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Soft Tissue Sarcoma

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The Surgery of Childhood Tumors

Abstract

Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that account for 7.4 % of all pediatric malignancies. They are generally classified into two broad categories: nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) and rhabdomyosarcomas (RMS) with RMS being the most common STS among children and adolescents. The epidemiology, clinical presentation and diagnosis of each tumor type as well as the staging work-up, treatment approach and anticipated outcomes will be discussed. Attention will be given to the surgical approach in the management of these tumors including a discussion of newer techniques such as sentinel lymph node biopsy. The following NRSTS histologies will be reviewed: alveolar soft part sarcoma, desmoplastic small round cell tumor (DSRCT), infantile and adult-type fibrosarcoma, fibrohistiocytic tumors, leiomyosarcoma, liposarcoma, malignant peripheral nerve sheath tumor (MPNST) and synovial sarcoma. A general overview of RMS will be given as well as a site based discussion of the disease.

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Abbreviations

AIEOP:

Associazione Italiana di Ematologiae Oncologia Pedaitrica

AJCC:

American Joint Commission on Cancer

ASPS:

Alveolar soft part sarcoma

ATFS:

“Adult type” fibrosarcoma

BSA:

Body surface area

COG:

Children’s Oncology Group

COG-STS:

Children’s Oncology Group Soft Tissue Sarcoma Committee

COL1A1:

Collagen type 1, alpha 1 gene

CT:

Computerized tomography

DFSP:

Dermatofibrosarcoma protuberans

Dox:

Doxorubicin

DSRCT:

Desmoplastic small round cell tumor

E:

Etoposide

EFS:

Event free survival

EWS:

Ewing sarcoma gene

FDG:

Fluorine-18-fluorodeoxyglucose

FDG-PET:

Fluorodeoxyglucose positron emission tomography

FHT:

Fibrohistiocytic tumors

FISH:

Fluorescent in situ hybridization

FNCLCC:

French Federation of Cancer Centers Sarcoma Group

FSRT:

Fractionated stereotactic radiotherapy

HART:

Hyperfractionated accelerated radiotherapy

HIPEC:

Hyperthermic intraperitoneal chemotherapy

I:

Ifosfamide

IFS:

Infantile fibrosarcoma

IGF-II:

Insulin-like growth factor-2

IGF-IR:

Insulin-like growth factor-1 receptor

IMRT:

Intensity-modulated radiation therapy

IRS:

Intergroup Rhabdomyosarcoma Group

IRSG:

Intergroup Rhabdomyosarcoma Study Group

LOH:

Loss of heterozygosity

MFH:

Malignant fibrous histiocytoma

MMS:

Mohs micrographic surgery

MPNST:

Malignant peripheral nerve sheath tumor

MRI:

Magnetic resonance imaging

NCCN:

National Comprehensive Cancer Network

NCI:

National Cancer Institute

NF1:

Neurofibromatosis type 1

NOS:

Not otherwise specified

NRSTS:

Nonrhabdomyosarcoma soft tissue sarcomas

OS:

Overall Survival

PCR:

Polymerase chain reaction

PDGF:

Platelet-derived growth factor

PDGFB:

Platelet-derived growth factor beta

PET:

Positron emission tomography

PFS:

Progression-free survival

POG:

Pediatric Oncology Group

PRE:

Pre-treatment re-excision

RMS:

Rhabdomyosarcoma

RT:

Radiation therapy

SEER:

Surveillance Epidemiology and End Results

STS:

Soft tissue sarcomas

STSC:

Soft Tissue Sarcoma Committee

VA:

Vincristine (V) actinomycin D (A)

VAC:

Vincristine (V) actinomycin D (A), and cyclophosphamide (C)

WHO:

World Health Organization

WLE:

Wide local excision

WT1:

Wilms’ tumor gene 1

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Authors and Affiliations

  1. Department of Surgical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

    Mary T. Austin MD, MPH

  2. Department of Surgery, University of Texas Medical School at Houston, Houston, TX, USA

    Richard J. Andrassy MD, FACS, FAAP

  3. Department of Surgery, University of Texas Medical School at Houston, Houston, TX, USA

    Richard J. Andrassy MD, FACS, FAAP

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  1. Mary T. Austin MD, MPH
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Correspondence to Mary T. Austin MD, MPH .

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  1. Surgical Paediatrics, University of Glasgow, Glasgow, United Kingdom

    Robert Carachi

  2. Indianapolis, Indiana, USA

    Jay L. Grosfeld

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Austin, M.T., Andrassy, R.J. (2016). Soft Tissue Sarcoma. In: Carachi, R., Grosfeld, J. (eds) The Surgery of Childhood Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48590-3_20

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