Intracranial Rosai-Dorfman Disease: A Case to Remember

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML), an alternative term for Rosai-Dorfman disease (RDD), is a rare benign idiopathic immune-related lymphoproliferative condition. The central nervous system (CNS) has been documented to be involved in RDD, although lymph nodes are the organs that are most frequently and primarily associated with the disease manifestation. Nonetheless, CNS involvement in RDD is rare and poorly understood. As a result, there is a lack of a solid basis for therapeutic approaches for CNS involvement in RDD. Here, we present a case of RDD with cerebral involvement, a rare presentation of RDD with atypical symptoms. A brief assessment of the radiographic appearance, histological findings, and the peculiar manifestations of the disease is provided.

Keywords: benign lymphoproliferative; hemophagocytic lympho-histiocytosis; intracranial neoplasms; neuro radiology; pet ct scan; rosai-dorfman disease; sinus histiocytosis with massive lymphadenopathy.

Figure 1. Histopathology of sinonasal mass biopsy specimen

Hematoxylin and eosin stain smear showing a macrophage with lymphocytic emperipolesis (red arrow).

Figure 2. Magnetic resonance imaging with contrast

A. Coronal contrast section showing sinonasal involvement (yellow arrow). B. Axial contrast section showing bilateral maxillary sinus, left sphenoid sinus involvement (yellow arrows).

Figure 3. Magnetic resonance imaging with contrast

A. Axial T1WI fast spin echo contrast sections with a yellow arrow pointing at left orbital involvement. B. Axial T1WI contrast section with a yellow arrow pointing at left orbital involvement.

Figure 4. Magnetic resonance imaging with contrast images

A. Axial T1WI contrast section showing multiple heterogeneously enhancing mass in the supra and parasellar region suggesting intracranial involvement of disease (yellow arrow). B. Coronal T1WI contrast section showing multiple heterogeneously enhancing mass in the supra and parasellar region suggesting intracranial involvement of disease (yellow arrow). C. Sagittal T1WI contrast section showing heterogeneously enhancing mass in the suprasellar region suggesting intracranial involvement of disease (yellow arrow).

Figure 5. Magnetic resonance imaging spectroscopy

A. Contrast-enhanced magnetic resonance imaging of the brain with voxel placement in the region of interest. B. Magnetic resonance imaging spectroscopy suggesting reduced N-acetylaspartate (NAA) and raised choline with an increased choline-creatinine ratio in the region of interest.

Figure 6. Positron emission tomography-computed tomography scan

A. Positron emission tomography-computed tomography scan of the head showing intracranial extension, the intracranial component of metabolically active mass (yellow arrow). B. Contrast-enhanced computed tomography scan of the brain showing intracranial extension, the intracranial component of metabolically active mass (yellow arrow). C. Positron emission tomography-computed tomography scan fusion image showing intracranial extension, the intracranial component of metabolically active mass (yellow arrow).

Figure 7. Positron emission tomography-computed tomography scan

A. Positron emission tomography-computed tomography scan of the head reveals an ill-defined minimally enhancing mass lesion in the masticator space on the left side, showing low-grade diffuse fluorodeoxyglucose uptake with standardized uptake value max of 6.1 with obliteration of the retroantral fat on the left side. There is a superior extension of the mass reaching up to the apex of the left orbit (yellow arrow). B. Contrast-enhanced computed tomography scan of the head reveals an ill-defined minimally enhancing mass lesion in the masticator space on the left side, showing low-grade diffuse fluorodeoxyglucose uptake with standardized uptake value max of 6.1 with obliteration of the retroantral fat on the left side. There is a superior extension of the mass reaching up to the apex of the left orbit (yellow arrow). C. Positron emission tomography-computed tomography fusion scan of the head reveals an ill-defined minimally enhancing mass lesion in the masticator space on the left side, showing low-grade diffuse fluorodeoxyglucose uptake with standardized uptake value max of 6.1 with obliteration of the retroantral fat on the left side. There is a superior extension of the mass reaching up to the apex of the left orbit (yellow arrow).

Figure 8. Positron emission tomography-computed tomography scan

A. Coronal positron emission tomography-computed tomography scan showing increased metabolic activity in the head region (yellow arrow). B. Sagittal Positron emission tomography-computed tomography scan showing increased metabolic activity in the head region (yellow arrow).