Bowman Sean,  Trinh Austin,  Tuburan Smyrna

Final Pr. ID: Poster #: CR-070

A 4 day old female, born at term without complications and with reported proper prenatal care was referred to a tertiary care center after her parents were notified of an abnormal 17-OHP on newborn screening. Work-up for suspected congenital adrenal hyperplasia was initiated. An abdominal ultrasound showed a 2.8 x 3.6 x 3.7 cm heterogeneously hypoechoic right suprarenal mass with peripheral calcification and large feeding vessels. An MRI abdomen was then obtained, which showed a round circumscribed 3.6 x 3.5 x 3.4 cm T1 hypointense and T2 iso-hyperintense heterogeneously enhancing mass. The radiologist raised the suspicion for an adrenocortical tumor, with neuroblastoma or teratoma as less likely etiologies. An MIBG scan was then obtained, revealing absence of activity in the region of the known mass, further supporting the diagnosis of an adrenocortical neoplasm. This information, in addition to normal levels of VMA and HVA, reduced the likelihood that the mass was of neural crest origin. IR then performed an ultrasound guided biopsy of the right adrenal mass. Pathology reported features consistent with an adrenal cortical neoplasm and without features of neuroblastoma. Due to concern for potential aggressive behavior, pathology recommended complete surgical excision. There is a paucity of literature secondary to the extremely low incidence of congenital adrenal cortical neoplasms. Therefore, a multidisciplinary committee, which included pediatric surgery and radiology, was held. The consensus was that the mass would be best treated by, and is amenable to, surgical excision. At 28 days old, the patient underwent an open right adrenalectomy. Pathology confirmed an adrenal cortical neoplasm of uncertain malignant potential. The post-operative course was without complications. Per pediatric hematology-oncology recommendations, a nuclear medicine whole body bone scan was obtained and demonstrated no osteoblastic metastasis. The patient was discharged and will continue to follow up with hematology-oncology and endocrinology as an outpatient. As illustrated above, the early and correct identification of a potentially aggressive neoplasm by radiology accelerated the treatment course, allowing for potentially curative interventions. This case demonstrates the necessity for multidisciplinary management to include both diagnostic and interventional radiologists and to communicate an appropriate range of differential diagnoses, regardless of incidence. Read More

Authors:  Bowman Sean , Trinh Austin , Tuburan Smyrna

Keywords:  Adrenal, Tumor, adrenal cortical neoplasm

Goncalves Luis,  Jamshidi Ramin,  Biyyam Deepa,  Patel Mittun,  Cornejo Patricia

Final Pr. ID: Poster #: SCI-006

The purpose of this case report is to alert the reader to a rare differential diagnosis for infra- as well as intradiaphragmatic lung sequestration. A 38-year-old G2P0010 pregnant patient was referred for fetal magnetic resonance imaging (MRI) due to a 14.2 x 20.2 x 18 mm left paraspinal hyperechogenic mass with no internal vascularity and no convincing systemic arterial feeding vessel concerning for neuroblastoma as seen by ultrasound (US) performed at 34 weeks. Fetal MRI performed the same week showed a homogeneous T2-hyperintense left paraspinal mass in close apposition to and associated with a small area of loss of continuity in the diaphragm. This has been previously reported by postnatal computerized tomography as the "split diaphragm" sign in a case of intradiaphragmatic lung sequestration (Meier AH, Eggli KD, Cillei RE. Intradiaphragmatic extralobar sequestration: a rare pulmonary anomaly. Pediatr Surg 200;44:e27-29). Thus, the differential diagnosis provided at the time was congenital intradiaphragmatic sequestration and neuroblastoma. The fetus delivered via uncomplicated spontaneous vaginal delivery at term. Postnatal US performed at the age of 15 days showed a left hyperechogenic mass extending from the left lower chest to the ipsilateral retroperitoneum through a small defect in the diaphragm, favored to represent an extrapulmonary lung sequestration. The left adrenal gland was normal. A follow-up CT performed at 7 months of age showed a 2.6 cm left paraspinal mass with no systemic arterial blood supply to support the diagnosis of sequestration. The differential diagnosis at the time included neurogenic tumor or a myofibroma arising from the diaphragm. Follow-up CT at 13 months of age showed similar findings. The patient underwent uneventful laparoscopic removal of the mass with a final pathological diagnosis of congenital adrenal rest. Congenital adrenal rest presenting as a diaphragmatic mass is rare and as been reported once in an adult patient with an adenoma in heterotopic adrenal tissue located in the left diaphragm, diganosed because of mass effect in the gastric fundus during an uper gastrointestinal series (Keirns MM. Two unusual tumors of the diaphragm. Radiology 1952; 52:542-547). We hope this report raises awareness of this entity as a potential differential diagnosis for prenatal masses seen in close relationship with the adrenal gland and/or diaphragm. Read More

Authors:  Goncalves Luis , Jamshidi Ramin , Biyyam Deepa , Patel Mittun , Cornejo Patricia

Keywords:  lung sequestration, neuroblastoma, adrenal rest

Aslan Mine,  Kalyoncu Ucar Ayse,  Arioz Habibi Hatice,  Namdar Yesim,  Kurugoglu Sebuh,  Adaletli Ibrahim

Final Pr. ID: Poster #: CR-027

Wolman disease is a rare autosomal recessive inherited disease characterized by storage of cholesterol esters and triglycerides in lysosomes due to a deficiency of lysosomal acid lipase. Clinical signs such as persistent vomiting, diarrhea, hepatosplenomegaly, growth retardation and liver dysfunction occur in the first weeks of life. Most cases die in the first year of life. Here, we aim to present radiological findings of Wolman disease.
An asymptomatic girl two months of age was admitted to the pediatric genetic disease service with the history of Wolman disease in two brothers who had died in the neonatal period. Abdominal X-ray imaging displayed calcification of the bilateral adrenal gland regions. Hepatosplenomegaly and bilateral adrenal gland posterior acoustic shadowing due to calcifications were revealed by abdominal sonography. Magnetic resonance imaging showed enlarged hypointense adrenal glands in all sequences and hepatosplenomegaly. The lysosomal acid lipase levels were low compatible with Wolman disease.
Multimodality radiologic imaging methods should be performed to display hepatosplenomegaly, hepatosteatosis, bilateral adrenal gland enlargement and calcification in Wolman disease. Read More

Authors:  Aslan Mine , Kalyoncu Ucar Ayse , Arioz Habibi Hatice , Namdar Yesim , Kurugoglu Sebuh , Adaletli Ibrahim

Keywords:  Wolman Disease, Sonography, Adrenal gland, calcification, magnetic resonance imaging

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

Willard Scott,  Birkemeier Krista

Final Pr. ID: Poster #: CR-029

We present the case of a 14 year old Asian male with a history of poorly controlled congenital adrenal hyperplasia (CAH) and growing testicular adrenal rest tumors. While on high dose steroid therapy, the lesions decreased in size from 1.1 x 1.0 cm to 0.5 x 0.8 cm. However, they subsequently increased in size, measuring 2 to 3 cm in length when the patient was not compliant with the steroid regimen due to Cushingoid side effects. The masses were bilateral, symmetric, and located at the mediastinum testis, all findings consistent with testicular adrenal rest tumors as opposed to Leydig tumor. With growth, they coalesced to form undulating masses with hyperechoic and hypoechoic layers mimicking the cerebrum, an appearance more typically seen in the adrenal glands at initial diagnosis of CAH. Current practice guidelines in much of the developed world typically result in early diagnosis and advanced therapy, making testicular adrenal rest tumors of this size and appearance unique. Read More

Authors:  Willard Scott , Birkemeier Krista

Keywords:  congenital adrenal hyperplasia, CAH, testicular adrenal rest tumor, TART

Takahashi Marcelo,  Moreira Marcia,  Motta Giovanna,  Gual Fabiana,  Soares Juliana,  Davaus Taisa

Final Pr. ID: Poster #: EDU-039

Girls with congenital adrenal hyperplasia can present with a wide variety of urogenital malformations. Understanding these malformations and the anatomical relations between the pelvic structures is paramount for surgical planning. Imaging of these patients can be done by various methods such as genitography, magnetic resonance or endoscopy.
More recently some authors have begun to propose perineal ultrasound as an alternative for imaging of these patients for pre-operative planning.
In this educational poster we will use perineal ultrasound images and detailed illustrations to:
1) Review the spectrum of genital and lower urinary tract malformations related to congenital adrenal hyperplasia in girls.
2) Explain the transperineal ultrasound technique and normal anatomy.
3) Explain the transperineal ultrasound findings and utility in girls with congenital adrenal hyperplasia.

Contents:
Normal anatomy
Pathologic changes in congenital adrenal hyperplasia
Review of what is relevant to the referring surgeon.
Ultrasound technique
Ultrasound findings in normal patients
Ultrasound findings in congenital adrenal hyperplasia. Read More

Authors:  Takahashi Marcelo , Moreira Marcia , Motta Giovanna , Gual Fabiana , Soares Juliana , Davaus Taisa

Keywords:  congenital adrenal hyperplasia, ultrasound, perineum