staging

Steps

1. Establish a differential

2. Stage locally and systemically

3. Biopsy

4. Definitive Treatment

1. Establish a Differential

Lesion detected on X-ray

Questions

- what do you think it is?

Definition

Anterolateral bowing

True bone dysplasia

- hamartomatous area in the tibia

- non union or potential non union through pathological fracture in this region

The defect in the bone is filled with mature, fibrous connective tissue

- not really a pseudoarthrosis

- by definition, a pseudarthrosis has a cleft lined by fibrocartilage containing fluid and bounded by a capsule

Most common sarcomas 

1. MFH

2. Liposarcoma

3. Rhabdomyosarcoma (Paediatric)

4. Synovial Sarcoma 

5. Leiomyosarcoma

6. Fibrosarcoma

Ten Tissues of Origin

1.  Adipose / LS

2   Fibrous / FS

3.  Fibrohistiocytic / MFH

Principles

Treatment algorithm similar to OS

Overall prognosis similar to OS

- 70% long term survival

Definition

A malignant neoplasm composed of small round cells of uncertain histogenesis

Genetics

Recent data suggests it is of neuroepithelial derivation

- ? neuroectodermal cells