PMC5977054_01
MRI brain showing a right orbital mass and a left mastoid mass.
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He underwent imaging studies which revealed a left mastoid/middle ear fossa mass and a right orbital mass (Figure 1).
CT chest/abdomen/pelvis showing soft tissue encasing the adrenal glands, kidneys, infrarenal abdominal aorta, and soft tissue in the presacral space consistent with Erdheim-Chester disease.
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CT scans revealed extensive bilateral intraconal enhancing soft tissue, infiltrative soft tissue involving the right sphenoid wing with extension into the pterygopalatine fossa and right temporal fossa, and enhancing soft tissue identified within bilateral frontal sinuses (Figure 2).
PET scan showing varying degrees of negligible, low-grade, and mild-to-moderate metabolic uptake consistent with multifocal soft tissue and osseous manifestations of Erdheim-Chester disease.
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Repeated CT evaluations over the next 3 years demonstrated no evidence of progression of disease on PEGylated interferon alpha-2a therapy (Figure 3).
MRI brain showing calvarial mass lesion extending intracranially.
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MRI brain showed interval development of left calvarial mass lesion extending intracranially, associated with an epidural soft tissue component measuring 4 x 1.5 x 1.5 cm (Figure 4).
Representative sections of Langerhans cell histiocytosis. (a) Low-power (20x) image demonstrates a diffusive cellular infiltrate with prominent eosinophils. (b) High-power (100x) image demonstrates populations of eosinophils, macrophages with foamy cytoplasm, and large, variably elongated spindled cells with indented, grooved nuclei and eosinophilic granular cytoplasm. These cells are diffusely positive for (d) S100 and (e) CD1a and (c) focally positive for CD68, consistent with Langerhans cells.
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The patient underwent resection of the mass with the pathology demonstrating both the skull and dural excisional specimens consistent with Langerhans cell histiocytosis with immunohistochemistry noting the cells as CD1a-positive, S100-positive, and largely CD68-negative (Figure 5).