Adolescent and young adult oncology, version 2.2014.

The NCCN Guidelines Insights on Adolescent and Young Adult (AYA) Oncology discuss the fertility and endocrine issues that are relevant to the management of AYA patients with cancer. Fertility preservation should be an essential part in the treatment of AYA patients with cancer. The NCCN Guidelines recommend discussion of fertility preservation and contraception before the start of treatment. Oophoropexy and embryo cryopreservation are the 2 established options for fertility preservation in women. Semen cryopreservation before the start of treatment is the most reliable and well-established method of preserving fertility in men. AYA women with cancer also have unique contraception needs, depending on the type of cancer, its treatment, and treatment-related complications. Management of cancer during pregnancy poses significant diagnostic and therapeutic challenges for both the patient and the physician. AYA women diagnosed with cancer during pregnancy require individualized treatment from a multidisciplinary team involving medical, surgical, radiation, and gynecologic oncologists; obstetricians; and perinatologists.

Adolescent and young adult oncology. Clinical practice guidelines in oncology.

Cancer is the leading cause of death among the adolescent and young adult (AYA) population, excluding homicide, suicide, or unintentional injury. AYA patients should be managed by a multidisciplinary team of health care professionals who are well-versed in the specific developmental issues relevant to this patient population. The recommendations for age-appropriate care outlined in these NCCN Guidelines include psychosocial assessment, a discussion of infertility risks associated with treatment and options for fertility preservation, genetic and familial risk assessment for all patients after diagnosis, screening and monitoring of late effects in AYA cancer survivors after successful completion of therapy, and palliative care and end-of-life considerations for patients for whom curative therapy fails.

Severe thrombocytopenia with iron deficiency anemia.

Although severe thrombocytopenia has been reported among children with iron deficiency anemia, the presence of both anemia and thrombocytopenia can suggest serious or malignant bone marrow dysfunction, requiring further work-up. This paper reviews the management and outcomes of patients with the diagnosis of iron deficiency anemia presenting with severe thrombocytopenia. The authors present 4 pediatric patients with iron deficiency anemia and severe thrombocytopenia at initial presentation. Charts were reviewed after approval by the institutional review boards at both St. Jude Children's Research Hospital and LeBonheur Children's Medical Center in Memphis, Tennessee. All 4 patients' hemoglobin concentrations and platelet counts normalized within 1 to 3 weeks of initiating iron supplementation, suggesting that in such patients iron supplementation can obviate the need for invasive diagnostic procedures. In addition, these patients all had a platelet "overshoot" phenomenon during iron therapy prior to normalization of platelet counts, the mechanism of which is still unknown. The literature exploring the mechanism behind the current clinical findings is reviewed.

Conservative management of pancreatic pseudocysts in children with acute lymphoblastic leukemia.

Treatment with asparaginase for acute lymphoblastic leukemia can cause acute pancreatitis. Complication of pancreatitis by pancreatic pseudocyst formation can prolong the hospital stay, delay chemotherapy, and necessitate long-term parenteral nutrition. We report 5 children with acute lymphoblastic leukemia who developed acute pancreatitis complicated by pancreatic pseudocysts. They required modifications to their chemotherapy regimen and prolonged parenteral nutrition but no surgical intervention. All 5 patients survive in first remission and their pseudocysts resolved after 3 to 37 months or continued to decrease in size at last follow-up. These cases illustrate that nonsurgical management of pancreatic pseudocyst is safe, though pseudocyst resolution may require many months. In addition, these patients demonstrate that oral feeding can be initiated after the acute episode of pancreatitis resolves even if a pseudocyst is present.

An unusual case of Ogilvie syndrome in a pediatric oncology patient receiving palliative care after failed treatment with neostigmine.

BACKGROUND: We present a case of severe refractory constipation in an adolescent oncology patient with widely metastatic clear cell osteosarcoma who was ultimately found to have Ogilvie syndrome, also known as acute colonic pseudo-obstruction (ACPO). Ogilvie syndrome is characterized by dilatation of the large intestine in the absence of mechanical obstruction, usually occurring in adult patients with serious underlying medical conditions and rarely seen in children. It is likely that chronic narcotic use, abdominal metastasis, and a paraneoplastic process contributed to development of ACPO in this patient. DISCUSSION: This case highlights an infrequent, but important, gastrointestinal complication that can occur in pediatric patients with serious comorbid disease. Ogilvie syndrome should be included on the differential diagnosis list in pediatric patients with refractory constipation. Our case illustrates the progression of therapies, including surgical intervention that can be undertaken to treat this disorder in children and adults while providing important considerations for clinicians treating patients of any age with this unusual clinical complication.

The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm.

Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other "hits." These possibilities and the evidence base to support them are explored.