Castleman Disease in a Child: A Rare Cause of Persistent Cervical Lymphadenopathy

Castleman disease (CD) is a rare, heterogeneous group of lymphoproliferative disorders characterized by distinctive histopathological changes. It can present as unicentric (UCD), involving a single nodal region, or multicentric (MCD), with generalized lymphadenopathy and systemic inflammation. Due to its rarity and overlapping features with infections and malignancies, diagnosis is often delayed. We report the case of a 12-year-old female who presented with progressive, multiple left-sided cervical swellings since 2023, associated with intermittent low-grade fever, headache, and fatigue. Despite empirical treatments with antibiotics and anti-tubercular therapy, her symptoms persisted. Investigations revealed anemia with hemoglobin E trait, hepatomegaly, abdominal lymphadenopathy, and minimal ascites. Cervical lymph node biopsy confirmed Castleman disease of mixed hyaline vascular and plasma cell type. Additional workup showed Epstein-Barr virus positivity, elevated IgE (>3000 IU/mL), and high IgG levels. The patient received intravenous methylprednisolone, followed by three cycles of rituximab with partial reduction of lymphadenopathy. Subsequently, a modified radical neck dissection (Type III) was performed, yielding multiple enlarged lymph nodes, the largest measuring 8 × 4 × 2 cm. Histopathology confirmed the diagnosis, showing atretic germinal centers, hyalinisation, intrafollicular vascular proliferation, and plasma cell infiltrates. This case highlights the diagnostic challenges of Castleman disease in children, its overlap with common conditions like tuberculosis, and the importance of multidisciplinary evaluation and combined medical-surgical therapy for optimal outcomes.