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.
• Large study describing the time frames of fracture healing in young infants. • Features of fracture healing develop in a logical progression. • Evidence provided for determining fractures are consistent with a proposed time frame. • It is of critical importance to have sound evidence for the dating of fractures.
Children with Beckwith-Wiedemann syndrome (BWS) have increased risk for development of embryonal tumors. We present the case of an infant with BWS who has hypomethylation of LIT1 gene in the 11p15.5 chromosomal region and at 6 months of age presented with simultaneous occurrence of neuroblastoma arising from the left adrenal gland and a right adrenocortical tumor. She underwent surgical resection of both tumors and remains tumor free 18 months after surgery.
An 11-year-old girl presented with a 2-month history of progressively worsening cough, daily fevers, and weight loss. A chest radiograph revealed multiple cystic cavitary lung lesions. An extensive infectious work-up was negative. Chest CT verified multiple cavitary lung lesions bilaterally, and [F-18]2-fluoro-2-deoxy-D-glucose ((18)F-FDG) positron emission tomography with CT (PET/CT) showed increased uptake in the lung lesions as well as regional lymph nodes. Subsequent biopsy of an involved lymph node confirmed classical Hodgkin lymphoma, nodular sclerosis type. This case represents an unusual presentation for a child with Hodgkin lymphoma and demonstrates a role for (18)F-FDG PET/CT in evaluating a child with cavitary lung lesions.
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