Department of Pathology, University of Oklahoma Health Sciences Center

NeuroTest Sample Question #41 Next question Previous question

Answer: D (Langerhans' cell histiocytosis) Level of difficulty: 2 NeuroTest NeuroLearn NeuroHelp

Discussion: 

• Pathology of the case: Two major populations of cells are illustrated here. Many of them are eosinophils (Þ). Many cells with small to medium amount of cytoplasm. The characteristic deeply grooved  "coffee bean" nuclei can be well appreciated at the level of light  (Þ) and electron microscopy. These cells are the neoplastic Langerhans' cells. Langerhans' cells are immunohistochemically reactive for CD1a and S100 protein. Ultrastructurally, Birbeck granules are also seen.  It should be noted that the amount of neoplastic Langerhans' cells may vary from abundant as in this case to scant. The same token is true for the amount of eosinophils. Other inflammatory cells and macrophages can also present. On cytologic preparation and also in histologic sections, macrophages closely resemble the neoplastic Langerhans cells. Macrophages, however, are negative for CD1a and S100. [Click here to see a case]. Clinically, Langerhans' histiocytosis often occurs in the head and neck areas as osteolytic lesions. The larger ones may extend into the soft tissue.
• Meningioma: Although meningiomas at the cranial base may invade the surrounding bone and through the bone into the surrounding soft tissue, they are not typically associated with a substantial amount of eosinophils and their nuclei are not grooved. Meningioma cells in cytologic preparations tend to stay in clusters with an epithelioid appearance. Pseudonuclear inclusion are also common. Meningioma cells, however, can also have nuclear groove.
• Acute infection with abscess: The polymorphonuclear leukocytes here are almost exclusively eosinophils. Neutrophils are almost always present in acute infection and abscess formation. Necrotic debris should also be seen. On the other hand, the "coffee bean" cells should not be representing a significant proportion of the cells in abscess formation.
• Hodgkin's lymphoma: The Reed-Sternberg cells are far more pleomorphic than the neoplastic Langerhans' cells. A pure background of eosinphils rather than a background of mixed inflammatory cells is rather unusual for Hodgkin's lymphoma.
• Myeloid sarcoma: The myeloid sarcoma cells are also far more pleomorphic than the Langerhans' cells. [Click here to see a similar case]

Comment: KarMing-Fung@ouhsc.edu