Coleman Jay,  Collard Michael,  Pfeifer Cory

Final Pr. ID: Poster #: EDU-087

Molecular biology has come to the forefront of modern oncology. Knowledge of specific genetic mutations within tumors drives prognostic information and can guide therapy. Keeping up with new terminology in oncology can be difficult for pediatric radiologists who often host oncology conferences and present regularly at grand rounds. The purpose of this educational exhibit is to describe DICER1 syndrome and discuss its implications for pediatric radiology. Read More

Authors:  Coleman Jay , Collard Michael , Pfeifer Cory

Keywords:  cystic nephroma, DICER1, pleuropulmonary blastoma

Tijerin Bueno Marta,  De La Puente Gregorio Alejandro,  Martinez-rios Claudia,  Malkin David,  Greer Mary-louise

Final Pr. ID: Poster #: SCI-045

To determine the spectrum of abnormalities associated with DICER1 syndrome.
To review imaging studies performed in oncologic staging and surveillance.
To delineate the screening protocol used in those with positive genetic testing. Read More

Authors:  Tijerin Bueno Marta , De La Puente Gregorio Alejandro , Martinez-rios Claudia , Malkin David , Greer Mary-louise

Keywords:  DICER1, Pleuropulmonary blastoma, pineoblastoma, cystic nephroma

Shewarega Annabella,  Lin Tony,  Christison-lagay Emily,  Stitelman David,  Hittelman Adam,  Morotti Raffaella,  Silva Cicero,  Rowe Erin

Final Pr. ID: Poster #: CR-018

Multicystic renal tumors (MCRTs) in children encompass a rare and diverse group of neoplastic entities, including cystic nephroma (CN), cystic partially differentiated nephroblastoma (CPDN), and cystic Wilms' tumor (CWT). CPDN occupies an intermediate status between the benign CN and the malignant CWT. Despite marked histological differences, distinguishing these tumors clinically is a complex undertaking.
A 33-month-old male child presented with abdominal pain and recurrent episodes of screaming and knee-drawn postures during sleep. The patient had a recent history of mild gastroenteritis, but no recent travel or sick contacts. Physical examination revealed a palpable abdominal mass. Abdominal ultrasound detected a distinct multicystic lesion in the upper left quadrant, separated from the spleen. While gastric involvement was considered, the lesion appeared isolated from the gastric bubble and fixed. Potential diagnoses included a unique gastric bezoar, vascular mass lesions, and an exophytic renal lesion. MRI revealed a large, multiloculated cystic lesion with septations, primarily contained within the renal boundaries, but protruding medially. Noticeable enlargement of adjacent retroperitoneal lymph nodes prompted consideration of an MCRT, including CWT. An atypical multicystic dysplastic kidney was also considered. The patient underwent surgical resection followed by histopathological analysis, which confirmed the diagnosis of CN.
While radiological imaging aids in diagnosing MCRT, distinguishing between CN, CPDN, and CWT often necessitates histopathological analysis. Vascular invasion can point to a diagnosis of CWT. The presence of solid elements is less helpful, as discerning whether these are part of the tumor or part of the native kidney can be difficult. The accuracy of the diagnosis plays a crucial role in determining the necessity for chemotherapy and/or radiation therapy. This case report highlights the diagnostic complexities associated with MCRTs in pediatric patients and underscores the significance of a multidisciplinary approach that incorporates clinical, radiological, and histopathological evaluations to provide the best possible care for these young patients. Read More

Authors:  Shewarega Annabella , Lin Tony , Christison-lagay Emily , Stitelman David , Hittelman Adam , Morotti Raffaella , Silva Cicero , Rowe Erin

Keywords:  Cystic Nephroma, Abdominal, Tumor