Pediatric Unicentric Castleman Disease of the Temporalis Muscle

Castleman disease (CD), which is also known as angiofollicular lymph node hyperplasia or giant node hyperplasia, is a rare spectrum of lymphoproliferative disorders.¹ Castleman disease is most popularly characterized by its clinical manifestation, unicentric or multicentric, as its clinical features, epidemiology, response to treatment, and prognoses differ along that divide.² Unicentric CD (UCD) is a unifocal lymphadenopathy that affects 1 lymph node or 1 chain of lymph nodes.¹ It typically presents with an isolated nontender mass that may be symptomatic because of compressive effects on adjacent structures but typically does not present with systemic signs or laboratory abnormalities.¹ In contrast, multicentric CD typically presents with multifocal, diffuse lymphadenopathy, hepatosplenomegaly, constitutional symptoms, and anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated inflammatory markers.³