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Radiological Findings, Disease course, and Management

 

Radiograph Of the Right Thigh 

                            03 January 2015

                   AP                                      Lateral 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

                                              

 

  • A Solitary mildly expansile geographic lytic lesion involving the diaphysis of femur with wide zone of transition

  • Focal endosteal scalloping seen along the anterior cortex

  • Solid periosteal reaction present

  • No cortical break

  • No associated soft tissue component

 

 MRI OF Right Thigh                                                            

                                      T2W

 

 

 

 

 

 

 

 

 

 

 

 

 

                                                                       

                                   

 

                                       T1W

                                      

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

                                 

                                    T1W FS Gd

 

                                  

              

 

 

 

 

 

 

 

 

 

 

 

Findings

Lesion

  • appears heterogeneously hyperintense on T2W

  • appears isointense on T1W images

  • shows strong heterogeneous enhancement in post contrast study

Surrounding soft tissues

  • Abnormal contrast enhancement

 

Provisional Diagnosis:

Ewing’s sarcoma on imaging findings.

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Initial biopsy report:

Ewing’s sarcoma

 

Course

  • Patient underwent repeat biopsy at CMC hospital, Vellore

  • Reported as Eosinophilic granuloma

 

Treatment

  • Patient underwent surgery

  • Lesion  curettage+ bone graft substitute +local pamidronate and rush rodding on 21/01/2015

 

Follow up Radiograph

                                18 January 2016

                                                           

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Follow-up after 1 year

Patient well and asymptomatic

 

Discussion

Also called Langerhans cell histiocytosis.

Etiology: Unknown 

          ? an inflammatory reaction to some agent of infection

Age:    Children and young adults

Site:    Any bone proximal to wrist and ankle

Most common:  Skull, ribs, femur, vertebrae, pelvis, mandible, humerus,                  and clavicles

In long bones:   Lesion is usually situated in the shaft. 

Subperiosteal new bone formation as seen in this case is an unusual finding resembling the classical description of Ewing’s tumor

Radiologic Findings

  • Affects the skeleton in a monostotic or polyostotic fashion

  • Presents as a well-defined osteolytic lesion with a benign periosteal reaction or

  • as an aggressive lesion with ill-defined margins and an aggressive type of periostitis mimicking Ewing sarcoma or osteomyelitis.

Sites involved with monostotic eosinophilic granuloma include

  • calvarium

  • mandible 

  • ribs 

  • long bones: usually shaft 

  • pelvis and

  • vertebrae

Tubular bone involvement 

  • diaphyseal and metaphyseal localization more frequent than epiphyseal localization

Long bones below knees and distal to the elbows rarely involved.

Lesions are

  • lytic

  • round or oval

  • expansile with ill-defined or sclerotic margins 

  • medullary cavity may be expanded

  • may be associated with cortical thinning, intracortical tunneling, and erosion of the cortex or an adjacent soft-tissue mass

  • Laminated periosteal new bone formation is common around the involved segment of bone

  • May appear rapidly over 3 weeks

MRI: Common findings

  • Focal lesion surrounded by extensive ill-defined bone marrow and     soft tissue reaction

  • Low signal intensity on T1-weighted images

  • High signal intensity on T2-weighted images

  • Considered to represent bone marrow and soft tissue edema (the flare phenomenon)

  • Early stages MRI findings nonspecific 

  • May simulate an aggressive lesion like osteomyelitis or Ewing's sarcoma or benign bone tumors like osteoid osteoma or chondroblastoma

              
Differential Diagnosis

In case of long bones differential diagnosis includes

  • Ewing sarcoma 

  • chronic osteomyelitis

  • Brodie abscess and 

  • chondroblastoma should be considered.

 

As seen in this case it is difficult to radiologically exclude Ewing's sarcoma which usually shows an irregular and interrupted periosteal reaction.

 

References

 

Case 4

                                                                                  Contributor

                                            Prof.Dr. S.K. Joshi

Diagnosis

Eosinophilic Granuloma

 
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