Paola Coccia

Anthracycline cardiotoxicity in childhood

Over the last 40 years, a significant advance has been made in the treatment of childhood and adult cancers. However, the increase of the survival rate points out medium-and long-term adverse effects that constitute a serious limitation for the quality of life in adults survived from a childhood cancer. Cardiovascular disease is an important cause of morbidity and mortality in adults treated with chemo-and radiotherapy for childhood cancers. Although some antitumor treatments are potentially cardiotoxic, anthracycline therapy and radiotherapy are mostly responsable for long-term cardiac damage. Anthracycline toxicity is generally limited to the myocardium, while radiation can cause injury to all components of the heart. The purpose of this review is to discuss the mechanisms of action of anthracyclines, their cardiotoxicity, the feasibility of screening, and the prevention of cardiac damage after treatment in childhood.

Efficacy and safety of transdermal buprenorphine in the management of children with cancer-related pain

The current study investigated the efficacy, safety, tolerability, and compliance of a transdermal buprenorphine delivery system for the management of chronic cancer pain in the pediatric population.

The role of diet on the clinical pharmacology of oral antineoplastic agents

PurposeThe number of oral anticancer agents has greatly increased in recent years. It is a well-known fact that food intake can induce significant variations in the bioavailability of these drugs. The aim of this review is to describe the interactions between diet and oral anticancer drugs in terms of the possible effects of such interactions on reducing the antineoplastic activity of the drug or increasing its side effects.MethodsThis was an analytical study of the numerous mechanisms leading to changes in the bioavailability of oral antineoplastic agents due to diet.ResultsFood–drug interactions can induce a delay, decrease or increase in the absorption of the oral chemotherapeutic agent. The concomitant intake of food and antineoplastic drugs influence the pharmacokinetic and pharmacodynamic drug processes depending on the composition of the food consumed and the specific interactions of the food with transport mechanisms (p-glycoprotein, multidrug resistance proteins) and intestinal enzymatic systems (cytochrome P450).ConclusionsIn prescribing an oral anticancer agent, clinicians must consider the possibility that the consumption of specific food items has the potential to interfere with the pharmacokinetics and pharmacodynamics of the prescribed drug.

MRI findings in Shwachman diamond syndrome

Shwachman Diamond Syndrome (SDS) is a rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction, and skeletal changes. Because of the heterogeneous clinical presentation and the limits of laboratory tests that assess pancreatic insufficiency, the diagnosis of SDS can be challenging. Pancreatic lipomatosis, a typical feature of this syndrome, is also difficult to assess by direct tissue sampling. In these circumstances, magnetic resonance imaging (MRI) provides a readily available, noninvasive tool to evaluate the pancreatic fat content. We report a case of a 12‐month‐old male in which abdominal MRI was used to confirm the clinical diagnosis of SDS. Pediatr Blood Cancer 2008;50:352–354.

Cardiac metastases of Ewing sarcoma detected by 18F-FDG PET/CT.

Positron emission tomography (PET) is widely used in the diagnostic evaluation and staging of different malignant tumors. The role of PET/computed tomographic scan in detecting distant metastases in the workup of Ewing sarcoma in children or young adults is less well defined. We report a case of a boy affected by a metastatic Ewing sarcoma with cardiac asymptomatic metastasis detected by 18F-FDG PET/computed tomography.

How to manage vaccinations in children with cancer.

The optimal use of routine childhood immunizations in children with malignancy is still a matter of debate. Despite their higher risk of contracting vaccine preventable diseases and of suffering important complications, there is little understanding of the magnitude of the possible benefit of administering active immunization in this population due to a paucity of clinical trial data. Our review focuses on the management of children with cancer and offers some suggestions regarding their vaccination schedules. Pediatr Blood Cancer 2011; 57: 1104–1108.

Two cases of pediatric high-grade astroblastoma with different clinical behavior

Astroblastoma is a rare glial tumor occurring in older children and defined by histological criteria as low or high-grade. We describe two children with high-grade astroblastoma. The first patient, with multiple recurrences of a frontoparietal tumor, died 10 years from diagnosis after progression of the disease despite surgery, radiotherapy and chemotherapy. The second patient underwent subtotal resection of a temporal mass; the residual tumor progressed five months after radiotherapy, but after a subsequent gross total resection the patient is now in complete remission 54 months from diagnosis. Although both patients had high-grade astroblastomas, there were histological differences between the two tumors, in particular regarding the proliferative index, which was 30% and 5–10%, respectively. High-grade astroblastoma is usually treated with surgery and radiotherapy, but may have an unpredictable behavior even when tumor excision is deemed complete. The two cases reported here illustrate the variable clinical course of this rare tumor. The proliferative index may be a useful tool to better define prognosis.

Treatment of pharyngeal non-Hodgkin lymphoma in a patient with Wiskott-Aldrich syndrome

Wiskott‐Aldrich syndrome (WAS) is characterized by primary immunodeficiency, thrombocytopenia and eczema. Patients with WAS have an increased risk to develop tumors. Non‐Hodgkin lymphoma (NHL) represents the most common malignancy occurring in WAS‐affected patients, diffuse‐large‐B‐cell lymphoma is the most frequently encountered variant. We describe a case of a patient with WAS and NHL in the pharynx, an atypical tumor site presentation. The patient was successfully treated with a reduced dose chemotherapy regimen plus anti‐CD20 monoclonal antibody. He is in complete remission 3 years from the start of treatment. Pediatr Blood Cancer 2012;59:318–319.

Errors Involving Patients Receiving Intrathecal Chemotherapy

Abstract Errors involving patients receiving intrathecal chemotherapy are a significant problem in oncology. Despite the improvement in the management of antineoplastic agents, unintentional intrathecal administration of chemotherapic drugs that are indicated only for systemic administration or intrathecal overdose of drugs regularly used for intrathecal chemotherapy, continue to occur. These events can result in severe neurotoxicity, usually fatal in outcome. We review reported cases of medication errors in intrathecal administration of chemotherapy described in the literature. Diverse rescue therapies have been proposed but the most effective means of managing these errors remains prevention.

Extragonadal yolk sac tumor outside of the midline of the body: a case report of a child with a yolk sac tumor of the pontocerebellar angle

Yolk sac tumor is a rare germ cell neoplasm occurring mainly in the gonads. Extragonadal yolk sac tumor is a very rare malignancy; its main distribution is along the midline of the body at three principal sites: mediastinum, central nervous system and retroperitoneum. Most yolk sac tumors are diagnosed between seven months and three years of age. We report a case of primary yolk sac tumor in a 13-month-old child. The tumor was located in the pontocerebellar angle, an atypical location that may not have suggested a yolk sac tumor as first diagnosis. We want to highlight the importance of performing tumor marker measurements during the first year of life, also for tumors located away from the midline.