Outcomes of a pediatric surgical oncology fellowship in a pediatric cancer institution.

Surgery plays an important role as part of the treatment plan in most children with malignant solid tumors in regards to initial biopsy, upfront resection, and delayed resection. Surgeons also play a critical role in the treatment of surgical complications that may arise during medical treatment. The pediatric surgical oncologist should be familiar with the current treatment guidelines, histology implications, chemotherapy and radiation side effects, tumor staging, and overall care of the child with cancer. Specific training in pediatric surgical oncology is not widespread internationally and it represents a potential undervalued intervention for improving global pediatric cancer care.

Guidewire Catheter Exchange in Pediatric Oncology: Indications, Postoperative Complications, and Outcomes.

BACKGROUND: Maintaining long-term central venous catheters (CVCs) in children undergoing chemotherapy can be challenging. Guidewire catheter exchange (GCE) replaces a CVC without repeat venipuncture. This study evaluated the indications, success rate, and complications of GCE in a large cohort of pediatric cancer patients. PROCEDURE: Medical records of pediatric cancer patients who underwent GCE at our institution between 2003 and 2013 were retrospectively reviewed. Variables analyzed included gender, age at GCE, primary cancer diagnosis, indication for GCE, absolute neutrophil count (ANC) at GCE, vein used, success rate, and postoperative complications (<30 days after exchange). RESULTS: A total of 435 GCEs performed in 407 patients (230 males and 177 females) were reviewed. Median age at GCE was 8 years (range, 0.2-24). Acute lymphoblastic leukemia was the most common diagnosis (50.6%). The primary indication for GCE was the desire to have an alternative type of CVC (71%). Other indications included catheter displacement (17%), catheter malfunction (11%), and catheter infection (1%). Median ANC at GCE was 2,581/mm(3) (range, 0-43,400). Left subclavian vein was more commonly used (57.7%). The success rate of GCE was 93.4% (406 of 435 procedures, 95% confidence interval: 91.0-97.5%). A total of 33 (7.5%) postoperative complications occurred including central line associated bloodstream infection (CLABSI) (n = 20, 4.5%), catheter dislodgement (n = 6, 1.4%), and catheter malfunction (n = 7, 1.6%). CONCLUSIONS: We conclude that GCE in pediatric cancer patients is associated with a high success rate and a low risk of complications. The most common postoperative complication, CLABSI, occurred at a rate significantly lower than following de novo CVC placement.

Safety of central venous catheter placement at diagnosis of acute lymphoblastic leukemia in children.

BACKGROUND: Central venous catheters (CVC) facilitate the management of patients with cancer. Optimal timing for placement of a CVC is controversial. We sought to determine whether early placement in children with acute lymphoblastic leukemia (ALL), a group at high risk for infection and thrombosis, was associated with an increased rate of surgical complications. PROCEDURE: We evaluated the incidence and risk factors for early surgical complications in children with ALL diagnosed between 2004 and 2009 at a single pediatric cancer center. RESULTS: One hundred seventy-two patients were studied. There were 17 episodes of bloodstream infection, for a 30-day incidence of 9.8% (95% CI, 5.9-15%). There were no surgical site infections and no CVC was removed due to infection. Early thrombosis occurred in only one patient, 3 days after CVC placement. Infection was not influenced by catheter type, patient age, body mass index, or fever at the time of placement. The infection rate was not statistically higher when the ANC was <500/mm(3) at the time of CVC placement (14.2% vs. 6.8%; P = 0.12). CONCLUSION: Early CVC placement at the time of diagnosis of ALL was associated with a low surgical complication rate with no catheters requiring removal due to infection. Utilizing our current methods of preoperative preparation, surgical management and postoperative CVC care, early placement of a CVC is safe in children with ALL even when their ANC is <500/mm(3) , but larger cohort studies would be helpful to further clarify this issue.

Treatment of metastatic osteosarcoma at diagnosis: a Pediatric Oncology Group Study.

PURPOSE: To estimate the duration of survival (S) of patients with metastatic osteosarcoma (MOS) at diagnosis treated with a multiagent, ifosfamide-containing chemotherapeutic and surgical regimen and to evaluate the toxicity of this regimen. PATIENTS AND METHODS: Thirty patients aged younger than 30 years received two courses of ifosfamide followed by surgery on the primary tumor and metastatic sites. Patients then received a postsurgical multiagent chemotherapeutic regimen that consisted of high-dose methotrexate (HDMTX), ifosfamide, doxorubicin, and cisplatin. RESULTS: The 5-year event-free survival (EFS) rate was 46.7% (95% confidence interval [CI]; 28.5 to 64.9) and 5-year S rate was 53.3% (95% CI; 35.1 to 71.5). Three patients with bone metastases and one patient with lymph node metastases died. Twenty-six patients presented with pulmonary metastatic nodules only. Eight of these patients had at least eight nodules at diagnosis and had an estimated 5-year EFS rate of 25.0% compared with 66.7% for the 18 patients with less than eight nodules (P=.06). Fourteen patients presented with bilateral lung metastases and had a 5-year EFS rate of 35.7% compared with the 12 patients who presented with unilateral involvement and had a 5-year EFS rate of 75.0% (P=.03). The hematopoietic toxicity experienced by the patients during the entire regimen was relatively mild. Seven patients had renal toxicity characterized by hypophosphatemia and/or hypokalemia. CONCLUSION: This ifosfamide-containing regimen is tolerable and effective in the treatment of patients with osteosarcoma (OS) who present with lung metastases. However, better regimens are required for this group of patients.

Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: The National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either STD courses (3 days) or PI treatment with doxorubicin. RESULTS: The 2-year relapse-free survival (RFS) rates for LR patients were 91.3% for 544 randomized to treatment with PI and 91.4% for 556 randomized to treatment with STD chemotherapy (P = .988). The 2-year RFS rates for HR patients were 87.3% for 299 randomized to treatment with PI and 90.0% for 288 randomized to treatment with STD chemotherapy (P = .865). CONCLUSION: We conclude that patients treated with PI combination chemotherapy for LR or HR WT or clear cell sarcoma of the kidney have equivalent 2-year RFS to those treated with STD regimens. PI drug administration is recommended as the new standard based on demonstrated efficacy, greater administered dose-intensity, less severe hematologic toxicity, and the requirement for fewer physician and hospital encounters.

Unsuspected pulmonary vascular abnormalities associated with diaphragmatic hernia.

The pulmonary vasculature of 12 newborn infants who died with unilateral diaphragmatic hernias was studied. Four developed severe ventilatory insufficiency after birth and promptly died. Their lungs were both hypoplastic and airless dur to compression by displaced abdominal viscera and mediastinal shift. Corrective surgery permitted lung expansion and adequate ventilation for one to two hours in five infants. Subsequently, ventilatory insufficiency supervened and they died. Postoperative blood gases revealed a right-to-left ductus arteriosus shunt in one infant. Ten of the infants with hernias had a significantly greater mass of muscle in pulmonary arteries than did matched controls. This may partially explain the fetal-type circulatory infants.

Correction of essential fatty acid deficiency in newborn infants by cutaneous application of sunflower-seed oil.

Two newborn infants receiving long-term, fat-free parenteral nutrition developed essential fatty acid (EFA) deficiency. Biochemical evidence of EFA deficiency was documented in plasma, red blood cells, and adipose tissue and included a decrease in arachidonic and linoleic acids, an increase in 5,8,11-eicosatrienoic acid, palmitoleic and oleic acids and a trienoic/tetraenoic ratio of more than 0.4. Cutaneous application of sunflower-seed oil, a source rich in the essential fat linoleic acid, rapidly reversed the clinical and biochemical manifestations of deficiency in plasma.

Management of septic complications associated with Silastic catheters in childhood malignancy.

From January, 1979, to December, 1984, 63 Hickman or Broviac catheters were inserted into 50 high risk pediatric oncology patients (median age, 37 months). Catheters remained in place for an average of 241 days. Possible catheter sepsis and exit site infection accounted for the majority (39 of 76) of the complications of long term central venous catheterization. Neutropenia (absolute neutrophil count under 500/mm3) was associated with 70% of the catheter-related infections and 75% of the non-catheter-related infections. Catheters inserted during neutropenic episodes (23) were associated with an increased risk of subsequent septicemia (60% vs. 25%), a finding apparently related to their exposure to further neutropenia (38% vs. 16% catheter days). Of the 32 episodes of septicemia of unknown origin, 19 involved Gram-negative bacteria, 14 involved Gram-positive bacteria and 4 were caused by fungi. Five of these episodes involved multiple organisms. Staphylococcus epidermidis was the most common Gram-positive organism isolated (7 of 14). Four episodes of septicemia resolved before therapy and are considered false positive cultures. Of the other 28 episodes of septicemia, 25 (89%) were successfully treated without catheter removal including 3 episodes of fungemia and 4 of multiple organism sepsis. These data demonstrate the efficacy of antimicrobial treatment without catheter removal in the pediatric oncology population with catheter-associated infections including those associated with neutropenia, multiple organisms and fungemia.

Clinical and roentgenographic findings in splenic abscess.

A splenic abscess developed in a 16-year-old boy following a supposed viral illness and left lower thoracic trauma. Preoperative diagnosis was at first obscured, but a spleen scan suggested fractured spleen and a splenic arteriogram showed a "subcapsular hematoma" and an aneurysm of the left hepatic artery. A 1,800-gm spleen containing one large abscess and one small one was removed. Splenic abscess is rare and, before modern methods of spleen scan and arteriography, rarely diagnosed.

Primary pulmonary neoplasms of childhood: a review.

Primary tumors of the lung rarely occur in children. However, 230 well-documented cases, including the 2 presented in this review, have been identified in the English-language literature. One hundred fifty-one tumors in these reports were classified as malignant lesions and 79 as benign neoplasms. Bronchial "adenomas" constituted the largest group; most of these lesions were of the carcinoid variety, and 8% were definitely malignant. Forty-seven cases of bronchogenic carcinoma were reported in children under 16 years of age, although there were few squamous cell tumors (12%). Fifty-six percent of the benign tumors were classified as inflammatory pseudotumor. Most of the children in this collective series were seen with symptoms related to bronchial irritation or obstruction, such as cough, hemoptysis, atelectasis, or pneumonitis. Respiratory distress was an unusual symptom that was often associated with large tumors seen in the neonatal period. Approximately 20% of the children were totally symptomatic. The limited survival data that are available indicate that leiomyosarcoma and mucoepidermoid carcinoma have a more favorable prognosis in children than in adults. Survival with bronchial carcinoid tumors (90%), bronchogenic carcinoma (30%), and pulmonary blastoma (45%) appears to parallel that for adults. The experience with pulmonary rhabdomyosarcoma, as described in this review, emphasizes the importance of early diagnosis and the use of combined modes of therapy in the approach to these malignancies. Despite the rarity of primary pulmonary neoplasms in children, this diagnosis should be considered in young patients with solitary pulmonary masses or persistent, atypical pulmonary symptoms. It is hoped that early diagnosis will result in an improved prognosis and prevent life-threatening complications.

Transabdominal bone biopsy during staging laparotomy for Hodgkin's disease.

A bone marrow biopsy is one of the recommended components of the exploratory laparotomy performed for the staging of Hodgkin's disease. A simple method is described for obtaining a full thickness biopsy of the ilium via the laparotomy incision using the Cloward drill.

Update on solid tumor management in childhood.

Surgery will continue to play an important role in the multidisciplinary treatment of solid tumors in children. However, as the complexity of therapy increases, it is imperative that the pediatric surgeon become familiar with new radiation therapy techniques, new chemotherapeutic agents, and the advances that are being made in immunodiagnosis, immunotherapy, and molecular biology. New markers for various tumors are being discovered and have therapeutic and prognostic value that may have a significant impact on surgical therapy. New protocols are being developed within the various cooperative research groups, and the surgeon must participate actively, so that surgical questions of importance can be answered and so that adequate tissue specimens can be obtained for future investigations.

Wilms' tumor: diagnosis and treatment in the 1990s.

The present therapy of Wilms' tumor is a classic example of the modern multidisciplinary treatment of childhood malignancies, and the organization of the National Wilms' Tumor Studies (NWTS) has served as a prototype for other collaborative studies of childhood tumors. More than 80% of all children with Wilms' tumor can now be expected to have a long term relapse free survival. The NWTS is in large part responsible for this excellent outlook and continues its emphasis on decreasing the morbidity of therapy without sacrificing relapse free survival by tailoring therapy based on histology and accurate surgical staging. The pathological classification of Wilms' and its effect on therapy and prognosis is now being used as a guide in the study of other childhood malignancies. The surgeon continues to play a key role in the multimodality management of the child with a Wilms' tumor since subsequent therapy will be determined by the extent of the tumor at the time of operation. The identification of histological type, the presence or absence of residual tumor within the abdomen following resection, and the status of lymph node metastases are of utmost importance. Advances in cytogenetics and refinement of histopathologic staging will allow the pediatric surgical oncologist, pediatric oncologist, and pediatric radiation therapist to continue to modify therapy for specific patient groups in the future.

Hemopericardium from coronary artery laceration complicating extracorporeal membrane oxygenation.

OBJECTIVE: We report the clinical course and successful surgical treatment of hemopericardium resulting from coronary artery (CA) laceration in two patients with congenital diaphragmatic hernia (CDH) undergoing extracorporeal membrane oxygenation (ECMO) bypass. STUDY DESIGN: Retrospective case review. RESULTS: Two neonates with CDH had needle aspiration for either pneumothorax or pericardial effusion before initiation of ECMO. While on bypass, progressive hemopericardium led to narrow pulse pressure and decreased venous return that limited bypass flow. Widened cardiac silhouette on chest radiographs suggested hemopericardium; echocardiography was confirmatory in one case. The underlying diagnosis of CA laceration was made during pericardiotomy and treated with surgical patching. CONCLUSIONS: Pre-ECMO history of cardiothoracic needle aspiration is important because complications such as hemothorax or hemopericardium may arise once ECMO bypass is initiated. Inadvertent CA laceration may lead to acute hemopericardium, compromising venous drainage. However, CA laceration can be successfully repaired while the patient is on bypass.

Abdominal mass lesions in the newborn: diagnosis and treatment.

Most neonatal abdominal masses will be due to benign retroperitoneal lesions such as hydronephrosis and multicystic dysplastic kidney. Although history and physical examination, plain radiographs and ultrasonography will confirm most diagnoses, severe unilateral hydronephrosis, hemorrhagic neuroblastoma, and intraperitoneal cysts may provide diagnostic difficulties. Masses identified by prenatal ultrasound need careful evaluation as they may represent normal structures, nonsignificant variants, or physiologically significant anomalies. Many lesions will require operative intervention, which can be safely performed in small infants by trained personnel at facilities with appropriate support services. Genuine controversy exists in the management of some of these lesions including MDK, renal vein thrombosis, and acalculous cholecystitis.

Compression treatment of hemangiomas.

Compression therapy appears to be a safe and effective modality for treating involuting and non-involuting hemangiomas. Compression may induce earlier mature resolution in involuting hemangiomas, and may reduce the size of non-involuting hemangiomas--at least temporarily.

Renin producing Wilms' tumor.

A 4-yr-old boy with Wilms' tumor, hypertension, and hyperreninemia is presented. Three times as much renin was extracted from tumor tissue as from adjacent renal parenchyma, suggesting that the tumor itself was the primary source of the renin and cause of the hypertension. Other reported cases of hypertension and increased renin production with Wilms' tumor are reviewed. Hypertension is a very frequent finding in patients presenting with Wilms' tumor. The possibility that many of these neoplasms may be found to hypersecrete renin if properly investigated is discussed.

Peritoneal drainage as primary management of perforated NEC in the very low birth weight infant.

Advances in perinatal and neonatal care in the past decade have produced a change in the population of infants with perforated necrotizing enterocolitis (NEC) treated at our institution: the majority are now of very low birth weight (VLBW, < 1,000 g). Peritoneal drainage has been reported as an initial resuscitative procedure for unstable infants who have complicated NEC. Initial success with peritoneal drainage prompted us to adopt an aggressive approach to its use in this patient population. Since 1987, peritoneal drainage has been the primary treatment for most infants weighing less than 1,500 g who have perforation, and for unstable infants weighing more than 1,500 g. Perforation was documented by pneumoperitoneum or aspiration of meconium by paracentesis. Intestinal resection was performed in most infants weighing more than 1,500 g and in those for whom drainage was ineffective. Twenty-nine infants with low or VLBW (mean gestational age, 27 weeks; mean birth weight, 994 g) were treated with one or two drains in the right lower quadrant. Broad spectrum antibiotics were continued until all drains were removed, usually within 10 to 14 days. Nasogastric suction was continued until patency of the gastrointestinal (GI) tract was confirmed by a nonionic upper GI series. Six (21%) infants died, although one of the deaths occurred 5 months after drainage; the patient had chronic lung disease and an intact GI tract. Seventeen of the 23 (74%) survivors required no further operative procedure, and 6 (26%) required laparotomy and resection because drainage had been ineffective. Peritoneal drainage provided definitive treatment in 18 of 29 (62%) infants in this series.(ABSTRACT TRUNCATED AT 250 WORDS)

"Waterseal" gastrostomy in the management of premature infants with tracheoesophageal fistula and pulmonary insufficiency.

The perioperative management of premature infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) complicated by pulmonary insufficiency continues to be a challenge. Definitive repair is usually delayed or staged and a gastrostomy is initially placed to prevent reflux aspiration. In patients with decreased pulmonary compliance, gastrostomy placement results in decreased intragastric pressure leading to a loss of ventilatory pressure via the tracheoesophageal fistula. A technique using the principle of underwater seal to maintain effective ventilatory pressure after gastrostomy placement is described, and two illustrative cases are presented. After acute respiratory decompensation in these patients, the gastrostomy tube was connected to underwater seal resulting in improved respiratory status. The underwater seal is allowed to intermittently "bubble," thereby permitting partial gastric decompression. This technique maintains effective ventilatory pressure after gastrostomy placement in premature infants with EA/TEF and pulmonary insufficiency until definitive therapy can be achieved.

Esophageal atresia with triple congenital tracheoesophageal fistulae.

The surgical management of an infant with esophageal atresia and three (2 proximal and 1 distal) congenital tracheoesophageal fistulae is described. The pitfalls in the diagnosis of proximal fistulae are discussed.