Primary pigmented nodular adrenocortical disease with synaptophysin immunoreactivity in two thai children.

ABSTRACT

This paper reports the cases of two Thai children diagnosed with primary pigmented nodular adrenocortical disease (PPNAD). The first was a thirteen and a half year old male who presented with Cushing syndrome for three years and severe osteoporosis. He had hypercortisolemia, a non-suppressible dexamethasone suppression test, and low serum ACTH. A CT scan showed slight enlargement of both adrenal glands. A bilateral adrenalectomy was performed. The second case was a thirteen-year old female who presented with mild Cushing syndrome for one year with paradoxical response to high-dose dexamethasone suppression test. An MRI revealed suspected microadenoma of the left adrenal gland. Blood sampling showed a higher cortisol level from the left adrenal vein than from the right. A left adrenalectomy was performed, followed by a right adrenalectomy four months later. The pathologies were compatible with PPNAD. Immunostaining for synaptophysin was done in both patients. The cells in the adrenocortical micronodules of both cases were stained intensely with antiserum for synaptophysin, whereas the surrounding adrenal cortex did not.