Prakash Masand

Undifferentiated Embryonal Sarcoma of the Liver (UESL): A Single-Center Experience and Review of the Literature.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive pediatric malignancy. The purpose of this study was to review the clinical, radiologic, and pathologic features and outcome of children with UESL at our institution, in the United Network of Organ Sharing database and to review the existing literature to define the state of the art for children with UESL. Six children were diagnosed with UESL at the Texas Children’s Cancer Center between 1993 and 2014, 12 children underwent liver transplantation registered in the United Network of Organ Sharing database, and 198 children with UESL were described in 23 case series during 1978 to 2014. Patients were treated with multimodal treatment approaches including primary surgical resection, neoadjuvant and/or adjuvant chemotherapy, and liver transplantation resulting in overall survival reported between 20% and 100% with significant improvement over the recent years. We show that complete tumor removal remains the key element of treatment and our single-institutional experience and data in the published literature suggest that combination chemotherapy with ifosfamide and doxorubicin to facilitate complete surgical resection is an effective approach to cure children with UESL.

Outcomes of surgical intervention for anomalous aortic origin of a coronary artery: A large contemporary prospective cohort study

Objective The purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm. Methods All patients aged 2 to 18 years undergoing surgical intervention for anomalous aortic origin of a coronary artery between December 2012 and April 2017 were prospectively included. Patients underwent stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and retrospectively electrocardiogram‐gated computed tomography angiography preoperatively. Patients were cleared for exercise at 3 months postoperatively if asymptomatic and repeat stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and computed tomography angiography showed normal results. Results A total of 44 patients, with a median age of 14 years (8‐18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side‐side‐anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow‐up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance. Conclusions Surgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow‐up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death.

Relapsed hepatoblastoma confined to the lung is effectively treated with pulmonary metastasectomy

BACKGROUND In children diagnosed with hepatoblastoma (HB), the lungs are the most common site of metastasis at both initial presentation and relapse. Previous studies have encouraged pulmonary metastasectomy to achieve a disease-free state after resection of the primary hepatic lesion. However, there is no consensus about how to manage recurrent pulmonary metastasis. PROCEDURE A retrospective, multi-institutional review was performed from 2005 to 2014 to identify HB patients ≤18years of age who had disease recurrence associated with pulmonary metastases alone. RESULTS Ten patients between the ages of 8 and 33months were identified. Pulmonary metastatic recurrence was detected by measuring alpha-fetoprotein (AFP) levels and/or with CT scans of the chest. All patients subsequently underwent pulmonary metastasectomy without post-operative complications. At last follow-up, 8 patients were alive and had normal AFP levels. The 8 survivors had a median follow-up from therapy completion of 18.5months. Two patients who presented with extrapulmonary recurrence subsequently died of treatment refractory disease. CONCLUSIONS This review supports surgical resection as a safe and, in the context of multimodal therapy, efficacious approach to manage HB patients who present with isolated pulmonary relapse.

Vascular invasion is a prognostic indicator in hepatoblastoma

INTRODUCTION The data regarding vascular invasion as a prognostic factor in hepatoblastoma (HB) are conflicted. The purpose of this study is to examine the relationship between vascular invasion and outcomes. METHODS This is a retrospective review of patients <18 years old who underwent resection for hepatoblastoma from 1998 to 2015. Pathology reports were used to identify patients who had pathologic vascular invasion (VI), and those who did not (NVI). RESULTS Sixty-six children were identified with a median age at diagnosis of 21months (interquartile range: 10-33months). Pathologic vascular invasion was present in 42/66 (64%) patients. A significant difference (P=0.02) in 3-year overall survival (3YOS) was detected between NVI (95%) and VI (61%). Recurrent disease was present in 8/66 (12%) patients. A marginally significant difference (P=0.08) was found in 3-year recurrence free survival (3YRFS) between NVI (94%) and the VI (76%) groups. Patients with NVI had no metastatic disease, had a lower recurrence rate, universally responded to neoadjuvant chemotherapy, and were less likely to have small cell undifferentiated histology. Twenty-one children underwent orthotopic liver transplant (OLT), with no difference in 3YROS or 3YRFS. CONCLUSION Pathologic vascular invasion is associated with significantly worse 3YOS in HB, and lack of vascular invasion was associated with more favorable disease characteristics. The presence of pathologic vascular invasion did not confer a worse outcome in patients treated with liver transplantation in this cohort of patients. TYPE OF STUDY Retrospective review. LEVEL OF EVIDENCE Level III.

Focal nodular hyperplasia in children: An institutional experience with review of the literature

BACKGROUND Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature. METHODS A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013. RESULTS Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size. CONCLUSIONS Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.

Congenital Left Paraduodenal Hernia Causing Chronic Abdominal Pain and Abdominal Catastrophe

Paraduodenal hernias are the most common type of congenital internal hernia. Because of its overall rare incidence, this entity is often overlooked during initial assessment of the patient. Lack of specific diagnostic criteria also makes diagnosis exceedingly difficult, and the resulting diagnostic delays can lead to tragic outcomes for patients. Despite these perceived barriers to timely diagnosis, there may be specific radiographic findings that, when combined with the appropriate constellation of clinical symptoms, would aid in diagnosis. This patient first presented at 8 years of age with vague symptoms of postprandial emesis, chronic abdominal pain, nausea, and syncope. Over the span of 6 years he was evaluated 2 to 3 times a year with similar complaints, all of which quickly resolved spontaneously. He underwent multiple laboratory, imaging, and endoscopic studies, which were nondiagnostic. It was not until he developed signs of a high-grade obstruction and extremis that he was found to have a large left paraduodenal hernia that had volvulized around the superior mesenteric axis. This resulted in the loss of the entire superior mesenteric axis distribution of the small and large intestine and necrosis of the duodenum. In cases of chronic intermittent obstruction without clear etiology, careful attention and consideration should be given to the constellation of symptoms, imaging studies, and potential use of diagnostic laparoscopy. Increased vigilance by primary care and consulting physicians is necessary to detect this rare but readily correctable condition.

STANDARDIZED APPROACH TO PATIENTS WITH ANOMALOUS AORTIC ORIGIN OF A CORONARY ARTERY: RESULTS FROM THE CORONARY ANOMALIES PROGRAM AT TEXAS CHILDREN’S HOSPITAL

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data is lacking. Prospective, observational study of patients (pts) seen with AAOCA from 12/2012 to 04/2014. The

Diaphragmatic Hernia Associated With Absent Ductus Venosus and Anomalous Connection of an Obliterated Umbilical Vein to the Coronary Sinus

Umbilical vein anomalies are a rare congenital defect, which have been associated with absent ductus venosus, with few cases also involving a congenital diaphragmatic hernia. We describe a case of postnatal development of an anterior diaphragmatic hernia of Morgagni in a four-year-old patient diagnosed prenatally with mesocardia, absent ductus venosus with a large umbilical vein, a large secundum atrial septal defect, and patent ductus arteriosus.

Concurrent hepatic hemangioma and solitary fibrous tumor: diagnosis and management

Hepatic solitary fibrous tumor (HSFT) is a very rare benign liver tumor without well-defined findings on imaging. Even with multiphase advanced contrast-enhanced liver imaging, a definitive preoperative diagnosis is impossible. The diagnostic process can be further complicated when there are two concurrent lesions with different radiologic appearances. Here, we compare the findings of a commonly encountered liver lesion, hepatic hemangioma, with those of an exceedingly rare lesion, HSFT.

Hepatoblastoma Associated with Trisomy 18.

Very few reports exist in the literature regarding a possible association between trisomy 18 patients and the incidence of hepatoblastoma. Fewer reports exist on patients with multifocal hepatoblastoma. We reviewed our institutional database for the past 10 years and found three cases with this possible association to the tumor.