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Low-grade Nonrhabdomyosarcoma Soft Tissue Sarcoma

What is Peculiar for Childhood

Fawzy, Mohamed MD; Sedky, Mohamed MD; ElZomor, Hossam MD; El Sherbiny, Magdy MD; Salama, Emad MD; Mahdy, Ahmed MSc

Journal of Pediatric Hematology/Oncology: November 2016 - Volume 38 - Issue 8 - p 615–621
doi: 10.1097/MPH.0000000000000657
Original Articles
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Background: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence.

Aim of the Study: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients.

Patients and Methods: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013.

Results: Out of the reviewed cases 32 patients had LG tumors and were eligible for analysis. The male to female ratio was 1:1 and the median age was 7.5 years (range, 1 mo to 18 y). Desmoid fibromatosis (N=18) showed frequent local recurrence and nearly half of this group was alive without disease. No recurrence of the disease occurred in the nonfibromatosis group (n=14) and all patients were alive and free of disease. The 5-year overall survival was 88% for the entire group of study patients versus 45% for event-free survival. Tumors >5 cm in diameter and fibromatosis histology subtype were associated with lower EFS.

Conclusions: LG-NRSTS generally has good prognosis, with overall survival reaching 90%. However, aggressive fibromatosis usually runs a poorer course in the form of high incidence of local recurrence and lower survival rates. This needs to be further assessed in larger prospective studies including novel therapies in addition to the current conventional modalities.

Departments of *Pediatric Oncology

§Surgical Oncology, National Cancer Institute, Cairo University

Department of Pediatric Oncology, Children Cancer Hospital

Department of Pediatrics, National Research Center

Department of Pediatrics, Misr Insurance Hospital, Cairo, Egypt

The authors declare no conflict of interest.

Reprints: Mohamed Sedky, MD, Pediatric Oncology Department Children Cancer Hospital 57357, 1 Sekket El Imam St. Sayyeda Zeinab, Cairo, Egypt (e-mail: mohamed.sedky@57357.com).

Received December 20, 2015

Accepted June 29, 2016

Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.