J. Lester Gabrilove

Cushing's syndrome: A study of fifty patients

Abstract Clinical and laboratory data are reported in fifty patients with Cushings syndrome. Thirteen patients had adrenal carcinoma, eight a benign adrenal tumor, and in twenty-nine the Cushings syndrome was associated with non-tumorous adrenal cortical hyperfunction. One or both adrenals were examined in twenty-four patients with non-tumorous adrenal cortical hyperfunction. In thirteen the adrenals were found to be normal in size, in eleven they were enlarged. The presence of an adrenal cortical tumor could be correctly established by perirenal or retrorectal gas insufflation in three-fourths of the tumor group. The incidence of the various clinical signs and symptoms is indicated and the relationship of these manifestations to the underlying adrenal pathology is examined. Some electrolyte abnormality was demonstrable in half of the patients; evidence of disturbance in carbohydrate metabolism was obtained in almost all. The urinary excretion of neutral 17-ketosteroids was increased in half the patients with nontumorous adrenal cortical hyperfunction, onethird of the group with a benign adrenal tumor and two-thirds of those with adrenal carcinoma. All patients had an increase in the urinary excretion of either the formaldehydogenic fractions or the 17-hydroxycorticoids. Seven of the nine patients in whom the plasma level of the 17-hydroxycorticoids was determined demonstrated an increase. The response of the plasma level of the 17-hydroxycorticoids to the intramuscular administration of corticotropin, and also prednisone suppression studies, were of value in establishing the presence or absence of an adrenal tumor before operation. Treatment was determined by the nature of the adrenal pathology. Adrenal tumors were removed surgically, with proper preoperative preparation and postoperative care. In nontumorous adrenal cortical hyperfunction, pituitary radiation, unilateral adrenalectomy, or a combination of both measures, were employed. In approximately 75 per cent of this group a satisfactory remission was obtained. The remaining patients required bilateral adrenalectomy. Treatment was followed by complete disappearance of the clinical and laboratory abnormalities in some cases. The roentgen evidence of osteoporosis, however, remained unaltered, although bone pain subsided and spontaneous fractures no longer occurred. Postmortem studies were made in thirteen patients and the findings are recorded.Clinical and laboratory data are reported in fifty patients with Cushings syndrome. Thirteen patients had adrenal carcinoma, eight a benign adrenal tumor, and in twenty-nine the Cushings syndrome was associated with non-tumorous adrenal cortical hyperfunction. One or both adrenals were examined in twenty-four patients with non-tumorous adrenal cortical hyperfunction. In thirteen the adrenals were found to be normal in size, in eleven they were enlarged. The presence of an adrenal cortical tumor could be correctly established by perirenal or retrorectal gas insufflation in three-fourths of the tumor group. The incidence of the various clinical signs and symptoms is indicated and the relationship of these manifestations to the underlying adrenal pathology is examined. Some electrolyte abnormality was demonstrable in half of the patients; evidence of disturbance in carbohydrate metabolism was obtained in almost all. The urinary excretion of neutral 17-ketosteroids was increased in half the patients with nontumorous adrenal cortical hyperfunction, onethird of the group with a benign adrenal tumor and two-thirds of those with adrenal carcinoma. All patients had an increase in the urinary excretion of either the formaldehydogenic fractions or the 17-hydroxycorticoids. Seven of the nine patients in whom the plasma level of the 17-hydroxycorticoids was determined demonstrated an increase. The response of the plasma level of the 17-hydroxycorticoids to the intramuscular administration of corticotropin, and also prednisone suppression studies, were of value in establishing the presence or absence of an adrenal tumor before operation. Treatment was determined by the nature of the adrenal pathology. Adrenal tumors were removed surgically, with proper preoperative preparation and postoperative care. In nontumorous adrenal cortical hyperfunction, pituitary radiation, unilateral adrenalectomy, or a combination of both measures, were employed. In approximately 75 per cent of this group a satisfactory remission was obtained. The remaining patients required bilateral adrenalectomy. Treatment was followed by complete disappearance of the clinical and laboratory abnormalities in some cases. The roentgen evidence of osteoporosis, however, remained unaltered, although bone pain subsided and spontaneous fractures no longer occurred. Postmortem studies were made in thirteen patients and the findings are recorded.

ReviewCushing's syndrome: A study of fifty patients☆

Abstract Clinical and laboratory data are reported in fifty patients with Cushings syndrome. Thirteen patients had adrenal carcinoma, eight a benign adrenal tumor, and in twenty-nine the Cushings syndrome was associated with non-tumorous adrenal cortical hyperfunction. One or both adrenals were examined in twenty-four patients with non-tumorous adrenal cortical hyperfunction. In thirteen the adrenals were found to be normal in size, in eleven they were enlarged. The presence of an adrenal cortical tumor could be correctly established by perirenal or retrorectal gas insufflation in three-fourths of the tumor group. The incidence of the various clinical signs and symptoms is indicated and the relationship of these manifestations to the underlying adrenal pathology is examined. Some electrolyte abnormality was demonstrable in half of the patients; evidence of disturbance in carbohydrate metabolism was obtained in almost all. The urinary excretion of neutral 17-ketosteroids was increased in half the patients with nontumorous adrenal cortical hyperfunction, onethird of the group with a benign adrenal tumor and two-thirds of those with adrenal carcinoma. All patients had an increase in the urinary excretion of either the formaldehydogenic fractions or the 17-hydroxycorticoids. Seven of the nine patients in whom the plasma level of the 17-hydroxycorticoids was determined demonstrated an increase. The response of the plasma level of the 17-hydroxycorticoids to the intramuscular administration of corticotropin, and also prednisone suppression studies, were of value in establishing the presence or absence of an adrenal tumor before operation. Treatment was determined by the nature of the adrenal pathology. Adrenal tumors were removed surgically, with proper preoperative preparation and postoperative care. In nontumorous adrenal cortical hyperfunction, pituitary radiation, unilateral adrenalectomy, or a combination of both measures, were employed. In approximately 75 per cent of this group a satisfactory remission was obtained. The remaining patients required bilateral adrenalectomy. Treatment was followed by complete disappearance of the clinical and laboratory abnormalities in some cases. The roentgen evidence of osteoporosis, however, remained unaltered, although bone pain subsided and spontaneous fractures no longer occurred. Postmortem studies were made in thirteen patients and the findings are recorded.Clinical and laboratory data are reported in fifty patients with Cushings syndrome. Thirteen patients had adrenal carcinoma, eight a benign adrenal tumor, and in twenty-nine the Cushings syndrome was associated with non-tumorous adrenal cortical hyperfunction. One or both adrenals were examined in twenty-four patients with non-tumorous adrenal cortical hyperfunction. In thirteen the adrenals were found to be normal in size, in eleven they were enlarged. The presence of an adrenal cortical tumor could be correctly established by perirenal or retrorectal gas insufflation in three-fourths of the tumor group. The incidence of the various clinical signs and symptoms is indicated and the relationship of these manifestations to the underlying adrenal pathology is examined. Some electrolyte abnormality was demonstrable in half of the patients; evidence of disturbance in carbohydrate metabolism was obtained in almost all. The urinary excretion of neutral 17-ketosteroids was increased in half the patients with nontumorous adrenal cortical hyperfunction, onethird of the group with a benign adrenal tumor and two-thirds of those with adrenal carcinoma. All patients had an increase in the urinary excretion of either the formaldehydogenic fractions or the 17-hydroxycorticoids. Seven of the nine patients in whom the plasma level of the 17-hydroxycorticoids was determined demonstrated an increase. The response of the plasma level of the 17-hydroxycorticoids to the intramuscular administration of corticotropin, and also prednisone suppression studies, were of value in establishing the presence or absence of an adrenal tumor before operation. Treatment was determined by the nature of the adrenal pathology. Adrenal tumors were removed surgically, with proper preoperative preparation and postoperative care. In nontumorous adrenal cortical hyperfunction, pituitary radiation, unilateral adrenalectomy, or a combination of both measures, were employed. In approximately 75 per cent of this group a satisfactory remission was obtained. The remaining patients required bilateral adrenalectomy. Treatment was followed by complete disappearance of the clinical and laboratory abnormalities in some cases. The roentgen evidence of osteoporosis, however, remained unaltered, although bone pain subsided and spontaneous fractures no longer occurred. Postmortem studies were made in thirteen patients and the findings are recorded.

Ultrasonography of adrenal masses: usual features.

Gray-scale B-scan was successful in delineating adrenal masses in 40 patients, all confirmed pathologically. Two patients with extra-adrenal pheochromocytomas, two calcified cysts, and two patients with bilateral adrenal hyperplasia are demonstrated. The usual ultrasonographic features are presented in this paper; they correlated well with urographic, angiographic, CT, and pathological findings. The smallest mass demonstrated was an aldosteronoma (1.3 cm in size).

Ultrasonography of adrenal masses: unusual manifestations.

Forty patients with pathologically-proved adrenal masses, and two patients with extra-adrenal pheochromocytomas, were examined with gray scale B-scan. The authors describe unusual echo patterns of adrenal masses due to necrosis or hemorrhage, variations in the effects of large adrenal masses on surrounding organs and vessels, and the differential diagnosis. The complementary role of computed tomography is also discussed.

Effect of the Addition of Estrogen to Medical Castration on Prostatic Size, Symptoms, Histology and Serum Prostate Specific Antigen in 4 Men with Benign Prostatic Hypertrophy

A total of 4 men with benign prostatic hypertrophy who underwent medical castration therapy with a long-acting gonadotropin-releasing hormone agonist (leuprolide) for more than 6 months elected to add an estrogen transdermal patch (0.05 mg. to the skin biweekly) to the leuprolide regimen. The average prostatic size (transrectal ultrasound), serum prostate specific antigen (PSA) levels and symptoms of prostatism were dramatically decreased with leuprolide alone. The addition of estrogen for 6 months did not result in any change in prostate size, symptoms or serum PSA levels over that seen with leuprolide alone. The development of squamous metaplasia was noted in 1 man with leuprolide alone and in 1 man after the addition of estrogen. Immunohistochemical staining with anticytokeratin 903 antibodies reveals that squamous metaplasia appears to arise from prostatic basal cells. We postulate that the target cell for estrogen action in the prostate is the prostatic basal cell. In the absence of androgen the only direct effect of estrogens is the induction of squamous metaplasia.

Electron microscopy of a feminizing leydig cell tumor of the testis

The ultrastructural characteristics of a feminizing interstitial (Leydig) cell tumor of the testis were compared with those of normal Leydig cells and with the findings described in 10 published cases of Leydig cell tumor. The neoplastic Leydig cells superficially resembled normal Leydig cells. Similarities included abundant smooth endoplasmic reticulum, lipid, and microbodies. Contrastingly, Reinke crystalloids and paracrystalline inclusions were absent and lipochrome pigment and lysosomes very rare. The nuclei were large and contained enlarged, often multiple, nucleoli. The nuclear membranes tended to be irregular and undulating. Cytoplasmic membranous whorls and myelin figures were conspicuous. Fairly homogeneous fibrous septa were evident between single and grouped tumor cells. Despite several individual variations, there is a general resemblance between the neoplastic Leydig cells in this patient and those previously reported. No distinguishing ultrastructural characteristics were discerned between feminizing and virilizing Leydig cell tumors.

Cushing's syndrome and avascular necrosis of bone associated with carcinoid‐islet cell tumor of the pancreas

A 69‐year‐old woman with Cushings syndrome due to ACTH production by a pancreatic tumor had hypokalemia, osteoporosis, muscle wasting, and avascular necrosis of the humeral heads. She had an excellent response to bilateral adrenalectomy. These and other clinical and pathophysiologic aspects of the ectopic ACTH syndrome are discussed. The pancreatic tumor was histologically compatible with either an islet cell or a carcinoid tumor. The evidence for the histologic and functional overlap of these two types of tumor is reviewed.

ADRENAL CORTICAL FAILURE

Ailthough i t has long been established that the clinical manifestations of Addison’s disease represent the classic picture of adrenocortical failure, only in relatively recent times have the physiologic functions of the adrenal cortex been understood. It has remained for relatively contemporary investigations to establish the importance of this endocrine gland to the total body economy, contrasted with its relation to a few clinical entities representing its hyperfunctional or hypofunctional state. Whereas a t one time we thought of endocrinology as a field concerned specifically with orthodox glandular abnormalities, we now recognize that the glands of internal secretion, and particularly the adrenal cortex, play a significant role in all physiologic processes, and color and influence the manifestations of disease not heretofore considered endocrinologic in character. The functional integrity of the adenohypophyseal-adrenocortical-thyroidal apparatus is of paramount importance in the adequate response of the organism to stress, be i t the stress of a surgical procedure, pneumonia, coronary occlusion, or a host of other environmental hazards. Functional inadequacy of this system under such circumstances may result in disaster. It is particularly for these reasons that one must consider the possibility of latent or potential adreuorortical failure occurring apart from classic Addison’s disease.