Solitary Langerhans cell histiocytosis of the cavernous sinus with orbital extension in an adult

Excerpt

We report a solitary Langerhans cell histiocytosis (LCH) of the cavernous sinus with orbital extension in an adult. A 55-year-old male patient was admitted with worsening headache and visual impairment in the right eye. On physical examination, there was proptosis of the right eye. The remainder of the physical examination was normal. The patient had no endocrine manifestations of LCH, such as diabetes insipidus, hypogonadism, or growth failure; he also had no central nervous system manifestations, such as ataxia or cognitive dysfunction. An intravenous contrast-enhanced magnetic resonance imaging (MRI) examination was performed. This showed a lesion in the right cavernous sinus with relative heterogeneous mixed intensity on T2-weighted images. The lesion showed diffuse homogeneous enhancement after gadolinium contrast administration. The lesion invaded Meckel’s cave, enfolded the carotid artery for approximately 180°, and extended to the pterygopalatine fossa. The lesion also extended to the infraorbital region and erased the fatty planes around the lateral rectus muscle. A primarily malignant tumor was considered. No primary lesion was identified either on abdominal and thorax computed tomography (CT) or on bone scintigraphy. The patient underwent a right frontotemporal craniotomy, the tumor in the cavernous sinus was excised. On histological and immunological examination, CD1a was positive. The lesion was histopathologically confirmed as LCH (Fig. 1).

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