Sinus histiocytosis with massive lymphadenopathy

Area of Science:

• Pathology
• Oncology
• Otorhinolaryngology

Background:

• Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare, benign pseudolymphomatous condition.
• SHML typically presents with massive cervical adenopathy in children and young adults.
• Extranodal involvement is common in SHML.

Purpose of the Study:

• To describe the clinical features and outcomes of SHML patients with upper respiratory tract and/or salivary gland involvement.
• To evaluate if extranodal SHML, specifically in the head and neck, is associated with a more aggressive disease course.

Main Methods:

• Retrospective case series of 16 patients with SHML involving the upper respiratory tract and/or salivary gland.
• Clinical data, including presenting symptoms, treatment modalities, and outcomes, were analyzed.
• Comparison of clinical presentation and disease behavior between patients with and without ENT involvement.

Main Results:

• Nine of the 16 patients presented with ear, nose, and throat (ENT) manifestations, often causing significant symptoms.
• Treatments included surgery, antibiotics, irradiation, chemotherapy, and steroids, with no consistent response pattern observed.
• Patients with ENT involvement showed clinical similarity to those without, suggesting extranodal disease is not linked to increased aggressiveness.

Conclusions:

• SHML can manifest in the upper respiratory tract and salivary glands, presenting with prominent clinical symptoms.
• Extranodal involvement in SHML, including the head and neck region, does not appear to correlate with a more aggressive disease phenotype.
• Further research may elucidate optimal treatment strategies for SHML with extranodal involvement.