Cushing's syndrome in children

Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia)

“…3 and 4). Many of these cells contained deposits of a brown lipochrome pigment arranged as described by Chute et al (1949) in a peculiar ring-like pattern. Fat cells and lymphocyte-like round cells could be demonstrated especially around these giant cells, resulting in a picture resembling the so-called myelolipomatous formation of the adrenals.…”

“…with Cushing's syndrome showed the typical lesions of the so-called cortical microadeno¬ matosis or primary cortical nodular dysplasia ac¬ cording to Meador et al (1967). As early as in 1949, Chute et al (1949) gave an excellent macroscopic and microscopic description of this adrenal disease. Further well-documented cases have been pub¬ lished by Rose et al (1952), Kracht & Tamm (1960), Mosier et al (1960), Klevit et al (1966), Levin (1966), Meador étal.…”

Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2.

“…Although the existence of the complex as an unrecognized, inherited syndrome was first suggested in 1985 (16,17), combinations of several components of the syndrome, and their familial occurrence had been reported earlier. Thus, the pathologic findings of the adrenal glands, multiple, small, pigmented, adrenocortical nodules and internodular cortical atrophy, were described in children and young adults with Cushing syndrome as early as in 1949 (18)(19)(20)(21)(22). Similarly, by then, several familial cases of cutaneous and cardiac myxomas associated with lentigines (lentigo simplex) or ephelides and blue nevi of the skin and mucosae had been described under the acronyms, NAME (for nevi, atrial myxoma, myxoid neurofibromata, and ephelides) and LAMB (for lentigines, atrial myxoma, mucocutaneous myxoma, blue nevi) syndromes (16,17,(23)(24)(25)(26)(27)(28)(29)(30).…”

Cushing's Syndrome in Childhood

“…Steroid Findings.-Despite the probable zonation of function within the normal adrenal cortex (Chute et al, 1949;Landing, 1955), the tumour, a strikingly undifferentiated carcinoma, possebsed the ability to synthesize all the three major groups of adrenal hormones. While the simultaneous hypersecretion of glucocorticoid and androgen by adrenal tumours is commonplace, and that of glucocorticoid and aldosterone has been described, the present case offers a remarkable example of pluripotency of an adrenal tumour.…”