Foreign Body Reaction Following Use of a Novel Bone Graft Substitute in Pediatric Cranioplasty.

ABSTRACT: Pediatric cranioplasty is indicated to repair skull defects with a wide variety of etiologies. The choice of graft material used to fill the defect is of paramount importance to the long-term success of this procedure. A variety of synthetic products have been commercially developed to avoid donor site morbidity. Here, the authors present the case of a 13-year-old boy with cranial Langerhans cell histiocytosis who underwent cranioplasty with a novel, calcium phosphate-based bone graft substitute (Montage). The patient presented 2 years postoperatively with a foreign body giant cell reaction that required explantation of the graft. The authors discuss potential considerations in choosing the most appropriate graft, potential contributors to this late adverse outcome, and the need for further research into the use of novel allograft materials in pediatric cranioplasty.

Molecular genetic diagnosis of pediatric cancer: current and emerging methods.

A wide array of diagnostic tests are available to evaluate molecular abnormalities in pediatric cancer. Classic cytogenetics, FISH, flow cytometry, PCR, and Southern blot analysis are in widespread use throughout pediatric hospitals. Examples of the application of these methods in pediatric cancer diagnosis are reviewed. Newer methods such as CGH, SKY, gene expression microarrays and proteomic methods are under active investigation andwill almost certainly lead to significant advances in our ability to diagnose and treat pediatric cancer.

Complete surgical resection is curative for children with hepatoblastoma with pure fetal histology: a report from the Children's Oncology Group.

PURPOSE: Children with pure fetal histology (PFH) hepatoblastoma treated with complete surgical resection and minimal adjuvant therapy have been shown to have excellent outcomes when compared with other patients with hepatoblastoma. We prospectively studied the safety and efficacy of reducing therapy in all children with stage I PFH enrolled onto two consecutive studies. PATIENTS AND METHODS: From August 1989 to December 1992, 9 children with stage I PFH were treated on the Intergroup Hepatoblastoma study INT-0098 and were nonrandomly assigned to receive chemotherapy after surgical resection with single-agent bolus doxorubicin for 3 consecutive days. From March 1999 to November 2006, 16 children with stage I PFH enrolled onto Children's Oncology Group Study P9645 were treated with observation after resection. Central confirmation of the histologic diagnosis by a study group pathologist was mandated. The extent of liver disease was assigned retrospectively according to the pretreatment extent of disease (PRETEXT) system and is designated "retro-PRETEXT" to clarify the retrospective group assignment. RESULTS: Five-year event-free and overall survival for the 9 patients treated on INT-0098 were 100%. All 16 patients enrolled onto the P9645 study were alive and free of disease at the time of last contact, with a median follow-up of 4.9 years. Retro-PRETEXT for the 21 patients with available data revealed seven patients with stage I disease, 10 patients with stage II disease, and four patients with stage III disease. CONCLUSION: Children with completely resected PFH hepatoblastoma can achieve long-term survival without additional chemotherapy. When feasible, surgical resection of hepatoblastoma at diagnosis, without chemotherapy, can identify children for whom no additional therapy is necessary.

Ewing sarcoma as a second malignant neoplasm after acute lymphoblastic leukemia.

Second malignant neoplasms (SMNs) are being increasingly recognized. This report describes a case of a 7-year-old girl with a history of acute lymphoblastic leukemia (ALL) who presented with a mass in her humerus that was diagnosed as Ewing sarcoma. Second malignant neoplasms are relatively rare in survivors of ALL treated without radiation. Even more unusual is the development of Ewing sarcoma as the SMN.

Hepatoblastoma: assessment of criteria for histologic classification.

BACKGROUND: Comparison of outcomes in different clinicopathologic studies of hepatoblastoma requires reproducible histologic classification. This review examines the diagnostic criteria employed by different pathologists for the classification of subtypes of hepatoblastoma and identifies specific problem areas. PROCEDURE: A selected review of published literature is provided. RESULTS: Published studies demonstrate that uniform criteria have not been applied in the classification of hepatoblastoma. These discrepancies hinder attempts to compare outcome data from different studies. Sampling error and potential treatment effects further complicate analysis of the published literature on the relationship between morphologic classification and outcome. CONCLUSIONS: Standardized criteria are essential to allow reproducible histologic classification of hepatoblastoma. There is significant variation in diagnostic criteria used to define the major subtypes of hepatoblastoma in published studies. Additional potential problems are identified in sampling methods and treatment effects.

Blastic NK-cell-like lymphoma with T-cell receptor gene rearrangement.

Blastic natural killer (NK) cell lymphoma is very rare but has been recently classified as a distinct entity in WHO classification. However, the classification remains controversial, and the clinicopathologic spectrum is not completely understood. We report a unique case of cutaneous CD4(+) CD56(+) malignancy with a typical clinical presentation and immunophenotype of blastic NK-cell lymphoma in a 15-year-old Guamanian girl. The skin was the only site involved by the lymphoma. Molecular study showed clonal T-cell receptor gamma gene rearrangement. The patient has been disease-free till now (more than 12 months following bone marrow transplant). This case may represent a tumor at an early stage of a common developmental pathway for T-cells and NK-cells.

Clinical findings and lung pathology in children with cystic fibrosis.

Cystic fibrosis pulmonary disease is assessed by pulmonary function tests, arterial blood gases, and chest X-rays, but the correlation with lung pathology is unknown. We reviewed the clinical findings and lung pathology of 21 cystic fibrosis patients who had lung transplant. Pulmonary function tests, Brasfield scores, arterial blood gases, and age were correlated with lung pathology. All patients had severe Brasfield scores (9.0 +/- 3.2), airways obstruction (FEV1 25.6 +/- 5.6% predicted, FEF(25-75%) 11.0 +/- 4.5% predicted), and hyperinflation (residual volume [RV] 341.8 +/- 75.8% predicted). All patients were hypoxemic (PO2 64.2 +/- 8.2 mm Hg), and 5 of 21 (24%) were hypercapneic (PCO2 > 50 mm Hg). Pulmonary function tests and Brasfield scores were within a narrow range, and did not allow correlation with lung pathology. Small airway density (airways < 2 mm/cm2) decreased with increasing age. There were no differences in small airways inflammation and fibrous narrowing between the hypercapneic and nonhypercapneic patients, but the percent of smallest airways (airways < 0.35 mm) was significantly lower in the hypercapneic group. We conclude that there is significant correlation between airway pathology and increased age and CO2 retention. We speculate that decreased small airway density in older patients and the decreased proportion of smallest airways in hypercapneic patients is caused by increased dilatation of small airways.