Soft tissue sarcoma (Overview)

Shaukat Khanum Memorial Cancer Hospital and Research Centre
Jibran Mohsin
Soft tissue Sarcoma
(Overview)
Fellow Surgical Oncology
SKMCH & RC, Lahore

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Outline
 Introduction
 Etiology
 Histopathology
 Classification
 Grading
 Staging
 Outcome
 Presentation
 Imaging
 Biopsy
 Treatment approach
 Follow up

Introduction
In USA (2016)
 0.7% of all new cancer cases
 0.8% of all cancer deaths
 At time of Presentation
 60% - 80% localized or regional (lymph node
metastasis) disease
 15% - 20% metastatic disease

Introduction
 Overall 5-year survival rate 50 – 60 %
 Origin
 Mesoderm  connective tissue
 Ectoderm

Introduction

Etiology
 Mostly sporadic
 Risk factors
 Previous radiation exposure
 8 – 50 x risk
 Latent period 10 years
 Chronic lymphedema
 Axillary dissection
 Filarial infection

Etiology
 Genetic predisposition
 FAP / Gardner syndrome
 Li-Fraumeni syndrome (p53)
 Von Recklinghausen disease (NF1)
 Hereditary retinoblastoma
 Oncogene: MDM2, CDK4, N-myc, c-erbB2, ras.
 Tumor suppressor gene: Rb, p53
 Translocations

Etiology
 Trauma, and
 Occupational chemical
 phenoxyacetic acids and
 wood preservatives containing chlorophenols

Histopathology

WHO Classification of Tumors of Soft
Tissue and Bone (4th edition; 2013)

MDACC Grading
High grade Intermediate grade
UPS (formerly MFH) Alveolar soft part sarcoma Myxofibrosarcoma
Synovial sarcoma Ewing sarcoma Myxoid liposarcoma
Pleomorphic liposarcoma Primitive neuroectodermal
tumor
Extraskeletal myxoid
Chondrosarcoma
Dedifferentiated
liposarcoma
Osteosarcoma Solitary fibrous tumor
(hemangiopericytoma)
MPNST Mesenchymal
Chondrosarcoma
Low grade
Rhabdomyosarcoma Clear-cell sarcoma Well-differentiated
liposarcoma.
Epithelioid sarcoma

FNCLCC (Federation Nationale des Centres de
Lutte Contre la Cancer) sarcoma grading system

AJCC staging 8th edition
 Divided into different staging systems by anatomic
site
 Head and neck
 Extremity
 Trunk,
 abdomen/thoracic visceral organs,
 retroperitoneum, and
 Other histology /sites.

 T category criteria
 vary primarily by anatomic site, and
 Superficial versus deep distinction is eliminated
 Prognostic stage grouping is also unique to each
anatomic site category
 e.g. N1 disease
 Stage III B (retroperitoneal category)
 Stage IV (extremity/trunk category)

NOT INCLUED
 desmoid tumors/deep fibromatosis
 Kaposi sarcoma
 GIST

Outcome (Prognostic factors)
 Histologic grade
 histologic subtypes
 Nodal spread (< 5 %) with exceptions
 Metastatic potential
 large tumor size (>5 cm)
 Tumors located in the head/neck or deep trunk
 Age >64 years, and
 Positive or uncertain margin status
 Mutluifocality

Presentation
 depends on the anatomic site and depth of the
tumor.
 asymptomatic mass.
 compress or invade contiguous structures
 neurologic symptoms resulting from lumbar or pelvic
nerve compression or
 obstructive gastrointestinal symptoms.

Imaging
 Goal
 local extent of a tumor and
 to exclude metastatic disease
 MRI
 imaging of choice for STS of extremities
 CT abdomen and pelvis + bone imaging
 Locally advanced extremity myxoid liposarcoma

Imaging
 CXR for the all STS
 CT chest
 suspicious lesion on CXR
 high-risk tumor (large tumor size [>5 cm], deep tumor
location, or intermediate/high-grade histology).
 Brain imaging
 alveolar soft part sarcoma

Imaging
 CT abdomen/pelvis
 modality of choice for retroperitoneal sarcoma

Biopsy
 Core-needle biopsy +/- FNA
 sampling error
 failure to sample a non lipogenic component of
dedifferentiated liposarcoma
 PET scan may help to identify the presence of a highly
active component within the heterogeneous tumor

Treatment Approach
 requires a multidisciplinary approach
 Principles of treatment
 Maximal locoregional control with complete surgical
resection when possible
 preoperative or postoperative radiation therapy, and/or
chemotherapy.

Follow-Up
 To detect recurrence
 early recognition and treatment of recurrent disease can
prolong survival.
 Extremity sarcomas most frequently recur as pulmonary
metastases,
 whereas retroperitoneal or intraabdominal sarcomas
more commonly recur locally.

Follow Up
 For 0 – 2 years, 3 monthly follow up
 For 2 - 5 years, 6 monthly follow up
 After 5 years, annual follow up with
 Chest imaging
 Primary site imaging
 MRI (or US) for an extremity tumor or
 CT for intraabdominal or retroperitoneal tumors.

THANK YOU

Soft tissue sarcoma (Overview)

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