Abstract
Five patients (three females and two males, ages 12-21 years) had clinical features of the Cushing syndrome. Results of biochemical studies (in four patients tested) suggested the presence of an autonomously functioning adrenocortical neoplasm. However, radiologic examination of the adrenals did not show an adrenal tumor. The five patients underwent curative bilateral total adrenalectomy and did not manifest the Nelson syndrome postoperatively (follow-up, 0.5-22 years). The adrenal pathologic findings in these patients were similar. Gross findings included: 1) decreased, normal, or slightly increased total gland weight; 2) studding of the external and cut surfaces by small (<4 mm) black, brown, dark-green, red, or (rarely) yellow nodules; and 3) cortical atrophy and disorganization of the normal zonation between the nodules. Microscopically, the nodules were composed predominantly of enlarged, globular, cortical cells with granular eosinophilic cytoplasm that often contained lipofuscin. Twenty-six similar cases have been reported or communicated to us. Findings in these plus our five cases identify a special type of adrenocortical pathology associated with Cushing syndrome, for which we suggest the name “primary pigmented nodular adrenocortical disease.” There were three instances of familial involvement. Associated conditions include myxomas (cardiac, cutaneous and mammary), spotty pigmentation (cutaneous and mucocutaneous) and other types of endocrine overactivity (sexual precocity and gigantism).
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Aidan Carney, J. 120 PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE. RARE CAUSE OF THE CUSHING SYNDROME. Pediatr Res 19, 623 (1985). https://doi.org/10.1203/00006450-198506000-00140
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DOI: https://doi.org/10.1203/00006450-198506000-00140