Chemotherapy

A large retrospective analysis of the German and Italian experience with this tumor reported the following results:[1]

A large retrospective analysis of the German and Italian experience with this tumor reported the following results:[1]

Sixty-five percent of measurable tumors had objective responses to ifosfamide-containing chemotherapy regimens.

A large retrospective analysis of the German and Italian experience with this tumor reported the following results:[1]

The analysis did not conclusively demonstrate improved survival with chemotherapy.

A large retrospective analysis of the German and Italian experience with this tumor reported the following results:[1]

This retrospective analysis also noted a trend toward improved outcome with postoperative radiation therapy.

A series of 37 young patients with MPNST and NF1 showed that most patients had large invasive tumors that were poorly responsive to chemotherapy.[20]

A series of 37 young patients with MPNST and NF1 showed that most patients had large invasive tumors that were poorly responsive to chemotherapy.[20]

The progression-free survival (PFS) rate was 19%, and the 5-year OS rate was 28%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

Cohort 1: Patients with completely resected tumors (R0) who received surgery only (n = 13); the 5-year EFS rate was 92%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

Cohort 2: Patients with incompletely resected tumors (R1/R2) who received adjuvant radiation therapy (n = 4); the 5-year EFS rate was 33%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

Cohort 3: Patients with incompletely resected tumors (R1/R2) who received adjuvant chemotherapy (n = 7); the 5-year EFS rate was 29%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

Cohort 4: Patients who received either chemotherapy before surgical resection and/or who had nodal involvement (n = 27); the 5-year EFS rate was 52%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

The response rate to chemotherapy (partial response + complete response) in patients with measurable disease was 46%.

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) performed a prospective study in patients aged 21 years and younger with MPNST.[21] Surgical resection of primary tumors was classified as R0 if the resection was complete with negative microscopic margins, R1 if the margins were microscopically positive, and R2 if the resection left macroscopic residual tumor. Patients were nonrandomly assigned to one of the following four treatment groups:For patients who received chemotherapy, treatment consisted of four courses of ifosfamide/doxorubicin and two courses of ifosfamide concomitant with radiation therapy (50.4–54 Gy).

The presence of NF1 (51% of patients) was an independent poor prognostic factor for OS and EFS.

In a study of pediatric and adult patients with either sporadic (n = 14) or chemotherapy-naïve, NF1-associated (n = 34) MPNST, patients were treated with two cycles of ifosfamide and doxorubicin and two cycles of ifosfamide and etoposide.[22]

In a study of pediatric and adult patients with either sporadic (n = 14) or chemotherapy-naïve, NF1-associated (n = 34) MPNST, patients were treated with two cycles of ifosfamide and doxorubicin and two cycles of ifosfamide and etoposide.[22]

Response rates were lower in patients with NF1-associated tumors than in patients with sporadic tumors (17.9% vs. 44.4%). However, the premature closure of the study did not allow sufficient power to detect meaningful differences in objective responses between the two groups.

In a study of pediatric and adult patients with either sporadic (n = 14) or chemotherapy-naïve, NF1-associated (n = 34) MPNST, patients were treated with two cycles of ifosfamide and doxorubicin and two cycles of ifosfamide and etoposide.[22]

The rates of stable disease were similar between the two groups.