Cases reported "Adrenal Cortex Diseases"

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1/6. Cushing's syndrome due to bilateral adrenocortical adenomas with unique histological features.

    Cushing's syndrome due to bilateral cortisol-secreting adenomas rarely occurs. We present a case of Cushing's syndrome due to bilateral adenomas. Both adenomas had distinct cell compositions, and were compared with emphasis on immunohistochemical and enzyme histochemical analysis for cytochrome P450(11beta) and 3beta-hydroxysteroid dehydrogenase (3betaHSD). A 37 year-old female was diagnosed with ACTH-independent Cushing's syndrome based on physical findings and hormonal evaluation. High-resolution CT scan showed bilateral adrenocortical adenomas and atrophied glands. 131I-methylnorcholesterol incorporation into both glands suggested both adenomas were functional. Clinical diagnosis prior to surgery was ACTH-independent Cushing's syndrome due to functioning bilateral adenomas. The left adrenal gland was totally resected, while the right one was partially resected by laparoscopic approach. Both adenomas were black on cut sections, and were comparatively evaluated by immunohistochemical and enzyme histochemical analysis for P450(11beta) and 3betaSD. The left adenoma was 1.6 cm in diameter and had a complex cellular composition and enzyme expression similar to that of primary pigmented nodular adrenocortical disease (PPNAD), while the right adenoma was 1.8 cm in diameter with compact cells typical of a solitary cortisol-producing adenoma. Adjacent bilateral adrenal cortex showed marked atrophy, but contained several micronodules. serum cortisol levels, both at basal and after a low dodexamethasone, normalized thirteen months after surgery. In conclusion, the present case of Cushing's syndrome with bilateral adrenal adenomas demonstrated for the first time the simultaneous occurrence of two distinct adenomas, an ordinary cortisol-producing adenoma and a PPNAD-like adenoma. Further case reports of multiple adrenal adenomas should be well-analyzed to clarify whether the results from this case represent a new subgroup of ACTH-independent Cushing's syndrome.
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2/6. Autoimmune polyglandular syndrome associated with idiopathic giant cell myocarditis.

    The autoimmune polyglandular syndrome (APS) is characterized by a variable coexistence of several autoimmune diseases, affecting predominantly endocrine glands. In general two types of APS are distinguished. Type 1 APS is an autosomal recessive disorder often leading to insufficiency of the adrenal cortex, the parathyroid glands, and/or the gonads. This type of APS often affects the skin in form of chronic mucocutaneous candidiasis and ectodermal dystrophies (vitiligo, alopecia, keratopathy, dystrophy of dental enamel and nails). The second form of APS is a polygenic disease which usually involves the adrenal gland, the thyroid and the pancreatic beta-cells. In rare cases APS type 2 is associated with myasthenia gravis, autoimmune thrombocytopenic purpura, sjogren's syndrome or rheumatoid arthritis. Here we describe a case of APS with the unusual combination of type 1 diabetes, secondary adrenocortical insufficiency, growth hormone deficiency, and primary hypothyroidism associated with lethal idiopathic giant cell myocarditis. The combination of APS and idiopathic giant cell myocarditis which is a rare, frequently fatal autoimmune disorder of myocardium affecting most commonly young individuals has not been reported so far.
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3/6. Benign hemorrhagic adrenocortical macrocysts in beckwith-wiedemann syndrome.

    A new pseudotumorous lesion found in the adrenal cortex of six infants with beckwith-wiedemann syndrome is described. These cystic masses were discovered either prenatally by using sonography or early in the neonatal period as palpable flank masses. Imaging studies, including sonography and CT, could not confidently exclude malignancy. After the masses were removed surgically, histologic examination showed them all to be benign hemorrhagic macrocysts within the capsule or permanent cortex (in contrast to neonatal adrenal hemorrhage, which usually occurs more centrally in the fetal cortex). The cysts were as large as 8 cm in diameter, and in one case a solitary cyst was predominant. Hemihypertrophy was present in all cases. Four of the six lesions were right-sided, and there was a male-female ratio of 5:1. Benign hemorrhagic adrenocortical macrocysts are a cause of abdominal mass in the fetus and neonate with beckwith-wiedemann syndrome.
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4/6. adrenoleukodystrophy. Preliminary report of a connatal case. light- and electron microscopical, immunohistochemical and biochemical findings.

    This is the first description of a connatal case of adrenoleukodystrophy. The clinical picture consisted of severe psychomotor retardation, convulsions and hypsarrhythmia, but no obvious signs of adrenal insufficiency. Pathologically, the adrenals were small. The entire cortex was largely replaced by large round cells. Ultrastructurally, some cells in the adrenal cortex contained inclusions with electron-lucent clefts surrounded by a membrane. The anterior pituitary lobe could be demonstrated to have produced ACTH. The central nervous system showed extensive zones of demyelination in the brainstem, the cerebellum and the right-sided capsula interna. In the demyelinated areas there was sudanophilic breakdown and an intense gliosis. Ongoing demyelination could also be demonstrated by the chemical analysis. In the gray matter there waere micropolygyria of the insular cortex and swollen nerve cells in the nucleus arcuatus. Ultrastructure revealed the type of inclusions in the microglia of the same type as in the adrenals, and a different type of inclusions in unidentifiable cells, possibly neurons. These latter inclusions consisted of loosely stacked lamellar material. The findings are interpreted as further evidence of storage taking place in this disease.
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5/6. Primary adrenocortical micronodular dysplasia: enzyme histochemical and ultrastructural studies of two cases with a review of the literature.

    The adrenal glands from two patients with primary adrenocortical micronodular dysplasia (PAMD) were studied (no. 1, a 23-year-old man with cardiac myxomas and sarcoidosis; no. 2, a 16-year-old girl). The PAMD cells showed intense activity of 3 beta-hydroxysteroid dehydrogenase (3 beta DH), succinate DH, glucose-6-phosphate DH, alkaline phosphatase (AlPase), and other DHs and lysosomal hydrolases, giving a characteristic staining pattern. The staining patterns correlated well with ultrastructural findings. The larger adrenals (no. 1: 16.6 g) were associated with larger black nodules and internodular cortex (INC) with fairly well-retained enzyme activities, and contained cell clusters and single cells with intense AlPase activity with the appearance of PAMD nodules in the buds. The smaller adrenals (no. 2: 5.4 g) were associated with smaller black/yellow nodules, and had INC with weaker enzyme activity. Analysis of descriptions of INC in 25 previously reported cases revealed that the larger adrenals (more than 10 g) had less atrophic INC than the smaller ones. The characteristic enzyme pattern in PAMD cells explains the paradox that PAMD adrenals smaller than normal can cause Cushing's syndrome, and may be useful for investigating neoplastic and non-neoplastic counterparts in other adrenal lesions. The relationship between PAMD cells and INC is not simply one of autonomy versus atrophy, and both cell types may be stimulated by a certain common trophic factor. Thus, PAMD belongs to the category of hyperplasia.
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6/6. Preservation of adrenocortical function during surgery for bilateral pheochromocytoma.

    A case is reported of bilateral pheochromocytoma treated by bilateral adrenalectomy. A portion of the left adrenal cortex was left in the adrenal bed, although the arterial and venous blood supply had been ligated. A successful autograft was documented 15 years postoperatively. A review of the literature failed to reveal any such successful attempt in the management of bilateral pheochromocytoma. The patient has a normally functioning adrenal cortex that responds to adrenocorticotropic hormone stimulation in the usual manner. The procedure is recommended in cases of bilateral pheochromocytoma because of its apparent ease and because it obviates the need for a lifetime of adrenocortical steroid replacement.
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