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Presented by Edward McCarthy, M.D. and prepared by Maryam Farinola M.D.
Case 3: A 15 year old boy had pain in his knee for one year.
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1. Question
Week 214: Case 3
A 15 year old boy had pain in his knee for one year. A radiograph demonstrated a well-defined lytic lesion in the distal femoral metaphysis./images/langerhans 1.jpg
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/images/langerhans 3.jpgCorrect
Answer: Langerhan’s cell histiocytosis (eosinophilic granuloma)
Histology: Langerhans’ cell histiocytosis in bone, formerly known as eosinophilic granuloma of bone, occurs predominantly in children and young adults. Charactristically, radiographic lesions are well-defined and punched out. Histologically lesions consist of heterogenous mixture of cells including rounded macrophage-like cells (the Langerhans’ cells), multinucleated giant cells, and eosinophils. This heterogeneous cells population of inflammatory cells raises the possibility of osteomyelitis. However, the macrophage-like-cells in the Langerhans’ cell histiocytosis are positive with S-100 protein and the antigen CD-1A. Therefore, any case of osteomyelitis in children or young adults should be studied with these antibodies to exclude the possibility of Langerhans’ cell histiocytosis.
Discussion: The lesion is not osteomyelitis. Although histologically osteomyelitis may look like the present case. The presence of antibody CD-1A and S-100 protein in the stromal cell is diagnostic of Langerhans’ cell histiocytosis.
Radiologically this lesion may be confused with non-ossifying fibroma. It is a well defined lytic lesion in the metaphyseal region. However, the heterogenous population of inflammatory cells is not characteristic of non-ossifying fibroma. Also, the spindle cells and a storiform pattern which is seen in non-ossifying fibroma is not present in this case.
Incorrect
Answer: Langerhan’s cell histiocytosis (eosinophilic granuloma)
Histology: Langerhans’ cell histiocytosis in bone, formerly known as eosinophilic granuloma of bone, occurs predominantly in children and young adults. Charactristically, radiographic lesions are well-defined and punched out. Histologically lesions consist of heterogenous mixture of cells including rounded macrophage-like cells (the Langerhans’ cells), multinucleated giant cells, and eosinophils. This heterogeneous cells population of inflammatory cells raises the possibility of osteomyelitis. However, the macrophage-like-cells in the Langerhans’ cell histiocytosis are positive with S-100 protein and the antigen CD-1A. Therefore, any case of osteomyelitis in children or young adults should be studied with these antibodies to exclude the possibility of Langerhans’ cell histiocytosis.
Discussion: The lesion is not osteomyelitis. Although histologically osteomyelitis may look like the present case. The presence of antibody CD-1A and S-100 protein in the stromal cell is diagnostic of Langerhans’ cell histiocytosis.
Radiologically this lesion may be confused with non-ossifying fibroma. It is a well defined lytic lesion in the metaphyseal region. However, the heterogenous population of inflammatory cells is not characteristic of non-ossifying fibroma. Also, the spindle cells and a storiform pattern which is seen in non-ossifying fibroma is not present in this case.
