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Clinical Picture

Cushings conundrum: an unusual case of primary


pigmented nodular adrenal disease in a 60-year-old woman
Jasna Aleksova, Glenn Ward, Kim Taubman, Jason Tan, Stephen Farrell, Stuart Galloway, Richard J MacIsaac, Nirupa Sachithanandan

Lancet Diabetes Endocrinol 2016; A 60-year-old woman with resistant hypertension, macroscopic and histological appearance was consistent
4: 630 poorly controlled type 2 diabetes, and chronic obstructive with primary pigmented nodular adrenal hyperplasia
Published Online pulmonary disease requiring inhaled corticosteroids (also known as primary pigmented nodular adrenal
October 30, 2015
was referred for investigation of Cushings syndrome. disease [PPNAD]; gure C, D).
http://dx.doi.org/10.1016/
S2213-8587(15)00262-4 She had a BMI of 31 kg/m, abdominal obesity, and PPNAD is associated with Carneys complex in 90% of
Departments of Endocrinology
proximal muscle wasting. Investigations showed a white patients. Carneys complex is an autosomal
and Diabetes (J Aleksova MBBS, normal aldosterone-to-renin ratio, plasma meta- dominant multiple neoplasia syndrome, typically pre-
G Ward, Prof R J Maclsaac FRACP, nephrines, and renal artery Doppler ultrasound senting with the classic triad of myxomas, spotty skin
N Sachithanandan FRACP),
ndings, but increased 24 h urine free cortisol pigmentation, and endocrine overactivity. 60% of
Nuclear Medicine
(K Taubman FRACP), Surgery concentration (454 nmol/L [normal range <350 nmol/L]). Carneys complex in the white population is associated
(J Tan FRACS, S Farrell FRACS), Adrenocorticotropic hormone remained undetectable with germline-inactivating mutations in regulatory
and Pathology on several occasions for 12 months, during the subunit 1 of protein kinase A (PRKAR1A).
(S Galloway FRACPA),
diagnostic workup (<5 pg/mL). Because of diculty in To our knowledge, this patient is the oldest reported
St Vincents Hospital
Melbourne Fitzroy, Melbourne, ceasing use of inhaled corticosteroids, an overnight person to be diagnosed with PPNADmost patients
Australia 1 mg dexamethasone suppression test was done while are diagnosed before age 40 years. She has no other
Correspondence to: the patient was taking uticasone, showing an 8 am manifestations of Carneys complex and is currently
Dr Jasna Aleksova, Department cortisol concentration of 278 nmol/L (compared with a undergoing genetic testing for mutations in the
of Endocrinology and Diabetes,
normal range of <120 nmol/L). Persistent hyper- PRKAR1A gene. This case shows that PPNAD can be
St Vincents Hospital Melbourne,
Fitzroy, Melbourne, VIC 3065, cortisolism was then conrmed with intravenous latent for many years and should be considered in
Australia dexamethasone suppression testing done after inhaled dierential diagnosis of adrenocorticotropic hormone-
jasnaaleksova@hotmail.com uticasone was withheld for several days. An adrenal independent Cushings syndrome in elderly
CT scan showed an enlarged left adrenal gland, with an individuals.
8 mm nodule at the inferior pole (gure A), insucient Contributors
to account for the patients hypercortisolism. Functional JA searched the literature; collected, analysed, and interpreted data; and
imaging with iodocholesterol under dexamethasone contributed to the writing of the report. GW collected and interpreted
data. KT did the imaging. JT and SF analysed data and did the surgical
suppression showed bilateral uptake of labelled procedure. RM and NS collected and interpreted data and contributed to
cholesterol (gure B). Adrenal vein sampling under the writing of the report. SG reviewed the histopathology.
dexamethasone suppression was consistent with Declaration of interests
See Online for appendix bilateral autonomous cortisol secretion (appendix). The We declare no competing interests.
patient underwent a bilateral adrenalectomy. The

A B C D
x

Figure: Imaging and histopathological ndings


(A) Adrenal CT scan: the arrow shows an 8 mm nodule at the left inferior pole (Hounseeld units <5). (B) Iodocholesterol scan: bilateral uptake of norcholesterol, despite dexamethasone suppression
and non-functioning left adrenal nodule. (C) Macroscopic appearance: bulky adrenals, with multiple pigmented nodules of less than 1 cm. (D) Microscopic appearance: normal cortex (x) and adjacent
pigmented nodule (y), with presence of melanin staining within the cytoplasm of the nodule cells (20 magnication).

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