Caceres’ Corner: Case No. 20 (Update: Solution)

Dear Friends,

Muppet gratefully acknowledges the contribution of his good friend Dr. Manel Escobar who discovered and diagnosed the following case:

An 11-year-old girl with migrating bone pain for the last year, with normal radiographs, now presents with one month of low-degree fever. Chest and abdominal CT were unremarkable, except for the pelvic findings.

Diagnosis:

1. Fibrous dysplasia
2. Langerhans cell histiocytosis
3. Lymphoma
4. None of the above

11-year-old girl, AP pelvis

11-year-old girl, AP pelvis

11-year-old girl, axial CT

11-year-old girl, axial CT

11-year-old girl, axial CT1

11-year-old girl, axial CT1

11-year-old girl, corinal CT

11-year-old girl, corinal CT

Click here for the answer to case #20
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    07
    May 2012
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    DISCUSSION 11 Comments

    11 Responses to : Caceres’ Corner: Case No. 20 (Update: Solution)

    1. Giuseppe Sabino says:

      Langerhans cell histiocytosis…I think…

    2. Genchi Bari Italia says:

      Le lesioni sono addensanti: nel loro contesto ci sono aree litiche , ben definite e non ilfiltranti.La malattia è cronica, con febbre. Ecludere allora il linfoma.Escluderei la displasia fibrosa poliostotica.Rimarrebbe la istiocitosi a c. di Langerhans : ma sapendo le trappole del mio illustre collega, formulerei una diagnosi, inusuale per sede ed eetà: la M. di Erdheim-Chester

    3. Sonja says:

      No. 4 -none of the above; possibly CAFFEY – SYNDROME

      • Jose Caceres says:

        It is not Caffey’s, but you gave an intriguing diagnosis. Muppet did not think of it.

    4. Alice says:

      No. 4. I would want to rule out Myelodysplastic Syndrome and/or Leukemia.

    5. Albert says:

      Multiple, radiolucent, cortically centered iliac lesions. They appear with reactive cortical thickening and sclerosis around them.

      One of the lesions (left iliac in the 3rd image) seems to contain “bone inside bone”, it suggest a sequestrum. Only a few lesions can produce a sequestrum; classically, as far as I concern, basically 4: OSTEOMIELITIS, LYMPHOMA, EOSINOPHILIC GRANULOMA and some types of sarcoma (FIBROSARCOMA).

      With this, the only one of these four differentials that could go with the case (even atipically) is MULTIPLE EOSINOPHILIC GRANULOMA (3.Langerhans Cell Histicytosis).

      No more ideas by the moment…

    6. MAK says:

      Answer: 1
      This is most likely polyostotic fibrous dysphasia associated with McCune Albright syndrome.
      There are bilateral predominantly right pelvic Lucent lesions with associated sclerosis and bony expansion noted.
      Coxa vara.
      Accelerated bony maturity.
      This is associated with precocious puberty and bone pain may be due repeated fractures
      This is more common in young girls.

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