Severe Neutropenia at the Time of Implantable Subcutaneous Chest Port Insertion Is Not a Risk Factor for Port Removal at a Tertiary Pediatric Center.

PURPOSE: To determine if severe neutropenia at the time of chest port insertion is a risk factor for port removal and central catheter-associated bloodstream infection (CCABSI) in pediatric patients. MATERIALS AND METHODS: From May 2007 to June 2015, 183 consecutive patients (mean age, 9.9 y; range, 0.75-21 y) had a port inserted at a single tertiary pediatric center. Seventy-two had severe neutropenia at the time of port insertion (absolute neutrophil count [ANC] range, 0-500/mm3; mean, 185/mm3). Follow-up until port removal or death and CCABSI events were recorded. RESULTS: Within the first 30 days, similar incidences of CCABSI (12.5% of patients with severe neutropenia [n = 9] vs 4.5% of patients without [n = 5]), port removal for infection (2.8% [n = 2] vs 2.7% [n = 3]), and local port infection (2.8% [n = 2] vs 0.9% [n = 1]) were observed in both groups (P > .05), but the rate of CCABSI per 1,000 catheter-days was higher for patients with severe neutropenia (P = .045). Overall, similar incidences of CCABSI (18.1% [n = 13] vs 16.2% [n = 18]), port removal for infection (2.8% [n = 2] vs 7.2% [n = 8]), local port infection (2.8% [n = 2] vs 2.7% [n = 3]), and CCABSIs per 1,000 catheter-days (0.332 vs 0.400) were observed in both groups (P > .05). CONCLUSIONS: Port placement in patients with severe neutropenia can be performed without an increased incidence of port removal for infection. The majority of CCABSIs were successfully treated without port removal.

Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: Final report of the Head Start II and III trials.

PURPOSE: To report the final analysis of survival outcomes for children with newly diagnosed high-grade glioma (HGG) treated on the "Head Start" (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old. PATIENTS AND METHODS: Between 1997 and 2009, 32 eligible children were enrolled in HS II and III with anaplastic astrocytoma (AA, n = 19), glioblastoma multiforme (GBM, n = 11), or other HGG (n = 2). Central pathology review was completed on 78% of patients. Patients with predominantly brainstem tumors were excluded. Patients were to be treated with single induction chemotherapy regimen C, comprising four cycles of vincristine, carboplatin, and temozolomide. Following induction, patients underwent marrow-ablative chemotherapy and autologous hematopoietic cell rescue. Irradiation was used for patients with residual tumor after consolidation or >6 years old or at the time of tumor progression. RESULTS: The 5-year event-free survival (EFS) and overall survival (OS) for all HGG patients were 25 ± 8% and 36 ± 9%, respectively. The EFS at 5 years for patients with AA and GBM were 24 ± 11% and 30 ± 16%, respectively (P = 0.65). The OS at 5 years for patients with AA and GBM was 34 ± 12% and 35 ± 16%, respectively (P = 0.83). Children <36 months old experienced improved 5-year EFS and OS of 44 ± 17% and 63 ± 17%, compared with children 36-71 months old (31 ± 13% and 38 ± 14%) and children >72 months old (0% and 13 ± 12%). CONCLUSIONS: Irradiation-avoiding treatment strategies should be evaluated further in young children with HGG given similar survival rates to older children receiving standard irradiation-containing therapies.

Subsequent development of diffuse large B-cell lymphomas and Hodgkin lymphoma associated with primary immune disorder in a 6-year-old female: a case report and review of the literature.

Neoplastic lymphoid proliferation may arise from immune deficiency or disordered regulation of the immune system. Often the neoplasms are associated with viral agents, such as Epstein-Barr virus, human immunodeficiency virus, or human herpes virus 8. Lymphoproliferative diseases have been documented in a variety of primary immune disorders. The most commonly encountered neoplastic lesion is diffuse large B-cell lymphoma (DLBCL), although Hodgkin lymphoma (HL), Burkitt lymphoma, and peripheral T-cell lymphomas and/or leukemias have also been documented in rare instances. We report a case of a 6-year-old girl with unclassifiable primary immunodeficiency diagnosed with 2 different clones of DLBCLs and subsequently developed lymphocyte-depleted, classical HL. Both neoplasms were associated with Epstein-Barr virus. To the best of our knowledge, this is the first reported occurrence of primary immune disorder-associated lymphoproliferative disease with sequential development of DLBCLs and HL in a pediatric patient. Thorough surveillance is paramount for accurate assessment of the associated lymphoproliferative disease and in ascertaining likely transformation to, or de novo evolution of a different lymphoid neoplasm. This is also important in evaluating treatment response with appropriate therapeutic adjustments if clinically indicated.

Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection.

Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies. The first patient also had a concurrent cerebellar tumor (pilocytic astrocytoma), for which he first underwent craniotomy and resection, delaying the liver tumor resection by 10 weeks. They are alive and tumor free at 48 months (case no. 1) and 18 months (case no. 2) following neoadjuvant chemotherapy and liver resection.