Browsing by Subject "Adrenocortical adenoma"

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    Imaging characteristics of pathologically proven adrenal adenomas with myelolipomatous degeneration: correlation with clinical and pathologic features
    (Oxford University Press, 2022) Guccione, Jeffrey; Soliman, Moataz; Zhang, Miao; Habra, Mouhammed Amir; Collins, Katrina; Zhao, Jianping; Elsayes, Khaled M.; Pathology and Laboratory Medicine, School of Medicine
    Objectives: Adrenal adenoma with myelolipomatous degeneration (AMD) is a rarely reported and often overlooked entity. The aim of this study is to improve understanding of these lesions by characterizing the imaging findings with pathologic and clinical correlation. Methods: In the largest series to date, we report 11 nodules in 11 patients confirmed with a pathologic diagnosis of AMD. The available cross-sectional imaging and histopathologic features were reviewed by two radiologists and two pathologists, respectively. Clinical and laboratory data for each patient were obtained from the electronic medical records, when available. Results: All 11 patients had a CT prior to resection or biopsy of the adrenal nodule, with five having received an adrenal mass protocol study. An MRI was available in three patients. The median size of the nodules on imaging was 4.5 cm (range 2.8-8.7) and all but one had macroscopic fat. The largest focus of macroscopic fat had a median size of 0.7 cm (range 0.2-1.6) and on average was 14.4% the size of the tumor, using greatest dimensions. Four (36.4%) patients had a diagnosis of Cushing syndrome prior to nodule resection. Conclusions: Not all adrenal nodules with macroscopic fat on imaging are pure myelolipomas. An AMD should be considered, especially if the foci of fat are small and other features of an adenoma are present. Some may also be associated with Cushing syndrome. Advances in knowledge: Myelolipomatous degeneration within an adrenal adenoma has only rarely been previously reported with very few reports emphasizing the imaging features. There may be an association with cortisol hypersecretion and improved recognition of this entity could lead to changes in clinical management.
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    SUN-401 New Right Adrenal Nodule and Beckwith-Wiedemann Syndromein a Girl with Left Adrenocortical Tumor Resection
    (Oxford University Press, 2019-04-15) Alradadi, Rasha; Sanchez, Juan; Pediatrics, School of Medicine
    Background: The adrenocortical tumors have been reported in patients with Beckwith-Wiedemann syndrome, but the optimal way of management is not well studied. We report a case of left adrenocortical adenoma status post left adrenalectomy who subsequently diagnosed with Beckwith-Wiedemann syndrome, thereafter found to have a growing right adrenal nodule. Clinical Case: A 9-year-old female who was initially seen in Endocrinology clinic when she was seven months old. At that time, she was referred for pubic hair that was preceded by acne around the age of 5 months. Screening labs showed elevated DHEA 137 mcg/dl and testosterone 161 ng/d. MRI revealed a 2.5 cm well defined rounded homogenously enhancing soft tissue mass located at the anteroinferior to the lateral limb of the left adrenal gland. She had left adrenalectomy at seven months of age. Pathology of the mass revealed adrenocortical adenoma. She was referred to hematology and oncology. Very mild hemi-hypertrophy started to appear as patient got older, so further work- up revealed loss of methylation of DNA at the DMR2 which is consistent with Beckwith Wiedemann Syndrome. All symptoms had resolved, and she had multiple screening including Ultrasounds and AFP. All her images and AFP remained normal. Then at the age of 5 years, she started to have new pubic hair and acne. CT scan revealed right adrenal thickening and nodularity with a 5 mm nodule. It has been monitored with frequent MRI alternated with ultrasounds. The nodule has increased in size gradually over the years to 15.79 mm x 8.72 mm. PET scan showed focal increased FDG activity within the 1.1 cm x 0.7 cm right adrenal nodule. Bone age was normal. After consultations of multiple national centers, there was no consistency about how to manage adrenal tumors in patient with BWS. Therefore parents were given choices of total right adrenalectomy vs partial adrenalectomy vs observations. Parent chose watching waiting, so decision was made to follow up with MRI every three months. Conclusions: Although patients with BWS get screened with abdominal U/S frequently for adrenal tumors as the part of the tumor surveillance, there is no consensus on management options if such tumors are detected.This case raises the need for uniform guidelines to outline the optimal approach of management of adrenocortical tumor in patient with BWS.