[Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma].

UNLABELLED: Soft tissue sarcoma has a primitive mesenchymal origin and represents a heterogeneous group of malignant entities with a continuous rising frequency in the age range below 18. Rhabdomyosarcoma (RMS) constitutes 5.8% from the whole amount of pediatric solid tumors, taking the fourth place after CNS tumors, neuroblastoma, and Wilms tumors. The other category of non-rhabdomyosarcoma tumors in children and teenagers represents 3% of the solid malignancies under 18 years old. Our study looks at the particularities of the pathological, clinical, therapeutic aspects and the statistical correlation with prognosis. We included in the study a well-known category of soft tissue tumors called of uncertain malignancy.

MATERIAL AND METHOD: The study was made between January 1995 and July 2005 in the Pediatric Department of Hematology and Oncology of the "Sf. Maria" Clinical Emergency Hospital Iaşi on a group of 58 de patients ranging between 0 and 18 years old.

RESULTS

Positive diagnostic confirmation was established on pathological grounds in optic microscopy and immuno-histo-enzymology performed on bioptic samples in Department of Pediatric Surgery and analyzed in Pathology Laboratory of "Sf. Maria" Clinical Emergency Hospital Iaşi. Based on histological examination 19 cases (32.75%) were of rhabdomyosarcoma type with following subtypes: alveolar--7 patients, embryonic-- 9 cases, fusiform - 2 cases, bothrioid--1 case), 8 cases were undifferentiated soft tissue sarcomas and one patient had a tumor of pleiomorphic type; 13 children (22.41%) had non-rhabdomyosarcoma soft tissue sarcomas: 6 fibrosarcomas, 2 synovial sarcomas, 1 leiomyosarcoma, 1 Kaposi sarcoma, 1 case of malignant peripheral nerve sheath tumor, 1 case of angioma tumor, one liposarcoma; 16 cases were included in soft tissue tumors of uncertain origin (fibromatosis--6 cases, fibrous histiocytoma--4 cases, hamartoma--cases, myoblastoma--1 case, fibro-xanthoma--1 case, hemangioendothelioma--1 case); 1 PPNET (Askin tumor).

CONCLUSIONS

The continuously augmented incidence of soft tissue sarcoma in young ages and the advanced stages initial presentations of tumors require a much more serious and rhythmic survey at general practitioner level. An improvement of diagnostic means is useful, taking into account the limits of optic microscopy. Therapy must be stage and histology adapted; lowering risk toxicity of good prognosis cases and enhancing doses when required are the only reasonable options to avoid over treatment and to have a much better survival rate.