Synovial Sarcoma of Childhood and Adolescence: A Multicenter, Multivariate Analysis of Outcome

Okcu, M. Fatih; Munsell, Mark F.; Treuner, J.; Mattke, Adrian; Pappo, Alberto S.; Cain, Alvida M.; Ferrari, Andrea; Casanova, Michela; Özkan, Alp; Raney, Beverly

doi:10.1200/jco.2003.07.008

Cited by 212 publications

(199 citation statements)

References 24 publications

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“…Radiotherapy is of most benefit with larger tumors and in the case of inadequate margins. This is consistent with other studies, 22 and in accordance with recent STS recommendations. 19,28 Original Article 2996…”

Section: Discussionsupporting

confidence: 94%

“…A significantly better survival was previously demonstrated for patients aged <40 years, 10 17 years, 11 13 years, 22 and 35 years 23 ; the latter study included only patients with metastatic disease. Two other studies found age to be a continuous variable associated with survival.…”

Section: Discussionmentioning

confidence: 80%

“…4,[6][7][8][9][10][11]15,22 Only in the study by Ladanyi et al was size not found to influence OS in the subgroup of localized SS patients, but it did when metastatic patients were included. 12 The finding in the current series that larger tumors were more frequently reported in patients with metastatic disease compared with those with localized disease reinforces the significance of tumor size in SS.…”

Section: Discussionmentioning

confidence: 96%

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Synovial sarcoma

Palmerini

Staals

Alberghini

et al. 2009

Cancer

154

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BACKGROUND: The optimal treatment for synovial sarcoma remains controversial. Treatment, outcome, and prognostic factors in patients treated in a single institution were examined. METHODS: Synovial sarcoma patients who underwent surgery at the Rizzoli Institute between 1976 and 2006 were identified and analyzed. RESULTS: Characteristics of the 250 patients (128 female; 122 male) included: median age, 37 years (range, 7‐83 years); 177 (71%) with tumors in the lower extremity, 40 (16%) with tumors in the upper extremity, and 33 with tumors in the trunk (13%); primary lesion size >5 cm in 121 patients (55%); and 204 (82%) patients with localized disease and 46 (18%) with metastatic disease at the time of presentation. All patients with localized disease underwent surgery. Twenty‐four percent of patients underwent amputation. Adequate surgical margins were achieved in 88% patients. In patients with localized disease, radiotherapy was administered to 103 (50%) patients, and chemotherapy to 98 (48%). With a median follow‐up of 5.5 years (range, 1‐30 years), the 5‐year overall survival rate was 10% for patients with metastatic disease and 76% for patients with localized disease (P = .0001). The 5‐year event‐free survival was 58% in patients with localized disease. Multivariate analysis indicated that size, age, histologic subtype, and the use of radiotherapy were independent factors for event‐free survival. CONCLUSIONS: In those patients with localized disease, a good rate of cure can be achieved. Age, size, histology, and use of radiotherapy influence prognosis, whereas to the authors' knowledge, the role of adjuvant chemotherapy remains unproven. Cancer 2009. © 2009 American Cancer Society.

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Section: Discussionsupporting

confidence: 94%

Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 96%

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Synovial sarcoma

Palmerini

Staals

Alberghini

et al. 2009

Cancer

154

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“…Although the incidence rate of SS peaks in the third decade of life [1], 30% of SS cases occur in patients younger than 20 years of age [2].…”

Section: Introductionmentioning

confidence: 99%

“…Most present with a painful lump which gradually grows over time. In pediatric cohorts, survival rates of up to 75-84% [2,[4][5][6][7][8][9] have been reported. These are superior to those reported in adults (survival rates ranging from 25-71%) [4,[10][11][12][13][14].…”

Section: Introductionmentioning

confidence: 99%

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Speth

Krieg

Kaelin

et al. 2011

Journal of Children's Orthopaedics

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Purpose Synovial sarcoma (SS) is an aggressive softtissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. Methods We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients (n = 8) was 20.1 years (12.1-27.6) and for nonsurvivors (n = 5) 4.9 years (range: 2.6-9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. Results Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5-and 10-year survival rates were 77 and 61%, respectively. The mean time until a local recurrence (n = 5) was 3.2 years (range: 0.7-10.2), while there was a mean time of 2.1 years until the occurrence of late metastases (n = 5; range: 0.8-4.8). A high tumor grade and having a tumor in the trunk were adverse factors in terms of overall, local recurrence-free, and metastasis-free survival. Patients with wide resections or amputations had fewer local recurrences than patients with marginal or intralesional resections. Conclusion Inadequate primary excision of SS results in incomplete excision in the majority of cases. The tumor site, size, and histological grade should be considered when determining a risk-adapted treatment for SS, and wide surgical excision is the surgical intervention of choice. While local recurrence and late metastases appear to occur after a shorter time period in pediatric patients than in adults, in view of the tendency for late recurrence and metastasis with SS, follow-up should be at least 10 years.

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