A term 8-day-old male presented to an outside emergency department with acute respiratory distress. Prenatal history was unremarkable except for prenatal diagnosis of a right sided nonspecific congenital lung malformation (CLM), possibly a congenital pulmonary airway malformation (CPAM). Anatomy ultrasound reported a right mid-lung echogenic lesion measuring with two small internal cysts. Fetal MRI demonstrated a non-segmental T2-hyperintense lesion in the peripheral right lower lobe with two small peripheral cysts and no feeding vessel. The lung mass to volume ratio was 0.05 with favorable observed to expected total fetal lung volumes. Initial examination revealed absent breath sounds on the right, and chest radiograph confirmed a large right tension pneumothorax. A chest tube was placed, and he was transferred to a higher level of care. CT angiography of the chest demonstrated a pleural-based multicystic lesion with internal air–fluid levels and focal solid nodular components, without a systemic feeding vessel. On day 12 of life, he underwent a right lower lobe wedge resection. Intraoperatively, surgeons noted an exophytic cystic lesion arising from the medial aspect of the right lower lobe on a narrow stalk. Pathology findings revealed a multicystic lesion consistent with Type 1r pleuropulmonary blastoma (PPB). Follow up genetic testing revealed a positive test for DICER-1 genetic mutation. 6 month follow up CT was negative and he continues to be followed at higher care institution. PPB is a rare, aggressive tumor originating from primitive mesenchymal cells of the lung or pleuraprimarily affecting infants and young children. PPB occurs in 1 in 250,000 live births and is associated with DICER1 gene mutations. Early detection is essential, offering a window for curative surgery before the disease progresses. PPB is difficult to distinguish from a benign CLM and is more commonly diagnosed in infancy. As of 2022, fewer than 6 confirmed cases of PPB were diagnosed prenatally. Despite the diagnostic overlap between PPB and CLM, pediatric radiologists play a key role in raising early suspicion, guiding surgical planning, and prompting genetic evaluation when imaging features deviate from typical benign patterns. This case highlights the diagnostic complexity and clinical features of the rare occurrence of prenatal diagnosis of pleuropulmonary blastoma, a malignancy that may be underrecognized due to its overlap with benign cystic lung lesions. Read More

Meeting name: SPR 2026 Annual Meeting , 2026

Authors: Gardner Kyle, Gagnon Marie-helene

Keywords: DICER1, Congenital, Pleuropulmonary Blastoma

A 16 year old previously healthymale patient who initially presented with daily recurrent fevers and chills lasting 5-10 minutes. His parents brought him to a tertiary care hospital after developing vomiting and weight loss in the setting of progressively worsening fevers. Initial work up was remarkable for elevated CRP and a negative infectious workup. An MR enterography ordered to evaluate for a cause of weight loss demonstrated incidental lung base lesions. Follow up CTA revealed a lesion within the right ventricle and multiple lung nodules, concerning for endocarditis and septic emboli. He underwent two surgical resections over two months for the ventricular mass. Pathology revealed findings consistent with necrotizing endocarditis. During his hospital course, he was found to have a left cephalic venous thrombosis and was placed on Xarelto, later developing a right subclavian vein thrombus while on anticoagulation. He was readmitted 1 month later after presenting for right chest and right upper quadrant pain. Initial chest X-ray revealed right lower lobe mass and follow up CTA revealed two large right pulmonary artery pseudo-aneurysms. He underwent cardiac catheterization for pre-operative coiling of the pseudo-aneurysms and subsequent right lower lobectomy. Pathology revealed necrotizing arteritis and pulmonary artery pseudo-aneurysms. Based on clinical presentation and further work up, he was diagnosed with Hughes Stovin Syndrome and started on Cytoxan and Apixaban. While not yet considered in remission, his symptoms have improved since initiating treatment. Hughes Stovin Syndrome (HSS) is a rare disorder characterized by thrombophlebitis as well as multiple pulmonary and/or bronchial aneurysms. There is a male predilection, usually presenting between 12-48 years. There have been less than 40 cases published in the English literature. HSS is thought to be a variant of Bechet’s but does not present with oral or genital ulcers. The lack oral and genital ulcers in this patient excluded a diagnosis of Bechet’s. The underlying cause of HSS is unclear but is believed to be due to angiodysplasia or infection. Since there is no formally described diagnostic criteria, the clinical presentation of thrombophlebitis with pulmonary artery aneurysms characterize the disease. We present this case to illustrate the clinical presentation of a rare and possibly under recognized syndrome. Read More

Meeting name: SPR 2022 Annual Meeting & Postgraduate Course , 2022

Authors: Gagnon Marie-helene, Richer Edward, Alazraki Adina

Keywords: Vasculitis, Aneurysm, Thrombophlebitis

The purpose of this educational exhibit is to: 1.Review the embryology, classification, and imaging features of anorectal malformations (ARMs). 2.Review the indications and techniques of Contrast-enhanced colosonography (CeCS) in pediatric patients with ARMs. 3.Provide sample cases and clinical courses of patients with specific ARMs. Anorectal malformations(ARMs) are a spectrum of congenital anomalies involving abnormal development of the distal rectum and anus, leading to an interruption or misconnection of the normal passage between the rectum and the perineum. The condition occurs in about 1 in 5,000 live births and can range from mild to complex. Children with ARMs require preoperative imaging to assess the presence and location of a fistula. Identifying the location of these fistulas is crucial for surgical planning. Contrast-enhanced colosonography (ceCS) has emerged as a valuable tool in diagnosing pediatric ARMs, offering superior anatomical visualization and assessment compared to traditional imaging techniques like fluoroscopy, providing more sensitive and specific evaluation of fistula with real-time contrast filling. CeCS avoids ionizing radiation, and ultrasound contrast agents have a high safety profile with low incidence of adverse events compared to other contrast agents. Contrast-Enhanced colosonography (CeCS) technique should include survey greyscale ultrasound examination of the pelvis to assess for adequate window. Lumason, the ultrasound contrast agent, is prepared and injected into a bag of saline, with a ratio of 1:500 mL. The mucous fistula is examined, and a small Foley catheter is inserted by the radiologist, then balloon is inflated to create a seal. Contrast is then instilled by gravity drip, and perineal structures are imaged through three standard approaches- transabdominal, transperineal and posterior sagittal approaches. As well as reviewing the topics above, this review will also provide examples of ARM cases demonstrating high, intermediate, and low fistulas seen on CeCS. Overall, CeCS enhances the diagnostic accuracy and management of pediatric ARMs. By providing detailed anatomical insights and allowing for accurate classification, it plays a critical role in guiding treatment strategies and improving long-term outcomes for affected patients. Read More

Meeting name: SPR 2026 Annual Meeting , 2026

Authors: Xu Hongmin, Gagnon Marie-helene, Ali Sumera, Linam Leann

Keywords: Anorectal Malformation, Contrast Enhanced Ultrasound, Pre-Surgical Planning

Germ cell tumors of childhood are most often gonadal in origin. Extragonadal germ cell tumors are located characteristically in the midline arising intracranially, in the mediastinum, pelvis or retroperitoneum. These tumors are generally easily diagnosed due to typical sites of origin and characteristic imaging findings. However, germ cell tumors can be associated with unusual clinical syndromes or imaging features that can perplex the clinician/radiologist. We will review and illustrate atypical imaging/clinical manifestations and complications of germ cell tumors in childhood, based on our experience at two large children’s hospitals. These atypical findings include: Atypical metastatic disease: a) Burned out tumor – Testicular primary not typically evident with viable metastatic disease in retroperitoneal lymph nodes distant viscera, b) Growing teratoma syndrome (increasing size of tumor/pseudoprogression despite appropriate therapy), c) gliomatosis peritonei (maturation into glial tissue during therapy with pseudoprogression on FDG-PET), d) ossified pulmonary metastasis mimicking granulomas Antibody mediated paraneoplastic syndromes: a) anti–N-methyl-D-aspartate (NMDA)– receptor antibody–mediated encephalitis associated with ovarian teratomas, b) anti-Ma2 antibody-mediated encephalitis associated with testicular germ cell tumors Endocrine manifestations: a) Precocious puberty/gynecomastia due to hormonal (hCG) production, b) hyperthyroidism (TSH stimulation and struma ovarii) Local complications: a) ovarian torsion (common), b) ruptured teratoma or dermoid cyst (uncommon) Unusual primary tumors: a) Multifocal primary (e.g. pineal and suprasellar germinoma), b) Currarino triad, c) fetus-in-fetu, d) malignant transformation Read More

Meeting name: SPR 2022 Annual Meeting & Postgraduate Course , 2022

Authors: Gagnon Marie-helene, Parikh Ashish, Taylor Susan, Derenoncourt Paul-robert, Ponisio Maria, Khanna Geetika

Keywords: growing teratoma, gliomatosis peritonei, NMDA encephalitis

Pediatric stomach tumors encompass a broad range of pathologies. These tumors are rare in the pediatric and adolescent population and can be easily misdiagnosed. In addition, approach to imaging diagnosis in this population may differ from adults. In this educational exhibit, we will focus on stomach tumors. This exhibit will provide viewers with: 1. A visual differential diagnosis of common and uncommon tumors in each section of the stomach with a focus on multi-modality imaging. 2. Review of common imaging findings for each tumor, highlighting high-yield characteristic findings of each. 3. Key findings and tips that can help the practicing radiologist differentiated between benign and malignant tumors. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Mulvey Tom, Gagnon Marie-helene, Richer Edward, Loewen Jonathan, Alazraki Adina, Khanna Geetika, Mitchell Sarah, Riedesel Erica

Keywords: Stomach, Tumor, Malignancy