Pediatric non metastatic non rhabdomyosarcoma soft tissue sarcomas (NRSTSS)‎ : five years experience from NCI-Egypt

الملخص EN

Purpose : Evaluation of demographic, pathological, and clinical patterns in addition to treatment outcome of pediatric NRSTS patients treated at the NCI, Egypt.

Procedure : 21 pediatric patients of NRSTS between 2001 and 2006 were included.

Clinical and pathological diagnosis and subtyping verification were done.

Patients' cohort formed of 3 treatment groups.

(1) Patients who underwent complete surgical resection with no adjuvant therapies.

(2) Patients who received chemotherapy and complete surgical resection, and group (3) Patients with localized unrersectable tumors for whom systemic chemotherapy only was given.

Demographic, clinicopathological variables, and treatment modalities were statistically evaluated and compared with the outcome.

Results : Tumors of unknown histiogenesis followed by MPNST and myxofibrosarcoma were the most frequent tumor subtypes.

Low tumor grade was in favor of better outcome.

With a median follow up of 2-years; respectively 100% and 81.1% of patients who had complete surgical resection of a localized disease with or without chemotherapy entered in CR (p=0.01).

Local failure rate was 27.2% among CR patients (n=17).

Two patients suffered local recurrance and one had distant disease metastasis.

Conclusion : Complete surgical resection with or without chemotherapy is the mainstay of therapy for localized NRSTS.

Tumor grade and surgical resection of NRSTS are 2 important predictors of prognosis.