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A 42-year-old woman with ACTH-dependent Cushing’s syndrome, treated with ketoconazole,
normalized urinary free cortisol (UFC) from 433.0 to 66.0
μg/day, but failed to reduce elevated serum androgen levels (DHEAS 4770 ng/ml).
Magnetic resonance imaging (MRI) showed a 3-mm microadenoma attached to the
pituitary stalk. After one year, treatment was stopped and UFC rose again to
936.0 μg/day
but a month later the patient presented acute headache and signs of steroid
withdrawal syndrome. Endocrine evaluation showed glucocorticoid and androgen
deficiency (UFC 5.0 μg/day;
DHEAS < 300 ng/ml); control MRI revealed disappearance of the microadenoma.
Cushingoid signs subsided and steroid replacement was initiated, proving
still necessary over two years after the episode.
|
|
Before treatment |
Under KTZ |
Without KTZ |
After tumor disappearance
1
month 1 year 2 years |
|
UFC |
432.7 |
66.4 |
936.0 |
4.7 |
6.6 |
15.0 |
|
Basal Cortisol |
25.6 |
|
|
3.6 |
1.0 |
7.6 |
|
Post Dx 1mg |
25.0 |
|
|
|
|
|
|
Post Dx 8 mg |
13.5 |
|
|
|
|
|
|
ACTH |
65 |
|
|
8.0 |
<10.0 |
16.0 |
|
T
Testosterone |
2.4 |
0.8 |
0.6 |
0.1 |
<
0.1 |
|
|
A |
3.4 |
4.8 |
4.0 |
0.7 |
0.3 |
0.4 |
|
DHEA-S |
7110 |
4770 |
57100 |
940 |
<300 |
<300 |
|
PRL |
13.5 |
|
|
13.5 |
|
29.1 |
|
TSH |
0.5 |
|
|
2.1 |
|
1.9 |
|
LH/FSH |
|
|
|
|
|
6.5
/ 8.7 |
UFC:
Urinary free cortisol; Dx: dexamethasone; KTZ: ketoconazole; A:
androstenedione; DHEA-S: dehydroepiandrosterone sulphate PRL: prolactin.
Pituitary MRI showing a 4-mm well-limited cystic area on the left side with
low signal intensity on T1 (A) and hyperintense on T2 (B). Note in both
sequences a second 3-mm small right superior hypointense area, attached to
the stalk (arrow).
MRI after presumed
pituitary apoplexy. Coronal view: note the disappearance of the hypointense
area attached to the stalk with persistence of the cystic lesion in the
left side.
Infarction or hemorrhage of a corticotrope adenoma could be a probable
underlying mechanism although its precipitating factor is unclear.
Ketoconazole withdrawal, through abrupt increase in cortisol production and/or
the interruption of a hypothetical inhibitory action on cell replication
followed by tumor growth and compromise of vascular supply, may be
considered as possible triggering factors. To the best of our knowledge,
this is the first report of spontaneous remission of Cushing’s disease
caused by presumed infarction of a microadenoma, unusually located in the
superior rim of the pituitary, attached to the stalk.
This
case was published in Pituitary 7:
45–49, 2004
“Spontaneous Remission of Cushing’s Disease
after Disappearance of a Microadenoma Attached to the Pituitary Stalk”
Analía B. Pignatta, Adriana G. Díaz, Reynaldo
M. Gómez and Oscar D. Bruno
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CLINICAL
CASE
