Incidence and Outcome of Pericardial Effusion in Pediatric Patients After Hematopoietic Stem Cell Transplant: A Single-institution Experience.

BACKGROUND: Pericardial effusion (PE) is a known complication after hematopoietic stem cell transplant (HSCT). Limited data is currently available regarding the incidence and outcomes of PE in pediatric HSCT. METHODS: We conducted a retrospective study on a cohort of patients who underwent HSCT between 2004 and 2015. Risk factors associated with development of PE were evaluated. RESULTS: In 111 HSCT, stem cell source was bone marrow in 37 (33.3%), peripheral blood-42 (37.8%) and cord blood-32 (28.8%). Incidence of PE after HSCT was 37.8%. Insignificant effusion (trivial or small) was noted in 30 (27.0%) transplants, and significant (moderate or large) PE in 12 (10.8%). There were no associations between incidence of effusion and stem cell source, graft versus host disease or CMV infection. Risk factors associated with development of PE included systemic hypertension (P<0.05), total body irradiation (P<0.05), and sinusoidal obstruction syndrome formerly known as venoocclusive disease (P=0.03). Overall mortality was 22.5% after HSCT, but 38.1% among those with effusion (P<0.05). None of these deaths were attributed to primary cardiac etiologies. CONCLUSIONS: The incidence of PE in this cohort of pediatric HSCT recipients is high and associated with higher morbidity and mortality.

Rhabdomyosarcoma in adults: new perspectives on therapy.

OPINION STATEMENT: Rhabdomyosarcoma (RMS) is well known as a pediatric disease. Most of the knowledge, like biology, genetics, and treatments of this disease, comes from studies done in that age group. The two subtypes of RMS, embryonic RMS and alveolar RMS, that affect mainly the pediatric population are well described in the literature and that has had an impact on the improvement in overall survival during the past 20 years. RMS in the adult population has a low incidence, therefor the study of RMS in this group is challenging. Pleomorphic RMS is the subtype that mainly affects adults and its biology and genetics are not yet completely understood and described. The risk factors for this tumor and the differences among adults and children is also poorly understood. The treatments for adults that have RMS are not standardized having an impact on the overall survival. Pleomorphic RMS has, compared to other adult sarcomas, poor overall survival. Adult patients with RMS have poor prognosis. The standardization of treatments for the adult population is necessary as maybe new treatments for this specific group. There are new treatment options that are being studied mostly in pediatrics and young adults. Immunotherapy is currently proposed as an important treatment possibility including different techniques like vaccination, antigen-mediated therapy, and immune checkpoints. Even if we have a better understanding of RMS, there are still unanswered questions. The improvements seen in the pediatric population are encouraging, but there is still the need to enhance better therapies for adults with RMS.

Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.