Os - Bone

0804267

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  F de 13 ans , tumefaction diaphysaire fémorale gauche.   13 yo girl, Swelling of the midshaft of left femur.  
       
     
     
     
     
     
  Biopsie chirurgicale:   Surgical biopsy:  
       
     
     
     
     
     
     
     
     
     
     
       
     
     
  Zone?      
     
     
 

Diagnostic proposé

 

 

 

 

 

 

 

 

Ostéosarcome périostéal

 

Proposed diagnosis

 

 

 

 

 

 

 

 

Periosteal osteosarcoma

 
 

Arguments

Tumeur diaphysaire, périostée, à extension en surface du fémur (La plupart des cas rapportés sont localisés au niveau du tibia ou du fémur)

La lésion semble être limitée à la corticale.

Prolifération productrice d'ostéoïde avec large composante chondroïde (parentée avec le chondrosarcome périostéal)

 

 

Tumor grows on the surface of the femur(or other long bone: Most of the reported cases have been located in the upper tibial shaft or femur )

Lesion is limited to the cortex (and only rarely invade the medullary cavity)

Osteoid forming lesion with a prominent cartilaginous component (Tumour closely related to juxtacortical or periosteal chondrosarcoma)

 
 

Abstracts on periosteal osteosarcoma in

 

 
 

Sarcoma. 2002;6(4):123-30

Periosteal Osteosarcoma: A Review of 17 Cases with Mean Follow-up of 52 Months.

Revell MP, Deshmukh N, Grimer RJ, Carter SR, Tillman RM

The.Royal Orthopaedic Hospital Oncology Service Bristol Road South Northfield Birmingham B31 2AP UK.

Purpose: Periosteal osteosarcomas are rare cartilage-rich bone tumours characterized by a juxtacortical eccentric position and are normally regarded oncologically as of intermediate to high grade.Their low incidence is mirrored by a small number of reported cases in the world literature. While there is general agreement that wide surgical excision is required, there is a paucity of evidence regarding adjuvant therapy. Previous reports have not indicated any consistent approach to this to allow appraisal.Patients and methods: We report 17 cases treated at our centre over 16 years. Our policy was to use chemotherapy when the tumour showed any features of high grade.Results: To date, no deaths have resulted from recurrence or metastasis of the tumour although there have been two deaths from other causes.Discussion: Comparison of survival with existing studies is made to draw conclusions regarding future treatment of this condition in terms of surgical and adjuvant approaches.

 
 

 

Clin Orthop Relat Res. 2006 Dec;453:314-7.

Periosteal osteosarcoma: long-term outcome and risk of late recurrence.

Rose PS, Dickey ID, Wenger DE, Unni KK, Sim FH

Departments of Orthopedic Surgery, 200 First Street SW, Rochester, MN 55905, USA.

The long-term outcome of periosteal osteosarcoma is not well defined. We sought to examine the disease-specific survival and risk of late recurrence or dedifferentiation in a cohort of 29 patients with average of 15.8 years followup. Disease-free survival was 83%, with five patients dying of disease at an average of 26 months after presentation. Survival was similar with respect to anatomic location, pathologic grade, and limb-salvage resection. All instances of local recurrence, metastatic disease, and death occurred within 3 years after presentation. There were no instances of dedifferentiation. Long-term disease-free survival is possible after resection of the local recurrence. Limb-salvage therapy seems to offer survival equivalent to amputation, and there does not seem to be a substantial risk of late recurrence, dedifferentiation, or disease progression.