Lankester Evelyn,  Graeber Brendon

Final Pr. ID: Poster #: EDU-038

Chronic non-infectious osteomyelitis (CNO) is an autoinflammatory disorder of unknown etiology that is characterized by sterile, painful, and destructive bone lesions and has its peak incidence in childhood. Its presentation can be highly variable, and when children present with solitary lesions having an indolent course, the clinical picture and imaging features at presentation can overlap with those of Ewing sarcoma (EWS), a rare, highly malignant, primary pediatric bone tumor with a poor prognosis. Osteomyelitis is a known imaging mimic of EWS, but CNO poses a particular problem because a lack of clinical markers usually indicative of infection can cause the disease to be mistaken for a malignancy at clinical workup as well as imaging. A misdiagnosis between these two diseases may have serious repercussions for treatment and clinical course with potentially devastating effects on patient outcomes. Read More

Authors:  Lankester Evelyn , Graeber Brendon

Keywords:  CNO, Ewing´s Sarcoma

Kryger Marc,  Hernandez Alejandra,  Herring Katye,  Riegler Lara,  Hook Marcus,  Daugherty Reza

Final Pr. ID: Poster #: CR-028

A 16 year old boy reporting several months of hip pain, weight-loss, and fatigue presented to our institution after a pelvis radiograph ordered by his family physician showed concerning findings. MR imaging of his pelvis demonstrated a large heterogeneously enhancing mass arising from the right iliac wing causing significant mass effect on the pelvic organs and soft tissues of the buttocks. A percutaneous biopsy confirmed the diagnosis of Ewing sarcoma.
A staging CT of the chest was performed, demonstrating multiple lung metastases. In addition to this finding, there was multifocal bilateral nodular tumor thrombus propagating along multiple subsegmental pulmonary arteries, with a pseudo tree-in-bud morphology. Given their course, these metastatic deposits appeared endovascular in origin. Read More

Authors:  Kryger Marc , Hernandez Alejandra , Herring Katye , Riegler Lara , Hook Marcus , Daugherty Reza

Keywords:  Ewing, Sarcoma, Metastasis

Kazelka Maryam,  Shao Lei,  Mcgraw Marty,  Neville Kucera Jennifer

Final Pr. ID: Poster #: CR-016

Spindle cell sarcomas are a rare and aggressive type of soft-tissue sarcoma. Although rhabdomyosarcomas are the most common childhood sarcoma, the spindle cell type is incredibly rare. A 25-year-old healthy G2P1001 female presents at 34 weeks gestational age with fetal growth restriction of a male fetus. Prenatal ultrasound showed a complex vascular mass exophytic to the distal spine, with a presumed diagnosis of sacrococcygeal teratoma (SCT). For further characterization, a fetal MRI was performed at 35 weeks gestation, which showed a solid appearing mass exophytic to the pelvis and involving the proximal thigh. Many features of the tumor were atypical for sacrococcygeal teratoma: the mass being more homogenous than expected, lacking cystic components that would normally been seen in a SCT, and involving the thigh but without definite involvement of the spine. It was also noted that the normal T1 meconium signal in the rectum was displaced posteriorly by the mass. At that time, an alternative differential diagnosis was suggested: sarcoma including fibrosarcoma or rhabdomyosarcoma, myofibroma, or less likely atypical sacrococcygeal teratoma. The baby was born via C-section at 37 weeks and 1 day gestation. A postnatal MRI showed the mass exophytic to the pelvis with some intrapelvic extension via the inguinal canal. Lack of spine involvement was confirmed. Signal characteristics of the tumor were similar to that of skeletal muscle, with a few focal areas of internal hemorrhage. The tumor had mass effect on multiple structures: deviating and tethering of the rectum to the right and stretching it inferiorly; displacing the anus posteriorly; uplifting the bladder; and abutting the base of the penis with no clear fat planes. Surgical resection was performed when the baby was 6 days old. Pathology evaluation revealed a sarcoma that was diffusely infiltrative with NTRK reactivity by immunohistochemistry. Molecular studies by next-generation sequencing reported TPM3-NTRK1 fusion in the tumor. Pathology was most suggestive of a spindle-cell sarcoma. It is important for the pediatric radiologist to be familiar with fetal soft tissue tumors to help facilitate family counseling and delivery planning. The radiologist should understand imaging features that may help distinguish more rare soft tissue tumors from sacrococcygeal teratomas. Although an exact diagnosis may not be possible prenatally, a multidisciplinary approach ensures the best possible patient outcomes. Read More

Authors:  Kazelka Maryam , Shao Lei , Mcgraw Marty , Neville Kucera Jennifer

Keywords:  Sarcomas, Fetal Magnetic Resonance Imaging, Soft Tissue Sarcoma

Yadava Rohtas

Final Pr. ID: Poster #: SCI-016

To see the profile of various Pediatric Abdominal Mass Lesions on MDCT and to evaluate the diagnostic accuracy of MDCT in detection and characterization of various Pediatric Abdominal Mass Lesions. Read More

Authors:  Yadava Rohtas

Keywords:  Hepatoblastoma, Ewing Sarcoma, Adrenal Pheochromocytoma

Shaikh Sikandar

Final Pr. ID: Poster #: SCI-025

The purpose of this study was to compare the diagnostic performance of 18F-FDG PET/CT and conventional imaging for staging and follow-up of pediatric osteosarcoma and skeletal Ewing sarcoma. Read More

Authors:  Shaikh Sikandar

Keywords:  Sarcoma

Shah Avanthi,  Alizadeh Ash,  Sweet-cordero Alejandro,  Wittber Frederick,  Azad Tej,  Chabon Jake,  Leung Stan,  Spillinger Aviv,  Liu Heng-yi,  Breese Marcus,  Diehn Maxamilian

Final Pr. ID: Poster #: SCI-061

Currently, biopsy serves as the gold standard to accurately diagnose disease in pediatric sarcoma patients, but the risks of anesthesia and surgery, along with a failure to characterize the true heterogeneity of disease make this method less than ideal. Progression and response to therapy are monitored by radiologic exams which lack the sensitivity to detect early relapse. Cell free DNA (cfDNA) is released into the plasma as they undergo apoptosis and necrosis. ctDNA represents a small fraction of cfDNA in cancer patients and contains tumor specific alterations. It holds promise as a highly sensitive and specific biomarker. A limitation in applying liquid biopsy in clinical practice is the need to develop PCR or other DNA analysis methods to detect alterations specific to a single patient. We have developed a more widely applicable off the shelf test that does not involve a patient specific design. Read More

Authors:  Shah Avanthi , Alizadeh Ash , Sweet-cordero Alejandro , Wittber Frederick , Azad Tej , Chabon Jake , Leung Stan , Spillinger Aviv , Liu Heng-yi , Breese Marcus , Diehn Maxamilian

Keywords:  sarcoma, ctdna, cappseq

Carlock Hunter,  Kelly Anna,  Chess Mitchell

Final Pr. ID: Poster #: EDU-033

Undifferentiated embryonal sarcomas of the liver (UESL) and mesenchymal hamartomas are rare pediatric tumors. Mesenchymal hamartomas are benign and often seen in children under two years of age, while UESL are malignant tumors often seen in children six to ten years of age; however, age ranges are not universally true. There is an unclear relationship between these two tumors. Prior studies have hypothesized that mesenchymal hamartomas may evolve into UESL given reports of UESL within portions of mesenchymal hamartomas; however, few cases of this exist in the literature. There is a common relationship of 19q13.4 chromosomal alterations between these two lesions, the MHLB1 gene locus.

These tumors are difficult to differentiate on imaging alone, and biopsy is often required for diagnosis. Shared imaging features between these two tumors include large size, cystic with solid components, patchy enhancement, and occasional fluid-fluid levels. UESL occasionally present with poor margins and are fast growing (mesenchymal hamartomas are typically well-defined). These features are not universally consistent, which can make diagnosis difficult.

In this educational exhibit, we present three cases highlighting the clinical presentation, imaging features, treatments, and outcomes of patients with UESL and mesenchymal hamartomas. We showcase multi-modal imaging appearances of these lesions (ultrasound, CT, MRI, angiography) and their post-treatment changes. In particular, one case describes a 5-year-old female presenting with a hemorrhaging UESL which was subsequently resected. Follow-up imaging showed a new hepatic lesion concerning for recurrence. Histopathology of this new lesion showed findings consistent with mesenchymal hamartoma without evidence of UESL recurrence or other malignancy. Through these cases, we highlight similarities and differences between mesenchymal hamartomas and UESL, hoping to further explore a potential association between these two tumors. Read More

Authors:  Carlock Hunter , Kelly Anna , Chess Mitchell

Keywords:  Sarcomas, Liver, Oncology

Cross Nathan,  Stanescu A. Luana,  Rudzinski Erin,  Hawkins Doug,  Parisi Marguerite

Final Pr. ID: Poster #: CR-057

Ewing sarcoma is the second most common malignancy of bone with an incidence of approximately 200 cases per year in the United States in children under 20. Extraosseous Ewing sarcoma originating in the vagina is rare, with only 8 cases reported to date in the literature. These highly aggressive tumors require a more intensive treatment regimen compared to other vaginal masses like rhabdomyosarcoma. While the imaging findings are non-specific, awareness of this clinical entity can facilitate early diagnosis and appropriate treatment, which may improve prognosis. Read More

Authors:  Cross Nathan , Stanescu A. Luana , Rudzinski Erin , Hawkins Doug , Parisi Marguerite

Keywords:  Ewing, sarcoma, vaginal, teenagers