Lipoblastomas are rare benign soft tissue tumors (6% of all pediatric soft tissue tumors) that are composed of immature lipoblasts and mature adipocytes. Lipoblastomas primarily occur in infants and children younger than three years of age and most frequently occur in the extremities. Less than 10 case reports exist of pelvic lipoblastomas. We report two children with pelvic lipoblastoma with the typical presentation of a rapidly enlarging, painless, fat-containing mass. A 30-month-old girl presented with an enlarging gluteal mass over a period of four months. CT performed at an outside institution showed a large fat-containing intra-pelvic lesion anterior to the sacrum that extended to the perineum. An atypical sacrococcygeal teratoma was suspected. Subsequently, MRI demonstrated a large fairly-well-circumscribed intra-pelvic lesion that followed fat on all sequences with the exception of a few thin, enhancing septations. A 14-year-old boy presented with a large firm scrotal/inguinal mass during a wellness visit. The child reported the lesion had been rapidly increasing in size over the past two months. Ultrasound showed a >15 cm inguinal mass that extended both into the pelvis and left hemiscrotum. The mass was homogeneous in echotexture and displaced but did not involve the epididymis and testicle. Based on concern for a malignant lesion, subsequent evaluation with PET-CT showed the lesion to be predominantly composed of fat with only low level F-18-FDG uptake in a few internal septations. In both cases, lipoblastoma was confirmed after surgical excision with pathologic examination and immunohistochemistry. Imaging serves to demonstrate the macroscopic fat content of lipoblastomas but cannot reliably differentiate lipoblastoma from liposarcoma. Therefore, imaging primarily contributes information about size and anatomic relationships relevant to surgical approach. Surgical excision is the treatment of choice; however, the lack of a capsule makes complete excision difficult and local recurrence is common. Close clinical follow-up is recommended for a minimum of five years, with imaging follow-up by MRI or CT utilized when the tumor’s anatomic location precludes or limits detection by physical examination. Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Ledbetter Karyn, Leino Daniel, Trout Andrew

Keywords: Lipoblastoma, Pelvic, Scrotal

An 11-day-old male presented with recurrent choking episodes. Chest radiograph demonstrated partial opacification of the right hemithorax with associated air- bronchograms. Due to concern for tracheoesophageal fistula, a single-contrast barium esophagram was performed and demonstrated an anomalous bronchus arising from the distal esophagus. Subsequent CT of the chest showed an esophageal bronchus arising from the gastroesophageal junction with branches extending to the right lower and right upper lobes. No additional intrathoracic anomaly was identified. The patient was then treated with right upper lobectomy, right lower lobe apical segmentectomy and resection of the esophageal bronchus. The esophageal bronchus is a rare communicating bronchopulmonary foregut malformation in which a bronchus arises from the esophagus. This anomalous bronchus may supply an entire lung or a single lobe. Although the lung parenchyma associated with the aberrant bronchus is classically supplied by the pulmonary circulation (thereby distinguishing it from a sequestration), additional anomalies are often seen. A few of the more frequently observed associated anomalies include pulmonary artery anomalies, esophageal atresia, duodenal atresia and tracheoesophageal fistula. Diagnosing an esophageal bronchus begins with chest radiography, which will demonstrate a pattern of airspace disease that resembles a lobar pneumonia. This pattern of opacification is atypical in the neonatal setting, where the vast majority of pulmonary diseases, including pneumonia and aspiration, typically manifest as diffuse and bilateral processes. As patients with an esophageal bronchus also present with feeding difficulties, obtaining an esophagram is the next step in making the diagnosis. The esophagram will demonstrate filling of an abnormal structure that is often seen to be directed cranially and, with continued opacification, may be observed as a branching structure overlying opacified lung. Finally, cross-sectional imaging can be obtained to delineate the vasculature associated with the esophageal bronchus and to assess for additional anomalies. The most common treatment of an esophageal bronchus is lobectomy. However, when the associated lung parenchyma is normal, performing an anastomosis between the esophageal bronchus and the tracheobronchial tree has been reported. On the contrary, pneumonectomy may be required in cases where anomalous vascularity precludes lobectomy. Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Ledbetter Karyn, Chernoguz Artur, Shaaban Aimen, Kraus Steven

Keywords: Esophageal, Congenital

Congenital lung lesions continue to be diagnosed prenatally with increasing frequency. While the etiologies frequently encountered have been well described, there are contradictions in the literature about the prevalence of pathologies. Additionally, no gender or lobar predilection has been confidently reported. The goal of this study was to delineate the epidemiology of congenital lung lesions and their mimickers at our institution to provide comparison to published data. Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Ledbetter Karyn, Adler Elena, Subramanyam Rajeev, Mahmoud Mohamed, Kline-fath Beth, Fleck Robert

Keywords: bronchial atresia, Congenital, CPAM