Alveolar soft part sarcomas in young patients: The French national NETSARC+ network experience.

BACKGROUND AND AIMS: Alveolar soft part sarcoma (ASPS) is an ultra-rare chemo-resistant sarcoma in children, occurring preferentially in young adults. We aimed to describe and compare its clinical presentation and behaviour in children and young adults to determine whether the same therapeutic strategy should be addressed for both populations. METHODS: National retrospective multicentre study of children (0-18 years) vs. young adults (19-30 years) included in the "ConticaBase" sarcoma database, treated for ASPS between 2010 and 2019 with pathology reviewed via the NETSARC + network. RESULTS: Overall, 45 patients were identified, 19 children (42%) and 26 young adults (58%). All ASPS diagnoses were confirmed with TFE3 rearrangement by immunohistochemistry or FISH. All clinical characteristics were balanced between both populations with frequent metastases at diagnosis (8/19 vs. 10/26). The therapeutic strategy was based on surgery (17/19 vs. 21/26), radiotherapy (8/19 vs. 12/26) ± systemic treatment (8/19 vs. 9/26). In patients with initially localized disease, metastatic relapse occurred only in adults (8/16), whereas metastatic progression was present in both metastatic groups (5/8 vs. 8/10). After a median follow-up of 5.2 years (range, 0.2-12.2), 5-year EFS was 74% [95%CI, 56-96] vs. 47% [30-74] (p = 0.071) respectively, and 5-year OS was 95% [85-100] vs. 85% [70-100] (p = 0.84). For localized tumours, 5-year MFS was 100% [100-100] vs. 60% [39-91] (p = 0.005). The 5-year OS of all patients with metastasis at diagnosis was 80.2% (62.2%-100%). CONCLUSIONS: ASPS appears to have the overall same clinical characteristics, but a more aggressive behaviour in young adults than in children. However, despite frequent metastases at diagnosis, long-term survival is high in both groups. Overall, the same therapeutic strategies may be considered for both populations.

[Uterin sarcoma, high-grade stroma, indifferenciated, referential]. / Sarcomes utérins du stroma de haut grade et sarcomes indifférenciés ­ Référentiels de prise en charge du Groupe Sarcome Français et du Groupe des Tumeurs Rares Gynécologiques.

High-grade endometrial stromal sarcoma (HGESS) and uterine undifferentiated sarcoma (UUS) are rare uterine malignancies arising from mesenchymal endometrial cells. They are characterized by aggressive behavior and poor prognosis. Median age of diagnostic is 55years. The most common symptoms are vaginal bleeding, abdominal pain, and pelvic mass. Approximately 65 % are diagnosed witch advance disease stage III or IV according to the International Federation of Gynecology and Obstetrics classification. Median overall survival is around 20months. The management of the disease must be discussed in multidisciplinary staff meetings. The standard management of HGESS and UUS is total hysterectomy with bilateral oophorectomy. Systematic lymphadenectomy is not recommended. Adjuvant therapies, such as chemotherapy and radiotherapy must be discussed. In case of oligo-metastasic disease, surgery of the primary tumor and metastasis must be discussed and if not operable the standard management is doxorubine-based chemotherapy.

Evaluation of response after SBRT for liver tumors.

Stereotactic body radiotherapy (SBRT) has developed over the last few years for the treatment of primary and metastatic hepatic tumors. The tumoral and adjacent peritumoral modifications caused by this radiosurgery limit the evaluation of response by anatomic imaging and dimensional criteria alone, such as with RECIST. This suggests that it is of interest to also take into account the residual enhancement and hyper metabolism of these hepatic targets. We have reviewed the English language literature regarding the response of hepatic lesions treated by SBRT, and found that only seven articles were specifically concerned with this problem. The response of the hepatocellular carcinoma after SBRT has been studied specifically with multiphase enhanced CT-scan. Criteria set by the European Association of Study of the Liver better estimate response at each time point of follow up than RECIST does. Non-enhancement, reflecting tumor necrosis, is additionally an early indicator of response with extended response in time and a best non-enhancement percentage is observed at 12 months. The response after treatment by SBRT of cholangiocarcinoma has not yet generated a specific report. Use of RECIST criteria is also inadequate in the evaluation of response after SBRT for hepatic metastases. Response of liver metastases to SBRT is better assessed with a combination of size and enhancement pattern. The occurrence of a lobulated enhancement during follow up is efficient to predict local progression in a specific, reproducible, and sensitive way. Patients with FDG-avid hepatic metastases are also better evaluated with PET-CT and functional criteria than routine imaging and metric evaluation alone.

Radiation plus docetaxel and cisplatin in locally advanced pancreatic carcinoma: a non-comparative randomized phase II trial.

BACKGROUND: We performed a randomized, non-comparative phase II study evaluating docetaxel in combination with either daily continuous (protracted IV) 5-fluorouracil or cisplatin administered weekly, concurrent to radiotherapy in the treatment of locally advanced pancreatic carcinoma. Results of the docetaxel plus cisplatin regimen are reported. METHODS: Forty chemotherapy-naive patients with locally advanced pancreatic carcinoma were randomly assigned to receive 5-fluorouracil and docetaxel or docetaxel 20mg/m(2) and cisplatin 20mg/m(2)/week, plus concurrent radiotherapy for 6 weeks. The radiation dose to the primary tumour was 54Gy in 30 fractions. The trial's primary endpoint was the 6-month crude non-progression rate. RESULTS: 51 patients from 7 centres were included in the docetaxel-cisplatin treatment group. Six-month non-progression rate was 39% (95% confidence interval: 26-53). Median overall survival was 9.6 months (95% confidence interval: 2.4-60.7); 6 complete and 8 partial responses were obtained. Six patients survived more than 2 years after their inclusion in the trial. Grade ≥3 toxicity was reported in 63% of patients; no treatment-related death occurred. Severe toxicities were mainly anorexia (22%), vomiting (20%) and fatigue (24%). CONCLUSIONS: Despite inadequate efficacy according to the main end point, this regimen gave a satisfactory rate of objective response (27%) with tolerable toxicity.

Low-dose intraoperative brachytherapy in soft tissue sarcomas involving neurovascular structure.

BACKGROUND AND PURPOSE: To evaluate intraoperative brachytherapy in the management of soft tissue sarcomas involving neurovascular structures, its impact on local control and complications. PATIENTS AND METHODS: Between 01/1989 and 12/2002, 98 patients received an intraoperative implant in conjunction with conservative surgery. Brachytherapy was part of the initial treatment (79 cases) or performed in recurrent disease (19 cases). We studied primary sarcomas involving neurovascular structures treated with conservative surgery and intraoperative brachytherapy (n = 6) or intraoperative brachytherapy and external irradiation (n = 73). Conservative surgery was performed as first treatment (51 cases), after chemotherapy (21 cases) and after primary external radiation (seven cases). Brachytherapy was performed according to Paris system rules. Patients were loaded with Iridium 192 (64 cases) or connected to a Microselectron PDR (15 cases). Mean dose given by brachytherapy was 20 Gy. Mean dose given of external radiotherapy was 46 Gy. RESULTS: With a median follow-up of 58 months, 5-year actuarial survival was 69% and local free disease at 5 years was 90%. Acute side-effects occurred in 22/79 requiring surgical repair in 10 patients. Late side-effects occurred in 35/79. No patient required amputation for complications. Prognostic factors were studied for the occurrence of acute and late side-effects and local control. CONCLUSIONS: Intraoperative brachytherapy is efficient with excellent local control rates in soft tissue sarcomas presenting with neurovascular involvement and offers an acceptable conservative option.