Sinus histiocytosis presenting as a mediastinal mass

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare, benign proliferative disorder of unknown etiology.
• It is characterized by massive, painless lymphadenopathy, typically affecting cervical lymph nodes, along with fever and elevated erythrocyte sedimentation rate.
• Extranodal involvement is common, affecting various organs including the central nervous system.

Observation:

• A 32-year-old male presented with a decade-long history of bilateral cervical adenopathy, parotid gland swelling, and a posterior mediastinal mass.
• The patient subsequently developed headaches, prompting neuroimaging.
• A computed tomography (CAT) scan of the head revealed a suprasellar mass.

Findings:

• Craniotomy and biopsy of the suprasellar lesion were performed.
• Histopathological examination confirmed the diagnosis of sinus histiocytosis.

Implications:

• This case underscores the importance of considering sinus histiocytosis in the differential diagnosis of suprasellar masses, particularly in patients with a history of lymphadenopathy.
• Early recognition and diagnosis are crucial for appropriate management and to prevent potential complications associated with mass effect.
• Further research into the pathogenesis and optimal treatment strategies for extranodal sinus histiocytosis is warranted.