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Editorial
Eliminating Cells Gone Astray
The therapeutic use of cells from healthy donors or patients is increasing. Decades ago, transfusion medicine and bone marrow transplantation provided the first successful cell therapeutics and established the foundations for cell delivery. Clinical investigation soon uncovered the double-edged…
Original Article
Inducible Apoptosis as a Safety Switch for Adoptive Cell Therapy
Although cellular therapies may be effective in cancer treatment, their potential for expansion, damage of normal organs,– and malignant transformation is a source of concern. In contrast, the toxic effects of small molecules usually diminish once the drugs are withdrawn. One approach to…
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Review Article
Current Concepts: The Tumor Lysis Syndrome
The tumor lysis syndrome is the most common disease-related emergency encountered by physicians caring for children or adults with hematologic cancers.– Although it develops most often in patients with non-Hodgkin's lymphoma or acute leukemia, its frequency is increasing among patients who have…
- CME
Case Records of the Massachusetts General Hospital
Case 38-2010 — A 13-Year-Old Girl with an Enlarging Neck Mass
Presentation of Case. Dr. Michelle L. Katz (Pediatrics): A 13-year-old girl was seen in the pediatric endocrinology clinic of this hospital because of an enlarging neck mass. Eight days earlier, the patient noted swelling of her neck and pain on swallowing. Two days later, her primary care provider…
- CME
Original Article
Outcome after Reduced Chemotherapy for Intermediate-Risk Neuroblastoma
Neuroblastoma is the most common extracranial solid tumor in childhood, accounting for 50% of neoplasms diagnosed in the first year of life. This disease has a heterogeneous course, ranging from spontaneous regression to inexorable progression and death, depending on the biologic features of the…
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Original Article
Anti-GD2 Antibody with GM-CSF, Interleukin-2, and Isotretinoin for Neuroblastoma
Neuroblastoma, a cancer of the sympathetic nervous system responsible for 12% of deaths associated with cancer in children under 15 years of age, is a heterogeneous disease, with nearly 50% of patients having a high-risk phenotype characterized by widespread dissemination of the cancer and poor…
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Review Article
Medical Progress: Recent Advances in Neuroblastoma
Neuroblastoma is an embryonal tumor of the autonomic nervous system, meaning that the cell of origin is thought to be a developing and incompletely committed precursor cell derived from neural-crest tissues. As may be expected with a disease of developing tissues, neuroblastomas generally occur in…
- CME
Neuroblastoma, an embryonal cancer of the autonomic nervous system, is the most common cancer diagnosed during the first year of life. Although neuroblastoma accounts for disproportionately high morbidity and mortality among childhood cancers, it has one of the highest rates of spontaneous and complete regression. The author discusses recent advances in our understanding of neuroblastoma.
Case Records of the Massachusetts General Hospital
Case 13-2010 — An 18.5-Month-Old Girl with Watery Diarrhea and Poor Weight Gain
Presentation of Case. Dr. Sara V. Bates (Pediatric Service): An 18.5-month-old girl was seen in the pediatric gastroenterology clinic of this hospital because of watery diarrhea and poor weight gain. The patient was born to a multigravida mother after a full-term gestation. She was breast-fed for…
An 18.5-month-old girl was seen in the pediatric gastroenterology clinic of this hospital because of watery diarrhea and poor weight gain. The child had been healthy until 12 months of age, when chronic watery diarrhea developed. Six months later, her weight had decreased to the fourth percentile for her age. Extensive studies of stool, ultrasonography of the abdomen, and upper and lower endoscopic examinations were normal. A sweat test was interpreted as borderline. A diagnostic test result was received.
Original Article
Cisplatin versus Cisplatin plus Doxorubicin for Standard-Risk Hepatoblastoma
Between 1990 and 1994, the International Childhood Liver Tumour Strategy Group (SIOPEL) conducted its first cooperative trial (SIOPEL 1), which set the standard of care for hepatoblastoma in most European countries. With a 5-year event-free survival of 66% and an overall survival of 75%, the trial…
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In this randomized trial involving children with standard-risk hepatoblastoma, treatment with cisplatin alone caused fewer adverse events and achieved rates of complete resection and survival that were similar to rates achieved with treatment with cisplatin plus doxorubicin. These findings suggest that doxorubicin is not needed in treating children with hepatoblastoma who are not at high risk for a poor outcome.
Original Article
Treating Childhood Acute Lymphoblastic Leukemia without Cranial Irradiation
Clinical trials have yielded 5-year event-free survival rates as high as 79 to 82% among children with acute lymphoblastic leukemia (ALL).– A major challenge is to reduce treatment-related late effects, which can occur in more than two thirds of long-term survivors. In a growing proportion of…
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This large trial of acute lymphoblastic leukemia in children aimed to determine whether intensive systemic and intrathecal chemotherapy could obviate the need for prophylactic central nervous system irradiation, which is associated with distressing late complications. The results show that, with meticulous monitoring of responses and toxic effects, it is possible not only to avoid cranial irradiation but also to improve the overall outcome.
Correspondence
IDH1 and IDH2 Mutations in Gliomas
To the Editor: Yan et al. (Feb. 19 issue) found that mutations of genes encoding isocitrate dehydrogenases (IDH1 and IDH2), as compared with no mutations, are associated with younger age and better prognosis in adults with gliomas. Their study and other, similar studies– prompted us to search for…
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Special Article
Health and Economic Implications of HPV Vaccination in the United States
In the United States, cervical cancer developed in an estimated 11,150 women and caused death in 3600 women in 2007. Infection with high-risk "oncogenic" types of human papillomavirus (HPV) is the cause of 100% of cervical cancers, 90% of anal cancers, 40% of vulvar and vaginal cancers, at least…
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The authors conducted a cost-effectiveness analysis of human papillomavirus (HPV) vaccination in the United States and estimated that vaccinating 12-year-old girls would cost $43,600 per quality-adjusted life-year (QALY) gained, as compared with the current screening practice. If vaccine-induced immunity wanes after 10 years, HPV vaccination would be much less cost-effective (>$140,000 per QALY).
Correspondence
Multiple Tumors in a Child with Germ-Line Mutations in TP53 and PTEN
To the Editor: TP53, a tumor-suppressor gene, is frequently inactivated by somatic mutations in cancer. Inheritance of a heterozygous TP53 mutation results in the Li–Fraumeni syndrome of a hereditary predisposition to cancer. A germ-line mutation of the PTEN gene is associated with Cowden's…
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Case Records of the Massachusetts General Hospital
Case 10-2008 — A 10-Year-Old Girl with Dyspnea on Exertion
Presentation of Case. A 10-year-old girl was seen in the multidisciplinary Airway, Voice, and Swallowing Center for Children at the Massachusetts Eye and Ear Infirmary, associated with this hospital, because of dyspnea and noisy respirations. Approximately 3 weeks earlier, a sharp pain in her chest…
A 10-year-old girl was seen because of dyspnea and noisy respirations. Three weeks earlier, sharp pain in the chest and shortness of breath had developed abruptly, followed by dyspnea and noisy respirations with exertion. Symptoms did not improve with the use of bronchodilators. A diagnostic procedure was performed.
Review Article
Current Concepts: Computed Tomography — An Increasing Source of Radiation Exposure
The advent of computed tomography (CT) has revolutionized diagnostic radiology. Since the inception of CT in the 1970s, its use has increased rapidly. It is estimated that more than 62 million CT scans per year are currently obtained in the United States, including at least 4 million for children.…
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The number of computed tomographic (CT) studies performed is increasing rapidly. Because CT scans involve much higher doses of radiation than plain films, we are seeing a marked increase in radiation exposure in the general population. Epidemiologic studies indicate that the radiation dose from even two or three CT scans results in a detectable increase in the risk of cancer, especially in children. This article summarizes the facts about this form of radiation exposure and the implications for public health.
Case Records of the Massachusetts General Hospital
Case 20-2007 — An 11-Year-Old Boy with a Calcified Mass in the Nose
Presentation of Case. Dr. Margo McKenna Benoit (Otolaryngology): An 11-year-old boy was referred to a pediatric otolaryngologist at the Massachusetts Eye and Ear Infirmary because of headaches and a right nasal mass. Bifrontal headaches had started approximately 5 years earlier; in recent months,…
An 11-year-old boy was seen because of a calcified mass in the ethmoid sinus and nose. He had a 5-year history of nasal congestion and headaches; 2 months before presentation, physical examination disclosed a mass in the right naris, and imaging studies showed a calcified mass that appeared to arise in the ethmoid sinus.
Editorial
HPV Vaccination — More Answers, More Questions
The availability of a "cancer vaccine" has elicited enormous enthusiasm from the medical community and the public, culminating in advocacy for mandatory vaccination against human papillomavirus (HPV) and a recommendation from the Centers for Disease Control and Prevention (CDC) that 30 million…
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Perspective
Politics, Parents, and Prophylaxis — Mandating HPV Vaccination in the United States
Cancer prevention has fallen victim to the culture wars. Throughout the United States, state legislatures are scrambling to respond to the availability of Merck's human papillomavirus (HPV) vaccine, Gardasil, and to the likely introduction of GlaxoSmithKline's not-yet-approved HPV vaccine, Cervarix,…
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Original Article
Age at Surgery for Undescended Testis and Risk of Testicular Cancer
Undescended testis, or cryptorchidism, which occurs in 2 to 5% of boys born at term, is one of the most common congenital abnormalities. Cryptorchidism is associated with impaired fertility and is a risk factor for testicular cancer. Among men who have had undescended testis, the risk of cancer is…
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In a study of almost 17,000 men who were surgically treated for undescended testis, with data culled from Swedish national registries, the risk of testicular cancer among men who underwent orchiopexy at 13 years of age or older was twice that among men who had surgery before the age of 13. Surgical treatment for undescended testis at an early age can prevent testicular cancer.
