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Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites.

Abstract：

BACKGROUND:Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. METHODS:Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent. RESULTS:Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery-bowel in 12 cases, lung-pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and/or chemotherapy, according to their characteristics. For the series as a whole, the 5-year event-free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae). CONCLUSIONS:This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Ferrari A,Magni C,Bergamaschi L,Cecchetto G,Alaggio R,Milano GM,Bertolini P,Basso E,Manzitti C,Di Martino M,Giurici N,Melchionda F,Cecinati V,Chiaravalli S,Affinita MC,Scagnellato A,Casanova M,Bisogno G

doi

10.1002/pbc.26490

subject

Has Abstract

pub_date

2017-09-01 00:00:00

issue

eissn

1545-5009

issn

1545-5017

journal_volume

pub_type

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Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites.