Castleman's Disease In Children Research Articles

Clinical features and treatment outcomes of Castleman disease in children: a retrospective cohort in China

Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children’s Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression.Conclusions: Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes.What is Known:• Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics.• The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics.What is New:• We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease.• Our work may improve the recognition and optimize the management of this rare disease in children.

Castleman Disease In Children: Diagnosis and Treatment

Background: We describe the experiences in diagnosis and results of treatment in pediatric Castleman disease. Method: Serial case reports. Result: From 2016 to 2019, we had 7 cases of pediatric Castleman disease: 3 boys and 4 girl. The median age at diagnosis was 147 months (121-173 months). Clinical manifestations were found in five cases. They were all unicentric Castleman disease (6 abdominal mass, one left infraclavicular mass). All patients were operated with postoperative period uneventful. The median time of postoperative follow up was 22.7 months (11-53 months) with no signs of relapse. Conclusions: Pediatric Castleman disease is a rare benign lymph node hyperplasia, it can be localised or disseminated. Operation is the treatment of choice for localised Castleman disease.

Castleman Disease in Children from Histopathology to Therapy

Background: Castleman disease (CD) describes a group of rare lymphoproliferative disorder with characteristic histopathology. It presents with heterogeneous clinical features whether unicentric (UC) or multicentric disease (MCD). The aim of the work was to describe clinic-pathological characteristics, management and outcome of different types of castleman disease in CCHE. It is a retrospective study, all children with (CD) treated from July 2007 till end of 2017 were analyzed as regard diagnosis, management and outcome. The results showed that, twelve patients with a median age of 11.5 years (ranging from 4 - to 17 years) were enrolled. Eight of them (66.6%) were males and 4 (33.3%) were females (33.3%). Histopathology was either hyaline vascular in 8 patients (66.6%) or plasma cell variant in 4 patients (33.3%). Nine patients (75%) had uni-centric disease (UC) with lymphadenopathy. Three patients (25%) were multi-centric disease (MCD); out of them one patient had immune bi-cytopenia, small intestinal thickening and splenomegaly and another one had extensive pulmonary involvement and respiratory distress, while the third patient had pleural effusion and ascites. HIV antibody was negative for all patients. Regarding the three MCD patients, IL-6 was normal in one patient, and extremely high in second one, while it hasn’t been done for the third patient. All UC patients underwent surgical excision and are alive with no active disease till now. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) as salvage therapy, except for one patient with pulmonary involvement who didn’t achieved clinical response and received single agent Rituximab with Anti IL-6. Unfortunately this patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. In conclusion, Uni-centric castleman disease is a localized surgically cured disease. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory.

Clinical and experimental study of Castleman disease in children.

Castleman disease (CD) is a rare lymphoproliferative disease that is often underdiagnosed or misdiagnosed, especially in children. For this reason, we describe the clinical manifestations, diagnosis and treatment of CD in 11 children. A retrospective study was performed to analyze the clinical features of 11 children with CD in a single institution from January 2001 to December 2012. All had computed tomography (CT) and lymph node resection for pathology diagnosis. The average age of patients was 9.67 ± 4.26 years (range 1.3-15.5 years) including eight males (72.73%) and three females (27.27%). All but two (18.18%) had multicentric Castleman disease (MCD). Human immunodeficiency virus (HIV) or human herpes virus 8 (HHV8) infected cells were not detected in all patients. All patients were misdiagnosed in outside hospitals without tissue examination. Only in one case, the preoperative CT scan suggested CD. After treatment, 10 out of 11 children with CD in our study were disease free in the follow-up period ranging from 12 to 136 months (average 65.1 ± 10.21 months). CD in children is rare, and is frequently misdiagnosed clinically. Our study shows that surgical resection is very effective in the treatment of unicentric Castleman disease (UCD). The rare UCD patient and all MCD patients treated with the modified NHLBFM-90 protocol had good prognosis.

Sustained Remission of Multicentric Castleman Disease in Children Treated with Tocilizumab, an Anti-Interleukin-6 Receptor Antibody

Multicentric Castleman Disease (MCD) is an idiopathic lymphoproliferative disorder, reported exceptionally in children and generally believed to be an autoinflammatory disease resulting in an increase of interleukin-6 secretion. Previous studies in adult patients suggested a beneficial role of the anti-interleukin-6 receptor antibody tocilizumab on the clinical and biologic disease manifestations of MCD. Here, we describe the efficacy and safety of tocilizumab in two children with MCD, which was diagnosed on the basis of clinical and histologic findings. In both cases, tocilizumab was administered intravenously at a dose of 8 mg/kg every 2 weeks. The tocilizumab treatment alleviated fever and restored growth rate in both patients. The patients' hypergammaglobulinemia, high C-reactive protein, and high erythrocyte sedimentation rates normalized simultaneously. Nevertheless, splenomegaly persisted in the first patient, and a secondary hepatic node appeared in the second patient. The side effects, essentially sustained thrombocytopenia, were mild in both cases. For the first patient, following an initial 10-month period, the interval between infusions was increased. This patient benefited from sustained remission for a period of 3 years. Tocilizumab was effective and safe in these two children with MCD.

Sustained remission of Multicentric Castleman Disease in children treated with tocilizumab

Background Multicentric Castleman Disease (MCD) is an idiopathic lymphoproliferative disorder, exceptionally reported in children, probably due to an increase of interleukin 6 secretion. MCD is characterized by systemic lymphadenopathy and constitutional inflammatory symptoms including severe growth retardation. Previous studies in adult showed that anti-interleukin 6 receptor antibody alleviated symptoms and biochemical abnormalities of MCD.

Cervical castleman disease in children: a case report

Cervical castleman disease in children: a case report

Cervical Castleman disease in children

We report a new case of cervical Castleman disease (hyaline vascular type) in a 6-year-old child. The mass was shown on computed tomography and magnetic resonance angiography (MRA) to be compressing the bifurcation of the right common carotid artery. At operation four masses were found, which were tightly adherent to both the artery and the right jugular vein. These were removed by blunt dissection and there were no signs of recurrence after 6 months. This is a rare tumour (we know of only 24 other reports), which is usually asymptomatic, and of uncertain prognosis.