Surgical Oncology
“Pediatric Surgical Oncology”
Guest Editor: Kenneth W. Gow, University of Washington, Seattle, WA, USA
Surgical Oncology on ScienceDirect: http://www.sciencedirect.com/science/journal/09607404
Abstracts:
Titilope A. Isholaa and Dai H. Chungb
aDepartment of Surgery, The University of Texas Medical Branch, 301 University Blvd., Galveston, TX 77555, USA
bSealy Center for Cancer Cell Biology, The University of Texas Medical Branch, 301 University Blvd., Galveston, TX 77555, USA
Neuroblastomas continue to remain a clinical challenge, despite advances in multimodal therapy. Currently, studies are aimed at novel targets for neuroblastoma directed toward poor prognostic indicators such as the MYCN oncogene and marked angiogenesis. There have also been recent discoveries in neuroblastoma pathogenesis involving epigenetic regulation and retinoic acid therapy. Understanding the intricate complexities of this tumor may lead to innovative agents for more effective combinational therapy.
Keywords: Neuroblastoma; MYCN; Tumor regression
Link to full-text: 10.1016/j.suronc.2007.09.005
Wilms tumor: Progress and considerations for the surgeon
University of Michigan, Ann Arbor Michigan, Associate Professor of Surgery, Vice Chair Surgery Renal Tumors Committee, Childrens Oncology Group, USA
Wilms tumor (WT) or nephroblastoma is the most common tumor of renal origin found in children. It accounts for 6% of all pediatric tumors and is the second most frequent intrabdominal solid organ tumor found in children. Initial survival rates in the early part of the last century was only 30%, but now long-term survival in both North America and European trials is approaching 85% with many low-stage tumors significantly higher. Treatment is now progressing towards “risk-based management”— based not only on stage and histology but also incorporating genetic markers [Dome JS, Grundy PE, Perlman EJ, Ehrlich PF, et al. Protocols for the renal tumors study. Childrens Oncology Group. 
www.childrensoncologygroup.org
. 2007.]. Within the multidisciplinary treatment team the surgeon plays a critical role in the diagnosis, staging and the surgeon's technical skills and judgment directs therapy and impacts outcome. The next generation of treatment for children with WT will focus on identifying subsets of patients who can be defined by some criterion as having a different outcome than their similar stage peers and who therefore require a variation in management. These include children with WT that have unsatisfactory long-term survival (less then 75%), patients of good survival but high potential for late effects and a final challenge are those children with both a poor survival and a high potential for late effects. This article presents a review of the most recent treatment considerations for WT with a focus on the surgeon's role to ensure a good outcome.
Keywords: Wilms tumor; Surgical protocols
Link to full text: 10.1016/j.suronc.2007.07.001
Pediatric surgical oncology: Management of rhabdomyosarcoma
Cynthia Leaphart and David Rodeberg
Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, 3705 Fifth Ave, Pittsburgh, PA 15213, USA
A malignant tumor of striated muscle origin, Rhabdomyosarcoma (RMS) is a childhood tumor that has benefited from 30 years of multimodality therapeutic trials culminating in a greater than 70% overall current 5-year survival. Prognosis for RMS is dependent on anatomic primary tumor site, age, completeness of resection, presence and number of metastatic sites, histology and biology of the tumor cells. Multimodality treatment is based on risk stratification according to pretreatment stage, postoperative group, histology and site. Therefore, pretreatment staging is vital for assessment and is dependent on primary tumor site, size, regional lymph node status, and presence of metastases. Unique to RMS is the concept of postoperative clinical grouping that assesses the completeness of disease resection and takes into account lymph node evaluation both at the regional and metastatic basins. At all sites, if operative resection of all disease is accomplished, including microscopic disease, survival is improved. Therefore, the surgeon plays a vital role in determining risk stratification for treatment and local control of the primary tumor for RMS.
KEYWORDS: Rhabdomyosarcoma prognosis; RMS; Surgical guidelines
Link to full-text: 10.1016/j.suronc.2007.07.003
Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)
Chan Hon Chui
Department of Pediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229 899, Singapore
Nonrhabdmyosarcoma soft tissue sarcoma (NRSTS) is a heterogenous group of tumors analyzed as a unique group because of the rarity of each histopathological subtype. Initial information available has been obtained from adult series or pediatric case studies. Recent reports by several multi-center groups have been published that evaluated prognostic factors and treatment protocols.
Established prognostic factors include tumor grade, invasiveness, size, and intergroup rhabdomyosarcoma study (IRS) group. Identification of sarcoma-specific chromosomal translocations has allowed for more accurate definitive diagnosis. Some may have prognostic significance and may offer as potential therapeutic targets. Surgery remained important, as many of the tumors are scarcely chemosensitive. With the development of multimodalities, surgical management has evolved over the years. Patients with localized unresectable disease are managed with surgery +/− radiotherapy with very good results. Localized unresectable disease is intermediate in behavior and prognosis. Patients are treated with neoadjuvant chemotherapy, +/− radiotherapy, followed by delayed surgical resection. Prognosis in patients with metastatic disease has been poor despite combination chemotherapy with surgical resection. The addition of ifosfamide and doxorubicin in the chemotherapy regimes has provided some improvement.
With the lack of new active agents, we will depend on advances in molecular techniques to develop novel targeted therapies, better molecular markers and histology-specific trials to evaluate this group of tumors.
Keywords: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS); Pediatric
Link to full-text: 10.1016/j.suronc.2007.07.006
Tumors of the liver in children
Rebecka L. Meyers
Chief Pediatric Surgery, University of Utah, Primary Children's Medical Center, 100 North Medical Drive, Suite 2600, Salt Lake City, UT 84113, USA
In this review we examine the diagnosis and treatment of pediatric liver tumors- both malignant and benign. The two most common malignant tumors are hepatoblastoma and hepatocellular carcinoma. Hepatoblastoma is seen in younger children, hepatocellular carcinoma in older children. Other malignant liver tumors are quite rare and include biliary rhabdomyosarcoma, angiosarcoma, rhabdoid tumor, and undifferentiated sarcoma. The commonly seen benign liver tumors in children are infantile hemangioma, mesenchymal hamartoma, and focal nodular hyperplasia. Rare benign tumors are hepatic adenoma, which is occasionally seen in teenage girls, and teratoma which is a very rare liver tumor in infants.
KEYWORDS: Pediatric liver tumors; Hepatoblastoma; Hepatocellular carcinoma; Hepatic sarcoma; Undifferentiated sarcoma; Rhabdoid tumor; Biliary rhabdomyosarcoma; Infantile hemangioma; Focal nodular hyperplasia; Mesenchymal hamartoma
Link to full-text: 10.1016/j.suronc.2007.07.002
Pediatric germ cell tumors
Zachary Horton, Marc Schlatter and Sarah Schultz
Helen Devos Children's Hospital, Grand Rapids Medical Education and Research Center, Michigan State University, Grand Rapids, MI 49503, USA
Pediatric germ cell tumors are a diverse group of neoplasms with variable clinical behaviors, depending upon the age and site of presentation. Most result from sporadic mutations, although environmental exposures and other genetic aberrations may play a role. Platinum-based chemotherapy has dramatically improved the event-free and overall survival outcomes of pediatric patients with malignant germ cell tumors over the past two decades. Prognosis is dependent on tumor stage and location. Patients with gonadal germ cell tumors have at least a 95% 5-year survival for early stage disease and at least a 85% 5-year survival for advanced stages. In general, extragonadal germ cell tumors carry a poorer prognosis with mediastinal location having the worst outcomes (70% 4-year survival).
Current trials are focused on maintaining similar excellent outcomes while reducing morbidity by reducing the dose and duration of chemotherapy. Cytogenetic research studies have found chromosomal aberrations specific to some of these tumors that may serve as prognosticators and even direct therapy.
Keywords: Germ cell tumors; Teratomas; Dysgerminomas; Endodermal sinus tumors; Yolk sac tumors; Choriocarcinoma; Sacrococcygeal tumors; Gonadal tumors; Ovarian tumors; Testicular tumors; Mediastinal tumors; Retroperitoneal tumors
Link to full-text: 10.1016/j.suronc.2007.07.005
Melanoma in children and adolescents
Cynthia D. Downard, Louis B. Rapkin and Kenneth W. Gow
Division of Pediatric Surgery and the Department of Hematology/Oncology/BMT, Aflac Cancer Center, Emory School of Medicine, Atlanta, GA, USA
Melanoma is rarely described in the pediatric population. However, recent studies show that the incidence may be increasing. The diagnosis of melanoma presents unique challenges in this age group. There may be predisposing factors that affect children more than adults. A high index of suspicion is necessary in order to make a timely diagnosis. Prompt surgical treatment by individuals with expertise in care of patients with melanoma with potentially curative excision and appropriate lymph node evaluation is important to optimize survival. Careful review of all specimens by an experienced dermatopathologist is important. Through cooperation with adult trials and potential inclusion of pediatric patients in evaluations of new therapies, further progress against this disease can hopefully be addressed in all age groups.
Keywords: Melanoma; Children; Adolescents
Link to full-text: 10.1016/j.suronc.2007.09.006
Minimally invasive surgery and childhood cancer
Randolph K. Cribbs, Mark L. Wulkan, Kurt F. Heiss and Kenneth W.Gow
Division of Pediatric Surgery, Emory School of Medicine, Atlanta, GA, USA
Over the last 2 decades, minimally invasive surgery (MIS) has become a significant tool for the diagnosis and treatment of malignant disease in adults. Despite initial reports of port-site metastases and peritoneal spread following laparoscopic resection of colorectal cancer in the 1990s, MIS is now commonly used for many applications in adult surgical oncology, including biopsy and resection of malignant disease in the chest and abdominal cavities, mediastinal and retroperitoneal lymph node dissection, staging of abdominal, pelvic and thoracic malignancies, and management of therapeutic complications. The use of MIS techniques in children is growing with the availability of smaller instruments and equipment more suitable to the pediatric patient. Herein, we review the role of MIS in the diagnosis, staging and treatment of malignant disease in children. We will also evaluate MIS as it applies to the palliation of disease and the management of treatment complications in childhood cancer.
Keywords: Minimally invasive surgery; Childhood cancer
Link to full-text: 10.1016/j.suronc.2007.09.002
Interventional radiology and the care of the pediatric oncology patient
Mark A. Bittles and Fredric A. Hoffer
Department of Radiology, Division of Interventional Radiology, Children's Hospital and Regional Medical Center and University of Washington, Seattle, USA
Interventional radiology has become increasingly involved in the diagnosis and management of the pediatric oncology patient. Percutaneous biopsy and needle aspiration can be performed for solid and liquid lesions with image guidance, both for the primary diagnosis and for management of sequelae of cancer therapy. Therapeutic options also can be performed with image guidance, including radiofrequency ablation and transarterial chemoembolization. When surgical resection is required, image guided tumor localization can be used to aid in identifying small lesions.
KEYWORDS: Pediatric; Oncology; Interventional; Radiology; Biopsy; Embolization; Radiofrequency ablation
Link to full-text: 10.1016/j.suronc.2007.07.004