PMC6302904_01
Metamorphosis of twin sisters over time with corresponding biological and imaging findings. (a, f, h) Photographs of patient A (left) and patient B (right) over time. Pictures were taken at ~18-month intervals: (a) at initial evaluation of patient A; (f) when patient B started to complain about weight gain; (h) ~36 months after adrenalectomy for patient A and 18 months for patient B. (b) Abolition of normal circadian cortisol rhythm with a constant ACTH suppression in patient A at presentation [at time of photograph in (a)]. (c) Results of a suppression test during which 8 mg of DXM was given overnight to patient A at the time of the photograph in (a): Plasma cortisol increased paradoxically from 101 ng/mL to 178 ng/mL. (d) A fusion image from 131I iodomethyl norcholesterol scintigraphy coupled with SPECT/CT scanner in patient A at the time of the photograph in (a). Scintigraphy was performed after ACTH suppression by DXM and revealed a bilateral uptake with a slight left predominance. (e) The left adrenal gland of patient A. Numerous pigmented, 1- to 4-mm nodules are visible. (g) Graph shows the restoration of circadian cortisol rhythm after left-side adrenalectomy in patient A. (i, j) Evolution of both patients' body weight over time. Time 0 corresponds to time of adrenalectomy. DXM, dexamethasone.
js.2018-00261f1.jpg
PMC6302904_01_js.2018-00261f1.jpg
F1
We report the case of 28-year-old twin female patients [patients A and B; Fig. 1(a), 1(f), and 1(h)]. ACTH suppression [Fig. 1(b)]. An 8-mg overnight dexamethasone suppression test showed a paradoxical response with plasma cortisol level rising [Fig. 1(c)]. Adrenal CT scan and MRI were both normal. 131I iodomethyl norcholesterol scintigraphy coupled with single-photon emission computed tomography (SPECT)/CT showed bilateral adrenal uptake with a slight left predominance [Fig. 1(d)]. Histology confirmed the presence of numerous cortical adrenal pigmented nodules of 1 to 4 mm in the adrenal gland [Fig. 1(e)]. One year after surgery, patient A had normal UFC level (41 microg/24 hours), regained her initial normal body weight (67 kg), and her cortisol and ACTH circadian rhythm were almost restored [Fig. 1(g)]. Patient B had normal UFC level (65 microg/24 hours)18 months after surgery, and had regained her initial normal 68-kg body weight [Fig. 1(j)]. At that time, 3 years after her surgery, patient A still had normal body weight (62 kg) and normal UFC level (51 microg/24 hours), showing that our rather conservative unilateral adrenalectomy approach had a favorable clinical outcome with longstanding remission of symptoms and uncompromised quality of life without adrenal insufficiency or hydrocortisone supplementation [Fig. 1(i)].