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Letters to the Editor |
Hospital Xeral-Cies de Vigo Vigo, Spain \
We read with great interest the illustrative case reported by Sarlis et al. (1), recently published in JCEM, about a patient with primary pigmented nodular adrenocortical disease (PPNAD), who was treated by unilateral adrenalectomy. Although his daily urinary free cortisol (UFC) rate normalized and the major clinical manifestations of Cushing’s Syndrome (CS) subsided, loss of a circadian cortisol rhythm persisted after surgery. Twenty-seven years later, the patient presented again with short stature, severe osteopenia, skeletal deformities, and myopathy. In the extensive series published by Carney and Young (2), subtotal adrenalectomy was performed in 17 patients. Among these patients, 6 were considered successfully treated. However, they only showed remission of the clinical features of CS despite the persistence of autonomous adrenocortical function observed in those patients who were tested. This case showed that adrenal autonomous hyperfunction, despite the lack of biochemical hypercortisolism may lead to significant morbidity over the course of several years.
We have a patient who, at 12 yr of age, had hypercortisolism
associated with unilateral adrenocortical uptake of 131I
iodomethylnorcholesterol. The initial diagnosis was ACTH-independent CS
by adenoma. Subsequent surgical exploration was performed. At the time
of operation, the adrenal glands appeared normal. The surgeon, guided
by the scintigraphic image, performed a unilateral adrenalectomy on the
right side. Histologic study of surgical material revealed PPNAD. One
year later, CS disappeared, and the patient showed increasing energy
and muscular strength, weight loss, and increased height velocity. UFC
rate and basal plasma cortisol were within normal ranges, but loss of
diurnal rythmicity (plasma cortisol 8 h = 10 µg/dL, 20 h =
9.7 µg/dL) and suppressed basal ACTH levels (8 h 
5 pg/mL)
were present. This case was previously published (3). The patient and
his parents refused a new adrenalectomy because he felt well. The
hormonal pattern described remained during 4 yr. Five years after
unilateral adrenalectomy, diurnal rythmicity (plasma cortisol 8 h
= 15.4 µg/dL, 20 h = 7.7 µg/dL) was spontaneously recovered,
associated with serum basal ACTH (8 h = 26.5 pg/mL) within normal
range. The patient remained free of clinical features of CS. His final
height is in accordance with his midparental height, and bone
densitometry revealed normal bone density. Thus our patient has had a
complete remission of PPNAD.
The possibility that stimulating antibodies play a role in the pathogenesis of PPNAD was suggested by Wulffraat et al. (4), who reported that immunoglobulins prepared from serum of patients with PPNAD stimulated adrenal DNA synthesis in vitro. Such stimulation of the adrenal cortex would be analogous to the stimulation of thyroid tissue by thyroid stimulating immunoglobulin in Graves’ disease. In Graves’ disease, spontaneous remission of the disease has been described (5). A similar mechanism could explain our patient’s outcome.
The present case suggests the following considerations: 1) it supports the view that an autoimmune mechanism may be involved in PPNAD; 2) some but rare cases of PPNAD may have complete remission of the disease, at least temporarily; and 3) some cases of PPNAD treated with unilateral adrenalectomy and which have presented with clinical remission of CS associated with normal UFC rates, must be carefully followed for some time before completion of total adrenalectomy.
Footnotes
1 Received June 18, 1997. Address
correspondence to: Dr. Ricardo V. Garcia-Mayor, P.O. Box 1691, 36201
Vigo, Spain. 
References
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