Use of PET/CT with Cosyntropin Stimulation to Identify and Localize Adrenal Rest Tissue following Adrenalectomy in a Woman with Congenital Adrenal Hyperplasia

PET/CT with (18)F-fluorodeoxyglucose (FDG) or using different radiocompounds has proven accuracy for detection of adrenal metastases in patients undergoing cancer staging. It can assist the diagnostic work-up in oncology patients by identifying distant metastases to the adrenal(s) and defining oligometastatic disease that may benefit from targeted intervention. In patients with incidentally discovered adrenal nodules, so-called adrenal "incidentaloma" FDG PET/CT is emerging as a useful test to distinguish benign from malignant etiology. Current published evidence suggests a role for FDG PET/CT in assessing the malignant potential of an adrenal lesion that has been 'indeterminately' categorized with unenhanced CT, adrenal protocol contrast-enhanced CT, or chemical-shift MRI. FDG PET/CT could be used to stratify patients with higher risk of malignancy for surgical intervention, while recommending surveillance for adrenal masses with low malignant potential. There are caveats for interpretation of the metabolic activity of an adrenal nodule on PET/CT that may lead to false-positive and false-negative interpretation. Adrenal lesions represent a wide spectrum of etiologies, and the typical appearances on PET/CT are still being described, therefore our goal was to summarize the current diagnostic strategies for evaluation of adrenal lesions and present metabolic and anatomic appearances of common and uncommon adrenal lesions. In spite of the emerging role of PET/CT to differentiate benign from malignant adrenal mass, especially in difficult cases, it should be emphasized that PET/CT is not needed for most patients and that many diagnostic problems can be resolved by CT and/or MR imaging.

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“…The FDG PET/CT is performed after ACTH stimulation which leads to increased visibility of FDG uptake in adrenal rests [157,158].…”

Section: Adrenocortical Adenomamentioning

confidence: 99%

Metabolic and anatomic characteristics of benign and malignant adrenal masses on positron emission tomography/computed tomography: a review of literature

et al. 2014

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“…Lila et al [24 ]reported the use of 18 F-FDG PET/CT in the detection of ectopic adrenal rests in a patient who developed Nelson's syndrome after bilateral adrenalectomy for Cushing's disease. Crocker et al [25] described a similar case of a young adult female patient with CAH with hyperandrogenism after bilateral adrenalectomy. They detected adrenal rests in both ovaries by using 18 F-FDG PET/CT after cosyntropin stimulation.…”

Section: Discussionmentioning

confidence: 99%

Ectopic Adrenal Rests in Congenital Adrenal Hyperplasia as a Cause of Androgen Excess after Adrenalectomy Detected by Pelvic Venous Sampling

Grinten¹,

Stikkelbroeck

Bulten

et al. 2013

Horm Res Paediatr

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Background: Patients with classic congenital adrenal hyperplasia (CAH) due to CYP21 deficiency are treated with supraphysiological doses of glucocorticoids to suppress elevated androgen production. This implies also side effects of high-dose glucocorticoids, possibly leading to iatrogenic Cushing's syndrome. Bilateral adrenalectomy has been suggested as the ultimate therapy in severe cases, when insufficient androgen suppression was obtained despite high glucocorticoid doses. Usually, ACTH levels rise after bilateral adrenalectomy, and this could imply an increased risk for the development of ectopic adrenal rests. In female CAH patients ovarian adrenal rests are not commonly detected by conventional radiological techniques. Methods: We report the case of an adult female CAH patient who underwent bilateral adrenalectomy in early puberty because of poorly controlled CAH. Results and Conclusions: Several years after surgery, she developed secondary amenorrhea and hair loss as a result of androgen overproduction in ovarian adrenal rests that appeared to be detectable only by pelvic venous sampling. After unilateral oophorectomy androgen levels normalized.

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“…The most radical treatment is the surgical removal of both adrenal glands. This approach must be carefully discussed as it can induce the development of TARTs in men and less frequently retroperitoneal adrenal rest tumors in women (73,74). Chemical adrenalectomy may be obtained using mitotane, an adrenolytic agent for which the mechanisms of action remain poorly understood.…”

Section: Treatment Of Classic Cah Adultsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

Bachelot

Grouthier

Courtillot

et al. 2017

European Journal of Endocrinology

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Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and in some cases aldosterone deficiency associated with androgen excess. Goals of treatment are to replace deficient hormones and control androgen excess, while avoiding the adverse effects of exogenous glucocorticoid. Over the last 5 years, cohorts of adults with CAH due to 21-hydroxylase deficiency from Europe and the United States have been described, allowing us to have a better knowledge of long-term complications of the disease and its treatment. Patients with CAH have increased mortality, morbidity and risk for infertility and metabolic disorders. These comorbidities are due in part to the drawbacks of the currently available glucocorticoid therapy. Consequently, novel therapies are being developed and studied in an attempt to improve patient outcomes. New management strategies in the care of pregnancies at risk for congenital adrenal hyperplasia using fetal sex determination and dexamethasone have also been described, but remain a subject of debate. We focused the present overview on the data published in the last 5 years, concentrating on studies dealing with cardiovascular risk, fertility, treatment and prenatal management in adults with classic CAH to provide the reader with an updated review on this rapidly evolving field of knowledge.

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Use of PET/CT with Cosyntropin Stimulation to Identify and Localize Adrenal Rest Tissue following Adrenalectomy in a Woman with Congenital Adrenal Hyperplasia

Abstract: Patients with CAH after an adrenalectomy may experience recurrent hyperandrogenism due to adrenal rest tissue. 18F-FDG PET/CT with cosyntropin stimulation accurately identified adrenal rest tissue not visualized with conventional imaging, allowing for successful surgical resection.

Cited by 45 publications

References 17 publications

Metabolic and anatomic characteristics of benign and malignant adrenal masses on positron emission tomography/computed tomography: a review of literature

Metabolic and anatomic characteristics of benign and malignant adrenal masses on positron emission tomography/computed tomography: a review of literature

Ectopic Adrenal Rests in Congenital Adrenal Hyperplasia as a Cause of Androgen Excess after Adrenalectomy Detected by Pelvic Venous Sampling

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

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