Faculty Opinions recommendation of Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group.

Author(s):  
Piotr Czauderna
2016 ◽  
Vol 2 (3_suppl) ◽  
pp. 29s-29s
Author(s):  
Linda Z. Abramovitz ◽  
Rehana Punjwani ◽  
Julia Challinor

Abstract 44 Purpose: Globally, nurses caring for children and adolescents with cancer work in diverse practice settings. Nurses in low- and middle-income countries (LMIC) are challenged by the limited scope of their professional practice, resources and education. Opportunities for education, networking and professional growth inspire and empower nurses to improve the care of patients and families. Nurses are key members of the International Society of Paediatric Oncology (SIOP), which provides ongoing professional development. In 2010, the SIOP Pediatric Oncology in Developing Countries Committee's Nursing Working Group was formed to support and learn from nurses working in LMIC. Methods: Utilizing the Cure4Kids website from St Jude, the group holds monthly business and educational meetings and continues to network and collaborate on numerous projects. PODC Nursing Working Group recently published baseline standards for pediatric oncology nursing care in LMIC. A survey will now assess the implementation of baseline standards. LMIC nurses are being mentored to write abstracts and grants to increase global recognition of their work. In addition, an education repository of teaching materials is being established. Summary: The PODC Nursing Working Group and project groups are well established and achieving project deadlines. Educational programs including topics on abandonment, nursing in LMIC and abstracts have been well received by both nurses and physicians from LMIC and HIC. Challenges have included internet connectivity and scheduling meetings during daylight hours across the multiple time zones. Following the launch of monthly meetings in late 2015, our membership has increased by 20% from 61 to 76 members from 29 countries. Conclusions: The SIOP PODC Nursing Group has created a sustainable nursing community and continues to work collaboratively beyond the annual conference. The compassion and commitment to support patients and their families by providing the best care possible is the common thread for pediatric oncology nurses. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST: No COIs from the authors.


2003 ◽  
Vol 21 (5) ◽  
pp. 793-798 ◽  
Author(s):  
Richard J. Stewart ◽  
Hélène Martelli ◽  
Odile Oberlin ◽  
Annie Rey ◽  
Nathalie Bouvet ◽  
...  

Purpose: To report the results of the Malignant Mesenchymal Tumors studies (MMT 84 and 89) of the International Society of Pediatric Oncology (SIOP) in males with nonmetastatic paratesticular rhabdomyosarcoma. Patients and Methods: From 1984 to 1994, 96 males were treated in SIOP protocols. Radical inguinal orchidectomy was recommended, but initial retroperitoneal lymph node dissection was not performed. Disease was staged according to the SIOP tumor-node-metastasis staging system. Treatment was stratified by stage. In the MMT 89 study, males with completely resected tumors at diagnosis received less chemotherapy (vincristine and dactinomycin) than patients in the MMT 84 study (ifosfamide, vincristine, and dactinomycin). Results: Median age at diagnosis was 65 months. Thirty-one tumors were larger than 5 cm, and 13 males were older than 10 years with a tumor larger than 5 cm. At a median follow-up of 7 years, 87 patients were alive; 79 were in first complete remission and eight were in second complete remission. Relapse occurred in 16 patients (17%). At 5 years, the overall survival (OS) rate was 92%, with an event-free survival (EFS) rate of 82%. OS and EFS were significantly worse for males with tumors greater than 5 cm and for males older than 10 years at diagnosis. Conclusion: Males with paratesticular RMS have an excellent prognosis except for a selected group of patients older than 10 years or with tumor greater than 5 cm. Intensified chemotherapy incorporating alkylating agents for this subgroup may be preferred to the use of systematic lymphadenectomy to improve survival while minimizing the burden of therapy.


1994 ◽  
Vol 12 (3) ◽  
pp. 516-521 ◽  
Author(s):  
P Rousseau ◽  
F Flamant ◽  
E Quintana ◽  
P A Voute ◽  
J C Gentet

PURPOSE The MMT 84 multicentric prospective trial of the International Society of Pediatric Oncology (SIOP) was designed to (1) test the effectiveness of ifosfamide 3 g/m2 on days 1 and 2, vincristine 1.5 mg/m2 on days 1 and 14, and dactinomycin 0.9 mg/m2 on days 1 and 2 (IVA) repeated every 21 days; and (2) reduce late effects of treatment by reserving radiation therapy to the primary site for patients not achieving a complete response (CR) to primary chemotherapy. MATERIALS AND METHODS Between 1984 and 1989, the MMT 84 study registered 34 children with nonmetastatic rhabdomyosarcomas (RMSs) and other malignant mesenchymal tumors (MMTs) of the orbit in this trial. RESULTS The 4-year event-free survival rate is 62% +/- 9% (SD) and the 4-year survival rate 86% +/- 7% (SD). A total of 11 local recurrences occurred, 10 among 22 patients treated without initial radiation. Salvage of local failure was achieved in nine of 11 patients with the use of radiation and additional chemotherapy, but three later developed distant metastases and two have died. One isolated regional lymph node failure has occurred, while no patient relapsed with isolated distant metastases. Six of 12 patients who failed are alive with no evidence of disease from 16 to 50 months after relapse. The treatment was well tolerated in all patients, except for one with renal tubular acidosis and one who died of cardiotoxicity. Twelve patients remain in first remission without the use of radiation to the primary tumor from 27 to 84 months. CONCLUSION Despite a higher incidence of local recurrence when treated by primary chemotherapy, early survival rates were not compromised and a significant number of patients avoided the late effects of radiation. However, longer follow-up is required to assess the ultimate outcome of patients treated in this manner.


2007 ◽  
Vol 25 (10) ◽  
pp. 1176-1182 ◽  
Author(s):  
Marie-Pierre Castex ◽  
Hervé Rubie ◽  
Michael C.G. Stevens ◽  
Carlota Calvo Escribano ◽  
Jérôme Sales de Gauzy ◽  
...  

Purpose To evaluate the outcome of children with an extraosseous Ewing tumor (EOE) according to treatment. Patients and Methods Children with EOE were treated either with the strategy used for malignant mesenchymal tumors (MMTs) by the International Society of Pediatric Oncology (SIOP) or with the French Society of Pediatric Oncology (SFOP) regimen used for osseous Ewing tumors (OET). The MMT strategy included vincristine/actinomycin for small and resected tumors or ifosfamide/vincristine/actinomycin for unfavorable sites or unresectable tumors. Surgical excision was to be attempted after four courses, followed by local irradiation in case of residue. Osseous Ewing (OE) protocol included three courses of cyclophosphamide/doxorubicin followed either by two similar courses in case of good response or two courses of ifosfamide/etoposide in case of no response. After resection of the primary, treatment included conventional chemotherapy in case of good histologic response and high-dose chemotherapy and radiotherapy for poor response. All diagnosis specimens were reviewed by the panel. Results Between 1989 and 1999, 63 patients were registered. Characteristics of patients treated by both protocols were similar. Five-year overall survival (OS) and event-free survival (EFS) of those treated with the OE protocol are 83% and 75%, respectively, which is significantly better than the OS and EFS of those treated with the MMT strategy (59% and 44%, respectively; P = .04 and .008, respectively). The size of the primary and the type of protocol influenced patients’ EFS. In multivariate analysis, only the regimen had an impact on OS and EFS. Conclusion Our study shows that patients with EOE should be treated with OE regimens, probably because of the use of anthracyclines.


1999 ◽  
Vol 17 (7) ◽  
pp. 2117-2117 ◽  
Author(s):  
Hélène Martelli ◽  
Odile Oberlin ◽  
Annie Rey ◽  
Jan Godzinski ◽  
Richard D. Spicer ◽  
...  

PURPOSE: To report the results of a conservative multimodal approach in girls with nonmetastatic rhabdomyosarcoma (RMS) of the genital tract, treated in International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors 84 and 89 protocols. PATIENTS AND METHODS: From 1984 to 1994, 38 girls with RMS of the genital tract (vulva, vagina, uterus) were treated in SIOP protocols. With the exception of patients with rare small tumors, which were resected at the start of the studies, all patients received initial chemotherapy (CHT) (ifosfamide, vincristine, and actinomycin D). Local treatment including surgery, brachytherapy (BT), and external-beam radiotherapy (ERT) was given only to girls who did not achieve complete remission (CR) with CHT or who subsequently relapsed. RESULTS: The primary tumor originated in the vulva or vagina in 27 girls and in the uterus in 11. The overall survival rate (± SE) was 91% ± 6% at 5 years, and the event-free survival rate was 78% ± 7%. At a median follow-up of 5 years, 30 girls were alive and in first CR and five were alive and in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT. Thirteen patients were treated with CHT alone. In 17 patients, local treatment was necessary to achieve complete local control, for a residual mass after initial CHT (10 patients), for viable tumor on biopsy (three patients), or for local relapse (four patients). The local treatment used was radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radical surgery (three patients), and conservative surgery with RT (one patient). CONCLUSION: Girls with nonmetastatic RMS of the genital tract have an excellent prognosis. We found no difference in outcome between uterine and vulvovaginal RMS. Local treatment does not seem necessary in patients who have a complete response to CHT. When a local treatment is needed, BT may be an alternative to radical surgery or ERT.


1991 ◽  
Vol 9 (12) ◽  
pp. 2177-2182 ◽  
Author(s):  
A Suarez ◽  
H McDowell ◽  
P Niaudet ◽  
E Comoy ◽  
F Flamant

The renal function of 74 children with malignant mesenchymal tumors in complete remission and who have received the same ifosfamide chemotherapy protocol (International Society of Pediatric Oncology Malignant Mesenchymal Tumor Study 84 [SIOP MMT 84]) were studied 1 year after the completion of treatment. Total cumulative doses were 36 or 60 g/m2 of ifosfamide (six or 10 cycles of ifosfamide, vincristine, and dactinomycin [IVA]). None of them had received cisplatin chemotherapy. Ages ranged from 4 months to 17 years; 58 patients were males and 42 females. The most common primary tumor site was the head and neck. Renal function was investigated by measuring plasma and urinary electrolytes, glucosuria, proteinuria, aminoaciduria, urinary pH, osmolarity, creatinine clearance, phosphate tubular reabsorption, beta 2 microglobulinuria, and lysozymuria. Fifty-eight patients (78%) had normal renal tests, whereas 16 patients (22%) had renal abnormalities. Two subsets of patients were identified from this latter group: the first included four patients (5% of the total population) who developed major toxicity resulting in Fanconi's syndrome (TDFS); and the second group included five patients with elevated beta 2 microglobulinuria and low phosphate reabsorption. The remaining seven patients had isolated beta 2 microglobulinuria. Severe toxicity was correlated with the higher cumulative dose of 60 g/m2 of ifosfamide, a younger age (less than 2 1/2 years old), and a predominance of vesicoprostatic tumor involvement. This low percentage (5%) of TDFS must be evaluated with respect to the efficacy of ifosfamide in the treatment of mesenchymal tumors in children.


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