Bone Sarcomas in Pediatrics: Progress in Our Understanding of Tumor Biology and Implications for Therapy.

The pediatric bone sarcomas osteosarcoma and Ewing sarcoma represent a tremendous challenge for the clinician. Though less common than acute lymphoblastic leukemia or brain tumors, these aggressive cancers account for a disproportionate amount of the cancer morbidity and mortality in children, and have seen few advances in survival in the past decade, despite many large, complicated, and expensive trials of various chemotherapy combinations. To improve the outcomes of children with bone sarcomas, a better understanding of the biology of these cancers is needed, together with informed use of targeted therapies that exploit the unique biology of each disease.

New, tolerable γ-secretase inhibitor takes desmoid down a notch.

A phase I trial of PF-03084014, an oral reversible γ-secretase inhibitor, in solid tumor malignancies shows drug tolerability in patients. Evidence of Notch pathway inhibition was demonstrated in peripheral blood. A surprisingly high rate of response was seen in desmoid tumors, a rare but sometimes locally aggressive sarcoma.

Understanding the role of Notch in osteosarcoma.

The Notch pathway has been described as an oncogene in osteosarcoma, but the myriad functions of all the members of this complex signaling pathway, both in malignant cells and nonmalignant components of tumors, make it more difficult to define Notch as simply an oncogene or a tumor suppressor. The cell-autonomous behaviors caused by Notch pathway manipulation may vary between cell lines but can include changes in proliferation, migration, invasiveness, oxidative stress resistance, and expression of markers associated with stemness or tumor-initiating cells. Beyond these roles, Notch signaling also plays a vital role in regulating tumor angiogenesis and vasculogenesis, which are vital aspects of osteosarcoma growth and behavior in vivo.

Evaluation of the psychometric properties of the Pediatric Parenting Stress Inventory (PPSI).

Objective: This work evaluated the psychometric properties of the Pediatric Parenting Stress Inventory (PPSI), a new measure of problems and distress experienced by parents of children with chronic illnesses.

Method: This secondary data analysis used baseline data from 1 sample of English-, Spanish-, and Hebrew-speaking mothers of children recently diagnosed with cancer (n = 449) and 1 sample of English- and Spanish-speaking mothers of children recently diagnosed with cancer (n = 399) who participated in 2 problem-solving skills training interventions. The PPSI was administered at baseline with other measures of maternal distress.

Strategies for the targeted delivery of therapeutics for osteosarcoma.

Background: Conventional therapy for osteosarcoma has reached a plateau of 60 - 70%, a 5-year survival rate that has changed little in two decades, highlighting the need for new approaches.

Objective: To review the alternative means of delivering effective therapy for osteosarcoma that reach beyond the central venous catheter.

Methods: Drawing on the author's own experiences providing care to high-risk osteosarcoma patients and reviewing the last two decades of literature describing sarcoma therapy, available information is summarized about potential osteosarcoma treatments that deliver therapy by a less conventional route.

Primary tuberculosis of bone mimicking a lytic bone tumor.

Causes of lytic bone lesions in children include benign, malignant, and infectious processes. Here, we present the case of a 3-year-old boy presenting with a lytic bone lesion and surrounding soft tissue mass sent for evaluation of possible malignancy versus osteomyelitis. Biopsy revealed granulomatous osteomyelitis, and subsequent purified protein derivative resulted in 20-mm induration.