Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Abstract

Background Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus‐8 (HHV8)‐negative multicentric CD (MCD), in a multi‐institutional cohort. Methods We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals. Demographic and clinical data were collected. Results Most patients (75%, 18/24) presented with UCD. All patients with MCD were HHV8‐negative. The most common histopathologic variant was hyaline vascular (75%, 18/24). Plasma cell variant occurred in 33% (2/6 [95% confidence intervals (CI), 4–78%]) of patients with HHV8‐negative MCD and 17% (3/18 [95% CI, 4–41%]) of patients with UCD. Systemic symptoms were present in 4 of 6 of patients with HHV8‐negative MCD and 8 of 18 of patients with UCD. Anemia and laboratory inflammation occurred in both UCD and MCD patients, with nonsignificantly higher rates of anemia and elevated C‐reactive protein in MCD patients. All but two UCD patients underwent gross total resection as definitive therapy. Among HHV8‐negative MCD patients, a combination of resection, chemotherapy, and immunotherapy was used. No UCD patients and three of six HHV8‐negative MCD patients experienced disease progression/relapse prior to lasting remission. There were no deaths. Conclusion Pediatric patients with CD most commonly have unicentric, hyaline vascular variant disease. Pediatric patients with both UCD and MCD commonly have systemic inflammation and, despite risk of progression/relapse in MCD patients, ultimately have excellent survival.

The French paediatric cohort of Castleman disease: a retrospective report of 23 patients

“…CRP level was also higher: 23.4 mg/dL (range 0-150) in children versus 2 mg/dL in adults [15]. Our results appeared to be similar to those observed in a recently published paediatric cohort of 24 patients [2] in which 44% of UCD patients had systemic symptoms.…”

“…The HV type is the most represented pathological type in paediatric UCD, 76.5%, as compared with adults, 68% [9]. Even if our patient cohort is small, paediatric patients may present more cervical lymph nodes than adults: 44 to 64.7% in children as compared with 26% in adults [2,9].…”

“…CD more likely presents as UCD (73.9-75% of cases) than MCD in children, whereas UCD represents 20.9% of CD cases in adult cohorts [2,9]. Indeed, the disease mechanism may be different because most adult cases occur in a context of immunodeficiency associated with both HIV and HHV-8 infection, unlike in children.…”

“…Two major disease phenotypes can be distinguished: unicentric CD (UCD), with involved lymph node(s) affecting a single station, and multicentric CD (MCD), with multiple lymph nodes and frequent inflammatory systemic symptoms [1]. The UCD phenotype is the most frequently reported in children and has the most favourable outcome [2].…”

International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease

“…Table 1 summarizes published data and data from the ongoing ACCEL-ERATE natural history registry 32 from the CDCN and University of Pennsylvania. 12,19,[33][34][35][36][37][38][39][40][41][42][43][44][45] The consensus focused on 2 main topics: treatment of resectable UCD and treatment of unresectable UCD. Levels of evidence were modeled after National Comprehensive Cancer Network guidelines.…”