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Desmoid fibromatosis in pediatric patients: management based on a retrospective analysis of 59 patients and a review of the literature.

Oudot C, Orbach D, Minard-Colin V, Michon J, Mary P, Glorion C, Helfre S, Habrand JL, Oberlin O - Sarcoma (2012)

Bottom Line: Conclusions.When surgery is required, surgical margins must be negative.Low-dose chemotherapy can be proposed as adjuvant therapy.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Oncology Department, Hôpital de la mère et de l'enfant, 8 rue Dominique Larrey, 87042 Limoges, France.

ABSTRACT
Background. Only limited data are available concerning desmoid tumor in children. Methods. Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed. Results. Median age was 6 years (range, 0-15). Tumors mainly involved the limbs (42%). Five cases occurred in a context of genetic disorder. Surgery was first-line treatment in 80% of cases. Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases. Various adjuvant therapies were used. Overall response to all systemic therapies was 33%. Thirty-eight patients developed one or more recurrences or progressions. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS. Conclusions. When surgery is required, surgical margins must be negative. Low-dose chemotherapy can be proposed as adjuvant therapy. Prospective trials must be developed to evaluate long-term response and side effects.

No MeSH data available.


Related in: MedlinePlus

Progression-free survival of the 59 patients according to age (±10 years).
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Related In: Results  -  Collection


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fig4: Progression-free survival of the 59 patients according to age (±10 years).

Mentions: In univariate analysis, younger age and head and neck sites were favorable prognostic factors for PFS. The 10-year PFS rate was 46% for children younger than 10 versus 6% for older children (P = 0.03—Figure 4). The 10-year PFS for patients with a head and neck (H/N) primary DT was better than for those with a limb or trunk DT (69% versus 14% versus 25%, resp., P = 0.01—Figure 5). Gender had no impact on PFS. The status of surgical margins did not appear to be a prognostic factor, as IRS staging was not found to be a significant prognostic factor for PFS (5-year PFS: 33% versus 16% versus 48% for IRSI versus IRSII versus IRSIII groups, respectively, P = 0.45). Ten-year PFS rates according to age (±10 year) and primary site (head and neck versus other primary) were statistically different: 62% for patients with H/N DT under the age of 10 versus 32% for patients with no H/N DT and under the age of 10 versus 5% for patients with no H/N DT and over the age of 10 (P = 0.01).


Desmoid fibromatosis in pediatric patients: management based on a retrospective analysis of 59 patients and a review of the literature.

Oudot C, Orbach D, Minard-Colin V, Michon J, Mary P, Glorion C, Helfre S, Habrand JL, Oberlin O - Sarcoma (2012)

Progression-free survival of the 59 patients according to age (±10 years).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3420075&req=5

fig4: Progression-free survival of the 59 patients according to age (±10 years).
Mentions: In univariate analysis, younger age and head and neck sites were favorable prognostic factors for PFS. The 10-year PFS rate was 46% for children younger than 10 versus 6% for older children (P = 0.03—Figure 4). The 10-year PFS for patients with a head and neck (H/N) primary DT was better than for those with a limb or trunk DT (69% versus 14% versus 25%, resp., P = 0.01—Figure 5). Gender had no impact on PFS. The status of surgical margins did not appear to be a prognostic factor, as IRS staging was not found to be a significant prognostic factor for PFS (5-year PFS: 33% versus 16% versus 48% for IRSI versus IRSII versus IRSIII groups, respectively, P = 0.45). Ten-year PFS rates according to age (±10 year) and primary site (head and neck versus other primary) were statistically different: 62% for patients with H/N DT under the age of 10 versus 32% for patients with no H/N DT and under the age of 10 versus 5% for patients with no H/N DT and over the age of 10 (P = 0.01).

Bottom Line: Conclusions.When surgery is required, surgical margins must be negative.Low-dose chemotherapy can be proposed as adjuvant therapy.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Oncology Department, Hôpital de la mère et de l'enfant, 8 rue Dominique Larrey, 87042 Limoges, France.

ABSTRACT
Background. Only limited data are available concerning desmoid tumor in children. Methods. Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed. Results. Median age was 6 years (range, 0-15). Tumors mainly involved the limbs (42%). Five cases occurred in a context of genetic disorder. Surgery was first-line treatment in 80% of cases. Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases. Various adjuvant therapies were used. Overall response to all systemic therapies was 33%. Thirty-eight patients developed one or more recurrences or progressions. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS. Conclusions. When surgery is required, surgical margins must be negative. Low-dose chemotherapy can be proposed as adjuvant therapy. Prospective trials must be developed to evaluate long-term response and side effects.

No MeSH data available.


Related in: MedlinePlus