Objective: To study the diagnosis and treatment of primary pigmented nodular adrenocortical disease (PPNAD).
Methods: The clinical data including symptom, endocrinal examination, surgical operation and prognosis of 4 cases of PPNAD hospitalized from 2000 to 2007 were analyzed respectively, relative literature were reviewed.
Results: 1 case received total adrenalectomy needed adrenocortical hormone postoperatively, 3 cases responded favorably to subtotal adrenalectomy and did not need adrenocortical hormone, however, 1 case was diagnosed of thyroid carcinoma 2 years after subtotal adrenalectomy.
Conclusions: PPNAD is a rare subtype of ACTH-independent Cushing's syndrome, the diagnosis is depend on symptom, endocrinal examination and pathology. Total adrenalectomy is suitable to the patients with obvious symptom; subtotal adrenalectomy to the patients with mild symptom, the lesion of other endocrinal organ must be followed up postoperatively.
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