Italian consensus conference on management of uterine sarcomas on behalf of S.I.G.O. (Societa' italiana di Ginecologia E Ostetricia).

BACKGROUND: Uterine sarcomas are very rare tumours with different histotypes, molecular features and clinical outcomes; therefore, it is difficult to carry out prospective clinical trials, and this often results in heterogeneous management of patients in the clinical practice. AIM: We planned to set up an Italian consensus conference on these diseases in order to provide recommendations on treatments and quality of care in our country. RESULTS: Early-stage uterine sarcomas are managed by hysterectomy + bilateral salpingo-oophorectomy according to menopausal status and histology; lymphadenectomy is not indicated in patients without bulky nodes, and morcellation must be avoided. The postoperative management is represented by observation, even though chemotherapy can be considered in some high-risk patients. In early-stage low-grade endometrial stromal sarcoma and adenosarcomas without sarcomatous overgrowth, hormonal adjuvant treatment can be offered based on hormone receptor expression. In selected cases, external beam radiotherapy ± brachytherapy can be considered to increase local control only. Patients with advanced disease involving the abdomen can be offered primary chemotherapy (or hormonal therapy in the case of low-grade endometrial stromal sarcoma and adenosarcoma without sarcomatous overgrowth), even if potentially resectable in the absence of residual disease in order to test the chemosensitivity (or hormonosensitivity); debulking surgery can be considered in patients with clinical and radiological response. Chemotherapy is based on anthracyclines ± ifosfamide or dacarbazine. Palliative radiotherapy can be offered for symptom control, and stereotactic radiotherapy can be used for up to five isolated metastatic lesions. CONCLUSIONS: Treatment of uterine sarcoma should be centralised at referral centres and managed in a multidisciplinary setting.

Cervical Mullerian adenosarcoma with heterologous sarcomatous overgrowth: a fourth case and review of literature.

BACKGROUND: Uterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers. Less than 8% of all cases are Mullerian adenosarcoma, a distinctive uterine neoplasm characterized by a benign, but occasionally atypical, epithelial and a malignant, usually low-grade, stromal component, both of which should be integral and neoplastic constituents of the tumor. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very aggressive variant, associated with post-operative recurrence, metastases, even when diagnosed in early stage. CASE PRESENTATION: We present a fourth MASO case derived from uterine cervix in a 72-year-old woman with metrorrhagia and a polypoid mass protruding through the cervical ostium. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, systematic pelvic lymph node dissection, omental biopsy and appendectomy were performed. Surgery treatment was associated with adjuvant whole-pelvis radiation (45 Gy) and adjuvant chemotherapy (cisplatin/ifosfamide). After nine months of follow up, the patient was free of tumor. CONCLUSIONS: The rarity of MASO of the cervix involves a management difficult. Most authors recommend total abdominal hysterectomy, usually accompanied by bilateral salpingo-oophorectomy. There is no common agreement on staging by lymphadenectomy during primary surgery and adjuvant chemo-radio therapy.

Extragenital Müllerian adenosarcoma with pouch of Douglas location.

BACKGROUND: Of all female genital tract tumors, 1-3% are stromal malignancies. In 8-10% of cases, these are represented by Müllerian adenosarcoma an extremely rare tumor characterized by a stromal component of usually low-grade malignancy and by a benign glandular epithelial component. Variant that arises in the pouch of Douglas is scarcely mentioned in the medical literature. CASE PRESENTATION: A 49-year-old para-0 woman, was seen at our OB/GYN-UNIT because she complained vaguely of pelvic pain. She had a mass of undefined nature in the pouch of Douglas. A simple excision of the mass showed low-grade Müllerian adenosarcoma with areas of stromal overgrowth. One and a half year after surgery, at another hospital, a mass was detected in the patient's posterior vaginal fornix and removed surgically. Six months later she came back to our observation with vaginal bleeding and mass in the vaginal fornix. We performed radical surgery. The pathological examination showed recurrent adenosarcoma. Surgical treatment was supplemented by radiation therapy. CONCLUSIONS: The case of Müllerian adenosarcoma reported here is the third known so far in the literature that was located in the pouch of Douglas. To date, only two other such cases have been reported, including one resulting from neoplastic degeneration of an endometriotic cyst.

Recurrent mullerian adenosarcoma like tumor of seminal vesicle.

Adenosarcoma like tumor of the seminal vesicle is reported herein. A 35-year-old male presented with mass in the pelvis between bladder and rectum, involving the seminal vesicle and prostate. Mass recurred after enucleation in four years. Histologically, the tumor was multicystic with bland ciliated lining epithelium and sarcomatous stroma. A wide excision was performed followed with chemotherapy and radiotherapy. Adenosarcomas have a low grade recurrent malignant potential and should be recognized.

Effective bladder sparing therapy with intra-arterial cisplatin and radiotherapy for localized bladder cancer.

PURPOSE: We evaluated the feasibility and efficacy of the organ preserving strategy of intra-arterial cisplatin and concurrent radiotherapy for localized bladder cancer. MATERIALS AND METHODS: Bladder preservation has been pursued over the decades in treatment regimens featuring radiotherapy alone or in conjunction with single or multiagent chemotherapy. The chemotherapy has consisted almost exclusively of intravenously administered drugs. There are theoretical and clinical data demonstrating a higher concentration of cisplatin within tumors following intra-arterial as opposed to intravenous delivery. This study was performed to evaluate whether this increased concentration would enhance radiosensitization and thereby increase the success of bladder preservation. RESULTS: We report on our prospectively collected experience during 15 years of treating 200 patients with localized bladder cancer using this regimen of 3 courses of intra-arterial cisplatin integrated with pelvic radiotherapy and reserving cystectomy for salvage as required. We report on the efficacy in terms of complete response rate, ultimate tumor-free bladder preservation, overall survival and patterns of failure. We detail the acute and chronic toxicity observed to date. CONCLUSIONS: This strategy has resulted in a durable high complete response rate and overall tumor-free bladder preservation rate of 75% while maintaining a survival comparable to that achieved with cystectomy. These results corroborate the hypothesis that intra-arterial administration of cisplatin enhances radiosensitization during pelvic radiotherapy.

Using intraoperative radiation therapy--a case study.

The introduction of a mobile linear accelerator in the OR has made intraoperative radiation therapy (IORT) more plausible. An IORT treatment can deliver a single high dose of radiation to a tumor or tumor bed after surgical resection or surgical exposure of high risk areas. This article details a case study in which IORT was used on a patient with sigmoid carcinoma and the procedure outcomes.

Toward a dynamic real-time intraoperative permanent prostate brachytherapy methodology.

PURPOSE: To evaluate dosimetry and source location relative to CT-based dosimetry when performing real-time dynamic permanent prostate brachytherapy (PPB) with inverse treatment planning. METHODS AND MATERIALS: A treatment algorithm for dynamic PPB was developed using inverse treatment planning. The technique utilizes real-time transrectal ultrasound prostate imaging connected to the treatment planning software. The implementation of the plan with the Mick interstitial gun is monitored with up-to-date dosimetry assessments based on the registration of each seed when placed. Real-time dose assessment is monitored and adjustments can be made during the case, if necessary. A final OR dosimetric (OR-D) assessment based on the registered seed locations is performed. Postoperative CT scans obtained at 3 weeks are used for traditional dosimetry analysis (CT-D). A matrix algorithm was developed to match the seed locations from the ultrasound registration to that of the CT-scan parameters. RESULTS: Twenty-six consecutive patients with clinically localized prostate cancer underwent PPB using the algorithm designed for dynamic real-time planning. The OR-D identified a mean D90 of 109% (range 100-118%) whereas the mean CT-D D90 at 3 weeks was 105% (range 89-122%) (p=0.894). Analysis of the OR-D V100 and V150 relative to the 3-week CT-dose V100 and V150 were also insignificant (p=0.112 and 0.167, respectively). Assessment of seed locations relative to the intraoperative ultrasound and postimplant CT identified a mean root-mean-square error of 4.6 mm (0-21 mm). The mean error for the x, y, and z coordinates were 2.01 mm, 2.24 mm, and 2.85 mm, respectively. CONCLUSIONS: This study reports the preliminary results of a new treatment algorithm for PPB that incorporates intraoperative inverse planning with dynamic dosimetry assessment during the case. Correlation was seen between the completed intraoperative, postimplant plan and the CT based plan at 3 weeks. Seed to seed deviations between the OR-D matched well with the CT-D. Additional study is necessary to assess whether this approach can assist in improving implant dosimetry and whether it appropriately documents the OR-dose without the need for postimplant dosimetry.

Angiosarcoma of the breast following breast preservation therapy and local radiation therapy for breast cancer.

This is a case of angiosarcoma following breast preservation therapy and local radiation therapy over a 3-year period.

Mullerian adenosarcoma of the uterus: case report and review of literature.

Mullerian adenosarcoma--a variant of mullerian mixed mesodermal tumor of the uterus--is typically composed of benign but sometimes mildly atypical glandular epithelial elements admixed with malignant sarcomatous stroma. This rare tumor, which accounts for only about 8% of all uterine sarcomas, usually originates in the endometrium and grows as a polypoid mass within the endometrial cavity. The most prevailing presenting symptom is abnormal vaginal bleeding and the most common finding is a polypoid mass protruding through a dilated cervical canal. The case of a woman, who at age 62 presented with symptoms and signs of acute pelvic inflammatory disease and on vaginal examination an infected mullerian adenosarcoma protruding through a dilated cervical canal was discovered, is reported. Treatment consisted of extensive antibiotic treatment and surgery comprised of total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by postoperative adjuvant pelvic radiotherapy. One year later, the patient is alive with no evidence of disease.