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From the American Head and Neck Society
August 13, 2020

Pediatric Unicentric Castleman Disease of the Temporalis Muscle

Author Affiliations
  • 1University of Missouri, Otolaryngology Residency, Columbia
  • 2Bon Secours Mercy Health, St. Elizabeth Boardman Otolaryngology Residency, Boardman, Ohio
  • 3University Hospitals, Otolaryngology Head and Surgery Department, Cleveland, Ohio
JAMA Otolaryngol Head Neck Surg. 2021;147(1):103-104. doi:10.1001/jamaoto.2020.1929

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

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