chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide with mesna yielded complete response. The patient's mother died of a similar tumor at age 52 years, and the patient's husband had adult Ewing sarcoma, constituting an unusual cluster of related tumors. CONCLUSIONS: Genetic recombination resulting in a chimeric transcript of the Ewing sarcoma gene and the Friend leukemia virus integration site is characteristic of these tumors. Surgical resection and multiagent chemotherapy may enhance survival.

Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy.

OBJECTIVE: We evaluated the predictive value of several proposed prognostic indicators and the effect of surgical management and adjuvant therapy on clinical outcome associated with leiomyosarcoma (LMS) of the uterus. METHODS: A medical record search of patients treated at Mayo Clinic from 1976 through 1999 was performed using the International Classification of Diseases, Ninth Revision codes for LMS and malignant neoplasm of the uterus. Study inclusion criteria included confirmation of the diagnosis of LMS of the uterus by a pathologist at our institution. Survival curves were generated using the Kaplan-Meier method. Multivariate analysis was performed using the Cox proportional hazards model. A case-control investigation was also performed. RESULTS: A total of 208 patients met study requirements. The median follow-up for survivors was 7.7 years. Multivariate analysis showed that high grade, advanced stage, and oophorectomy were associated with significantly worse disease-specific survival. Case-control investigations suggested that ovarian preservation does not adversely affect survival and that adjuvant pelvic radiation therapy does not significantly improve survival. An LMS risk-assessment index that was generated is highly predictive of survival. CONCLUSION: Tumor grade and stage (using modified criteria for endometrial cancer) appear to be valid prognostic indicators for LMS of the uterus. Ovarian preservation may be considered in premenopausal patients with early-stage leiomyosarcoma of the uterus. Additionally, adjuvant therapy does not appear to significantly affect survival. Finally, our highly predictive LMS risk-assessment index may be useful for counseling patients.

McIndoe procedure for vaginal agenesis: long-term outcome and effect on quality of life.

OBJECTIVE: The purpose of this study was to evaluate quality of life, sexual function, and long-term outcome in women after undergoing the McIndoe procedure for vaginal agenesis. STUDY DESIGN: This was a retrospective descriptive study of patients who were treated with the McIndoe procedure for vaginal agenesis. Participants answered a structured questionnaire to describe self-reported outcomes in quality of life, sexual function and satisfaction, and body image after the McIndoe procedure. Patient characteristics along with short- and long-term findings were abstracted from the medical record. RESULTS: Eighty-six patients responded to the questionnaire. Average age (+/-SD) at surgery was 21+/-6 years (range, 12-49 years). The mean number of years (+/-SD) since surgery was 23+/-12 (range, 2-50 years). Seventy-nine percent of the respondents stated that the McIndoe procedure improved their quality of life. Ninety-one percent of the respondents were sexually active, with 75% able to achieve orgasm. Reported self-image was improved in 55% of the women. CONCLUSION: The McIndoe procedure improves quality of life and sexual satisfaction and provides a functional vagina with minimal complications.