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A case of Cushing syndrome in a 25-year-old female diagnosed during pregnancy is presented. The pregnancy was complicated by diabetes mellitus, pregnancy induced hypertension, prematurity and intrauterine growth retardation. The patient underwent transphenoidal pituitary adenectomy after delivery for removal of a large pituitary adenoma. The mother and infant are well 10 months after delivery.
Cushing's syndrome is accompanied by many different skin symptoms. A case of a 43-year-old female patient with unrecognized adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is reported. Besides numerous skin lesions, the patient presented with a rapidly progressive leg ulcer due to venous thrombosis. The initial skin symptoms appeared on her lower leg one month prior to admission. The patient was treated with oral prednisone, however, rapid progression of the skin lesion was observed. On admission the patient presented with a large, very painful ulceration, partially covered with a crust, on the right lower leg with several satellite smaller ulcerations. The other leg showed no changes. Moreover, the patient had pronounced hirsutism and skin darkening on the face, and Muehrcke's lines were observed on all her nails. Microscopic findings of skin biopsy were uncharacteristic, with some signs of angiopathy and vasculitis. Based on the elevated serum levels of ACTH and cortisol, and the result of dexamethasone suppression test, ACTH-dependent Cushing syndrome was diagnosed. Sonography of leg veins revealed a recanalized thrombus of the right popliteal vein with reflux. The patient was transferred to surgical department where both suprarenal glands were removed. Upon suprarenal gland removal, significant improvement of the patient's general condition and rapid healing of the leg ulcer were observed. Briefly, this patient is presented to point out that a leg ulcer caused by venous insufficency may mimic fulminant purpura or some other type of vasculitis in patients with Cushing syndrome.
Ectopic production of ACTH is observed in 6% of patients with Cushing syndrome. Ten percent of these cases are related to endocrine pancreatic tumors. In a few cases a multiplicity of hormones are produced. The combination with a Zollinger-Ellison syndrome is very infrequent. In the present case, a female patient aged 54, there was an interval between the onset of Zollinger-Ellison and Cushing syndrome. The combination of bilaterally enlarged adrenals in the absence of an adrenal adenoma and the presence of Crooke-cells in the adenohypophysis in a patient with Cushing syndrome are of diagnostic significance. This trias should always alert the physician to the possibility of extrahypophyseal ACTH production.
Cushing syndrome (CS) caused by ectopic adrenocorticotropic hormone (ACTH) production (EA) poses major challenges diagnostically by mimicking the pituitary-dependent form of CS and therapeutically by producing severe, life-threatening hypercortisolemia. This retrospective follow-up study describes the clinical characteristics and course of EA in a large referral center. Computer-based cross-index codes for EA, CS, and bilateral adrenalectomy were used to identify patients treated at the Mayo Clinic between 1956 and 1998. EA was confirmed in 106 patients. Gender distribution showed a slight female predominance (61:45). Bronchial carcinoid was the most frequent cause of EA (25%), followed by islet cell cancer (16%), small-cell lung carcinoma (11%), medullary thyroid cancer (8%), disseminated neuroendocrine tumor of unknown primary source (7%), thymic carcinoid (5%), pheochromocytoma (3%), disseminated gastrointestinal carcinoid (1%), and other tumors (8%). No tumor was found in 16% of patients. Altogether, 28 patients were managed medically, and the others underwent curative tumor resection (13 patients) or bilateral adrenalectomy (65 patients). Surgically treated patients had longer survival, but this was most likely affected by treatment bias. The diagnoses of CS and ACTH-secreting neoplasm were usually concurrent, although, there were remarkable cases in which the two conditions were diagnosed several years apart. Curative resection of the tumor producing EA was possible in a small proportion of patients (12%). When curative resection is not possible, patients who are reasonable surgical candidates are likely to benefit from adrenalectomy. Additional experience with bilateral laparoscopic adrenalectomy should increase the number of patients who benefit from adrenal-directed surgery.
STUDY OBJECTIVE: Review and evaluation of diagnostic tests for the Cushing syndrome based on reports published since 1978. DATA IDENTIFICATION: Studies published in the English literature from 1978 through 1989 were identified using Index Medicus and cross searching of bibliographies. STUDY SELECTION AND DATA EXTRACTION: Studies of five or more patients for general analysis and smaller studies and case reports when pertinent. To develop criteria for the corticotropin-release hormone (CRH) stimulation test, only studies reporting individual patient data were analyzed. RESULTS: No new test is clearly better than existing tests in establishing a definitive diagnosis. Among tests to determine cause, the CRH stimulation test, by newly developed criteria, has a 91% sensitivity (95% CI, 85% to 95%) and a 95% specificity (CI, 82% to 99%), and the overnight high-dose dexamethasone suppression test has an 89% sensitivity (CI, 80% to 94%) and a 100% specificity (CI, 84% to 100%) for the pituitary Cushing syndrome. Magnetic resonance imaging has greater sensitivity for detecting adrenocorticotropin (ACTH)-producing pituitary adenomas than computed tomography. Inferior petrosal sinus sampling can correctly identify a pituitary cause in 88% (CI, 79% to 94%) of cases. CONCLUSIONS: Diagnosis is still best established by using 24-hour urine free cortisol measurements or low-dose dexamethasone suppression testing. The CRH stimulation test is an outpatient alternative to determine cause, and the over-night high-dose dexamethasone test may become the test of choice along with plasma ACTH measurements by radioimmunoassay in the initial evaluation of cause. Magnetic resonance imaging should be used to evaluate the pituitary Cushing syndrome, and inferior petrosal sinus sampling is most useful in problematic cases with uncertain cause.
A patient with chronic daily headaches developed overt signs of Cushing syndrome during treatment with serial occipital nerve block injections. Investigation demonstrated an exogenous source of corticosteroids as the cause of the Cushing syndrome in this patient, thus, implicating the corticosteroid component of the occipital nerve blocks. To our knowledge, this is the first report of Cushing syndrome caused by occipital nerve blockade. Caution is warranted in employing even usual therapeutic doses of synthetic corticosteroids, particularly in long-acting or depot preparations.
Pseudo-Cushing Syndromes (PCS) are a heterogeneous group of disorders, including alcoholism and depression, that share many of the clinical and biochemical features of Cushing's Syndrome (CS). It has been suggested that hypercortisolism of PCS may be the result of increased hypothalamic corticotropin-releasing hormone secretion in the context of a hypothalamic-pituitary-adrenal axis that is otherwise normally constituted. The substantial overlap in clinical features and daily urinary free cortisol levels between several patients with CS and those with PCS can make the differential diagnosis difficult. The most accurate tests in the distinction of CS from alcohol-induced PCS are dexamethasone-CRH and a midnight serum cortisol measurement. In depressed patients, the insulin tolerance test may be useful, although some overlap may exist. This brief review summarises the principal pathophysiological events of PCS and provides a useful strategy for differential diagnosis.
A 27-year-old women developed Cushing syndrome during pregnancy. The course of pregnancy was characterized by abnormally low urine and plasma estrogen levels despite the presence of a living fetus. The signs and symptoms of Cushing syndrome disappeared spontaneously within 2 months after delivery. Hormonal studies, however, suggested the presence of an adrenal tumor. An adrenocortical adenoma was found at surgery and removed.
STUDY OBJECTIVE: To assess the efficacy of 131I-6-beta-iodomethylnorcholesterol scintigraphy in the adrenocorticotropic hormone-independent Cushing syndrome and to compare this with computed tomography. DESIGN: Retrospective analysis of case series from 1977 to 1987. SETTING: Referral to the Division of Nuclear Medicine at a tertiary-care university medical center. PATIENTS: Twenty-four patients with a pathologically-confirmed diagnosis of the adrenocorticotropic hormone-independent Cushing syndrome had 131I-6-beta-iodomethylnorcholesterol scintigraphy and, in most cases, computed tomography. MEASUREMENTS AND MAIN RESULTS: Using 131I-6-beta-iodomethylnorcholesterol scintigraphy, adenomas were accurately seen as focal, unilateral tracer uptake in 14 of 14 patients. In carcinoma, the classic scintigraphic pattern of bilateral nonvisualization was observed in 3 of 4 patients, with ipsilateral uptake of tracer in 1 patient with a histologically well-differentiated malignancy. Computed tomography done during the same interval depicted abnormal adrenals in all cases of adenoma and carcinoma. In cortical nodular hyperplasia, however, computed tomography identified abnormal pairs of adrenals in only one of four cases studied, whereas scintigraphy showed typical patterns of bilateral increased uptake in all of the cases. CONCLUSION: 131I-6-beta-iodomethylnorcholesterol scintigraphy accurately shows the location and nature of adrenal dysfunction in the adrenocorticotropic hormone-independent Cushing syndrome and may be particularly useful in identifying the bilateral adrenal involvement in cortical nodular hyperplasia.
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Cushing syndrome can recur following an adrenalectomy. One of the primary causes is recurrence of adrenal carcinoma either locally or from metastases. Hyperplasia and hyperfunction of adrenal remnants may also occur if there is pituitary stimulation. We have a patient in whom recurrent Cushing syndrome developed from small nonmalignant deposits of adrenal tissue in the perirenal adipose tissue following adrenalectomy of a benign adenoma. These deposits were identifiable by computed tomography. A false-negative NP-59 iodocholesterol scan was instructive in pointing out some problems in the interpretation of this type of scan for adrenal tissue.
The occurrence of pregnancy in the face of untreated Cushing syndrome is rare because of the high incidence of ovulatory disturbances experienced by patients with the disorder. A total of 58 patients with 65 pregnancies has been reported in the literature to date. Although pituitary-dependent adrenal hyperplasia is the most common etiology of Cushing syndrome in the general population, adrenal adenoma is more common in the pregnant population. Significant maternal morbidity is attributable to hypertension, congestive heart failure, and poor tissue healing. Prematurity and intrauterine growth retardation account for most of the perinatal morbidity; perinatal mortality is substantial. Treatment directed at relieving hypercortisolism has been instituted during pregnancy: Pituitary or adrenal surgery, chemotherapy, and pituitary irradiation have all been reported, with variable results. Information is lacking on any alteration of maternal morbidity after treatment. The impact of therapy on perinatal outcome appears limited to a reduction in the prematurity rate, but overall numbers are small and such a conclusion should be viewed with caution. No significant maternal or perinatal complications secondary to treatment itself were reported.
A variety of diagnostic advances including radioimmunoassay of adrenocorticotropic hormone (ACTH) have increased the number of methods for laboratory investigation of Cushing syndrome.* However, experience with these procedures has led to a recognition of their limitations. We have developed an algorithm which incorporates these newer techniques and minimizes the number of procedures required to diagnose the various causes of Cushing syndrome. At present, we recommend pituitary surgical operations for pituitary-dependent Cushing syndrome because we believe this disease is caused by the development of a pituitary ACTH-secreting tumor.
Cushing syndrome due to primary adrenocortical nodular dysplasia was diagnosed in two patients, aged 3 years 9 months and 9.5 years. Subsequently, adrenalectomy was performed and followed by steroid replacement. In both cases, the adrenals were normal or only slightly enlarged and showed adrenocortical nodular dysplasia histologically. Small lymphocytic infiltrates consisting of T-cells and class II MHC positive macrophages were present in adrenal specimens of both the patients. Samples of protein A sepharose purified serum immunoglobulins from both children stimulated adrenocortical DNA synthesis and cortisol production in cultured guinea-pig adrenal segments in vitro in a dose dependent fashion. Adrenal stimulating immunoglobulins were also demonstrated in serum specimens of both patients' mothers. However, none of them had overt signs of adrenal disease. Our data support the view that autoimmune mechanisms may be involved in primary adrenocortical nodular dysplasia.
Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome in infants, children, and young adults. It is characterized by non-adrenocorticotropic hormone-dependent hypersecretion of cortisol by multiple, pigmented nodules of hyperplastic adrenocortical cells. With a single exception, adrenal glands have been described as normal with computed tomography (CT) in all previous series. Eight patients had Cushing syndrome due to surgically proved PPNAD. Four of the eight patients had stigmas of Carney complex (lentigines, calcified Sertoli cell tumors of the testes, and cardiac and soft-tissue myxomas). CT and/or magnetic resonance (MR) imaging demonstrated unilateral or bilateral nodularity in five of six patients examined. Macronodules (greater than 10 mm) were seen in the two oldest patients. As the clinical presentation of Cushing syndrome in this group of patients may be atypical (severe osteoporosis or short stature), the detection of multiple, small adrenocortical nodules with CT or MR imaging supports, or may even suggest, the diagnosis of PPNAD.
STUDY OBJECTIVE: To determine the usefulness of the continuous intravenous dexamethasone suppression test in the differential diagnosis of patients with the Cushing syndrome. DESIGN: Comparison of the results of this test with those of other tests in the study population and with data from the literature. SETTING: Referral-based endocrine division in a university hospital. PATIENTS: One hundred and twenty-one patients with the Cushing syndrome were evaluated: corticotropin (ACTH)-secreting pituitary tumors, 90 cases; adrenal adenoma, 13; adrenal carcinoma, 9; ectopic ACTH-secreting tumors, 6; corticotropin-releasing-hormone (CRH)-secreting tumors, 2; and autonomous bilateral micronodular adrenal hyperplasia, 1. The continuous intravenous dexamethasone suppression test was done in 101 patients. MEASUREMENTS AND MAIN RESULTS: Dexamethasone (1 mg/h) was administered intravenously for 7 hours. A cortisol decrease of at least 190 nmol/L was defined as a positive response. In identification of patients with ACTH-secreting pituitary adenomas, this test had a sensitivity of 100% (95% CI, 95% to 100%), a specificity of 90% (CI, 70% to 99%), and a diagnostic accuracy of 98% (CI, 93% to 100%). Only two patients had false test results. They both had CRH-secreting tumors. Cortisol secretion was also shown to be sensitive for feedback control in other tests, however. CONCLUSION: The continuous intravenous dexamethasone suppression test has better diagnostic accuracy than other tests that are currently used in the differential diagnosis of the Cushing syndrome, and this test is very convenient. The only false test results were found in patients with CRH-secreting tumors.
A twenty-one-year-old virilized woman with Cushing syndrome due to a huge adrenocortical carcinoma was successively treated with trilostane (3 beta-hydroxysteroid dehydrogenase inhibitor), subsequent adrenalectomy, and postoperative cis-platinum. Clinical or biochemical abnormalities peculiar to Cushing syndrome gradually subsided, and three and one-half years after the adrenal surgery, the patient delivered a normal female infant. This study points out some of the clinical and biochemical responses of each treatment.