The pediatric population is not immune to the various types of cancer encountered by the adult population in terms of the organs and organ systems affected. Despite advances in treatments and detection, cancer is still the number 2 cause of death within the pediatric population. The types of tumors encountered in the younger population are often composed of more uniquely immature cellular lineages and only a small percentage have preventable causes. The goal of our single institution presentation is to provide the interested viewer with a comprehensive review of several uniquely encountered immature pediatric tumors. Our presentation will include imaging examples utilizing multiple modalities demonstrating examples of pancreaticoblastoma , immature lipoblastoma, neuroblastoma, hepatoblastoma, a case of biopsy confirmed small cell undifferentiated renal rhabdoid tumor, and a unique case of pleuropulmonary blastoma. Explanations pertaining to the key imaging features of each entity as reported in the literature will be provided to supplement the case examples from our institution. Read More

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

Authors: Myers Ross, Dietsche Eric, Gnerre Jeffrey, Lecompte Leslie, Gilet Anthony

Keywords: Oncology, Gastrointestinal, Thoracic

Congenital heart diseases often occurs secondary to a variety of insults and rotational errors during development in utero. These can range from a spectrum of simple to more complex pathologies including arterial switches. It is important for radiologists to recognize the embryology and complications of arterial switches to further management. Today largely secondary to new advances in technology both corrected and uncorrected arterial switches are diagnosed more commonly; previously many patients with arterial switches may not have survived into adulthood. In utero, the primitive truncus is normally positioned anterior and midline. It eventually divides into the aorta and the pulmonary artery, which then rotates clockwise 150 degrees such that the pulmonary artery lies anterior to and left of the aorta. When variations occur it can result in a congenitally corrected transposition such as L- transposition of the great arteries (L-TGA) where the two ventricles are morphologically switched in position. With L-TGA, the truncus rotates 30-degrees clockwise which results in the aorta being anterior and leftward in relation to the pulmonary artery. In uncorrected transposition (R-TGA) the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, secondary to a 30-degree counterclockwise rotation of the primitive truncus, the aorta is then located rightward and anterior to the pulmonary artery. This condition needs to be corrected surgically due to cyanosis resulting from the right ventricle not being able to supply the systemic circulation. Other variations of arterial switches that can occur is situs inversus where the rotation of the aorta and pulmonary artery is completely opposite of that which would be considered normal. Lastly in truncus arteriosus, the primitive truncus does not divide into a separate aorta and pulmonary artery. We will provide several cases from our institution of arterial switches on imaging as well as diagramatic representative models describing the embryology of how arterial switches and rotations occur. We also discuss the repairs and complications of these cases. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Wu Jennifer, Myers Ross, Sadowsky David, Li Tianyang, Tsui Edison, Sonke Pierre-yves

Keywords: cardiac, Pulmonary

Müllerian Duct Anomalies (MDA) are a broad category of congenital deformities of the urogenital structures due to abnormal development, fusion, or resorption of the fetal Müllerian Ducts. By definition, the Müllerian Ducts are the paired fetal structures that are located medially to the Wolffian Ducts and develop into the Fallopian Tubes, uterus, cervix, and upper two-thirds of the vagina. The multitude of symptoms associated with MDAs vary broadly in severity, ranging from patients with no symptoms and incidentally discovered MDAs to patients with primary infertility, recurrent pregnancy loss and other reproductive problems. There are well documented associations of MDAs with other genitourinary malformations. The most commonly reported is renal agenesis, which accounts for up to 30% of all associated renal anomalies in the setting of MDA, however abnormalities including ureteral remnants with ectopic insertion or collecting system duplication have been reported as well. The overall incidence of MDAs varies, but have been estimated to be approximately 1-5% within the general population and up to 13-25% in patients with recurrent pregnancy loss. The most commonly accepted system of classification of the various types of MDAs is that of the American Fertility Society, which divides MDAs into seven categories. However, there is often confusion as to how to categorize MDAs demonstrating characteristics of more than one class. Medical imaging is of essential importance for both this reason and for appropriate planning of therapy. The imaging of suspected MDAs involves the implementation of a combination of 3D Ultrasound, Magnetic Resonance Imaging, Sonohysterography, Hysterosalpingraphy and/or laparoscopy. Sonography and MRI have the benefits of being the least invasive methods of diagnosis. The goal of our educational exhibit will provide an overview of the embryology and pathophysiology related to a broad variety Müllerian Duct Anomalies. We will discuss the common imaging techniques employed in the workup of patients with Müllerian Anomalies. In addition, we provide a plethora of interesting cases from our home institution including a case of Mayer-Rokitansky-Küster-Hauser Syndrome, Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA), cloacal abnormality, bicornuate uterus and other complex cases utilizing a variety of imaging modalities. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Myers Ross, Li Tianyang, Wu Jennifer, Sadowsky David, Paul Mary, Gerard Perry, Brudnicki Adele, Lecompte Lesli

Keywords: Mullerian Duct Anomalies, OHVIRA