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Review Question - QID 3363

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QID 3363 (Type "3363" in App Search)
A 7-year-old boy complains of pain in the right thigh for 1 month with no history of traumatic injury. A radiograph is provided in Figure A. A histology specimen from incisional biopsy is provided in Figures B and C. Which of the following is the most appropriate management?
  • A
  • B
  • C

Conservative treatment with crutches and repeat radiographs in 6 weeks

21%

395/1907

Wide excision

5%

103/1907

Wide excision and chemotherapy

61%

1155/1907

Radiation therapy alone

6%

120/1907

Curettage and bone grafting

6%

119/1907

  • A
  • B
  • C

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The clinical presentation, imaging, and histology is consistent with eosinophilic granuloma, a benign neoplasm of histiocytes. The radiograph shows a poorly defined lesion that has invaded the cortex of the femoral diaphysis. The diagnosis is confirmed by histology with the proliferation of Langerhans cells and the presence of eosinophils (red with bilobed nuclei). This form of histiocytosis is self-limiting and may be managed conservatively. Worsening pain and further destruction of the cortical bone would be indications for radiation therapy or curettage and grafting/cementation to prevent pathologic fracture. Pathognomonic findings for eosinophilic granuloma include coffee bean indented nuclei in Langerhans cells (blue arrow in Illustration A) and tennis-racket shaped Bierbeck granules within the Langerhans cell (shown on electron microscopy in Illustration B). Other commonly tested features of eosinophilic granuloma include spinal involvement resulting in vertebra plana, and the two histiocytosis syndromes (Hans Schuller Christian and Letterer-Siwe).

The unrelated publication by Elliot et al demonstrates patient safety with use of monoclonal antibody to TNF-alpha.

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