Current Pediatric Reviews (v.11, #3)

Meet Our Editorial Board Member: by Kathleen B. Schwarz (141-141).

Diagnostic Imaging of Fetal and Neonatal Abdominal and Soft Tissue Tumors by Usha D. Nagaraj, Beth M. Kline-Fath (143-150).
Imaging plays a key role in the diagnosis and staging of prenatal and neonatal tumors, and is essential in treatment planning. Though obstetrical ultrasound is the first choice prenatally, fetal MRI continues to play an increasing role as experience with this imaging modality increases. In the neonate, in addition to ultrasound and MRI, CT and nuclear medicine studies can also play an important role. We describe the prenatal and neonatal imaging findings of some of the most common congenital abdominal and soft tissue neoplasms including neuroblastoma, renal, liver and soft tissue tumors.

Perinatal Management of Fetal Tumors by Jose L. Peiro, Lourenco Sbragia, Federico Scorletti, Foong Y. Lim (151-163).
Progress in the last three decades in prenatal genetic diagnosis and advancement in prenatal imaging and characterization of fetal anomalies have allowed better preparation in family counseling and afforded the opportunity to consider prenatal treatment for congenital defects that would have fatal outcomes, among them the fetal tumors. Advances in fetal therapy and surgical approaches including minimally invasive procedures, have permitted not just the survival of rare tumor cases but improved long-term outcomes. In this review, we described some of the most common fetal tumors and their recommended management with emphasis on in utero treatment options.

Genetic Predisposition to Neonatal Tumors by Emily Quinn, Rose McGee, Regina Nuccio, Alberto S. Pappo, Kim E. Nichols (164-178).
The development of tumors within the first month of life is extremely rare and this very young age at presentation should raise concern for an underlying cancer predisposition syndrome. This chapter discusses genetic predisposition to neonatal solid tumors, specifically those involving the eyes, kidneys, liver, adrenal glands and lungs. For each tumor type, we discuss the most common associated predisposition syndromes and for eye and lung tumors, we also review the approach to diagnosis and management. The reader is referred to the other chapters of this volume for an overview of management details for the other tumor types. The management of neonates with cancer predisposing syndromes is challenging, with many patients demonstrating multisystem involvement. Accordingly, it is critical to use a team approach, where providers with expertise in neonatology, oncology, genetics, surgery and other pediatric subspecialties can appropriately tailor management of the patient and coordinate genetic evaluation of the family.

Current Management of Neonatal Neuroblastoma by Rodrigo B. Interiano, Andrew M. Davidoff (179-187).
Neonatal neuroblastoma accounts for less than 5% of all cases of neuroblastoma but carries a favorable prognosis with most patients being stratified into low- or intermediate-risk groups for recurrence of disease. The epidemiology of neonatal (age less than 30 days) neuroblastoma contrasts greatly with patients older than 30 days. Owing to the unique potential for spontaneous regression of this tumor type, several groups have sought to reduce therapy given to neonates with neuroblastoma and use a watch-and-wait approach for many low-risk patients. Ongoing studies also seek to reduce therapy for some intermediate-risk patients in an attempt to lessen exposure to surgical intervention and cytotoxic chemotherapy. In this review, we discuss the epidemiology, clinical presentation, staging, and treatment for neonates with neuroblastoma.

Current Management of Fetal and Neonatal Renal Tumors by Michael Berger, Dietrich von Schweinitz (188-194).
Fetal and neonatal renal tumors are rare. Nevertheless, in-depth understanding of their features can lead to early recognition and appropriate treatment, ultimately benefiting outcome. Despite the many obvious similarities, important distinctions exist between these tumors and their counterparts in older children. Likewise, some important distinctions exist between fetal tumors on the one hand and neonatal tumors on the other. In this article, we review the pertinent features of fetal and neonatal renal tumors and specifically point out the important individualities in their clinical management.

Current Management of Neonatal Liver Tumors by Max R. Langham, Wayne L. Furman, Israel Fernandez-Pineda (195-204).
This review is focused on the special issues and challenges confronting physicians and surgeons caring for an unborn child, or a newborn with a liver tumor. Liver tumors at this age are very rare and they make it difficult for pediatric surgeons to gain experience necessary to obtain good results. On the other hand, adult hepatobiliary surgeons faced with a fetus or infant with a liver mass are ill equipped to care for the patient even if they have done a high volume of adult liver surgery and are expert in the field. Often a team approach is the best solution.

Neonatal Germ Cell Tumors by John A. Sandoval, Regan F. Williams (205-215).
Excluding hemangiomas, germ cell tumors (GCTs) are the most common neoplasm in neonates, comprising a wide range of benign and malignant tumors with unique histological diversity. Although these tumors are relatively rare, antenatal ultrasonography has increased their detection before birth. Extragonadal GCTs (EGCTs) are the most common GCTs in neonates, with sacrococcygeal teratomas (SCTs) being the most prevalent EGCTs. SCTs are characterized as protruding masses arising from the sacrococcygeal region with or without variable extension into the presacral space. Patients with SCTs typically have an excellent prognosis, though this is dependent on the ease of surgical resection, the timing of diagnosis, and the malignant potential of the tumor. Whereas some GCTs can be diagnosed and treated prenatally, this review focuses on postnatal management of GCTs. We explore the embryological development, histopathology, biomarkers, clinical presentation, diagnostic features, and treatment of neonatal GCTs. Because the overall prognosis is excellent for the majority of patients with GCTs, we also discuss the potential long-term effects of antineoplastic agents used to treat patients with malignant disease.

Current Management of Neonatal Soft-tissue Sarcomas and Benign Tumors with Local Aggressiveness by Israel Fernandez-Pineda, Michael D. Neel, Bhaskar N. Rao (216-225).
Neonatal soft-tissue tumors are rare and comprise a heterogeneous group of neoplasms with substantial histological diversity. Treatment options include careful observation, primary surgical resection or medical therapy. Although histologically benign, some neoplasms do exhibit an aggressive local behavior. The most common soft-tissue sarcomas in this age group include rhabdomyosarcoma, fibrosarcoma, malignant rhabdoid tumor and hemangiopericytoma. Prenatal diagnosis on routine ultrasound or in the context of a known predisposition syndrome is increasingly becoming more common. Management of neonatal tumors requires a multidisciplinary team that includes obstetricians, neonatologists, pediatric oncologists, pediatric surgical specialists and psychological support for the family members. Treatment is particularly challenging due to the difficulty in appropriate dosing of chemotherapeutic agents or the limitations of the use of radiation therapy. Although surgical treatment is predominant in this age group, close observation may be appropriate, since spontaneous regression has been reported for certain histological subtypes.

Current Management of Vascular Tumors in the Neonate by Juan C. Lopez-Gutierrez (226-232).
Neonatal vascular tumours present particular difficulties in their diagnosis and treatment. The special behaviour of neonatal hemangioma and the aggressive but benign nature of kaposiform hemangioendothelioma provide examples of a modern need for a conservative approach. Recent advances in their pharmacological management have dramatically changed the therapeutical scope of this group of tumors with surgery playing a minor role in their treatment. Multidisciplinary teams management involving neonatology, pediatric surgery, dermatology, pathology, genetics and radiology specialists among others remains the best option for an optimal outcome.