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Management of pheochromocytomas in patients with multiple endocrine neoplasia type 2 syndromes.

OBJECTIVE: The authors sought to determine the optimal surgical management of pheochromocytomas that develop in patients with multiple endocrine neoplasia (MEN) type 2 syndromes. SUMMARY BACKGROUND DATA: The performance of empirical bilateral adrenalectomy in patients with MEN 2A or MEN 2B, whether or not they have bilateral pheochromocytomas, is controversial. METHODS: The results of unilateral or bilateral adrenalectomy were studied in 58 patients (49 with MEN 2A and 9 with MEN 2B). Recurrence of disease was evaluated by measuring 24-hour urinary excretion rates of catecholamines and metabolites and by computed tomography (CT) scanning. RESULTS: The mean postoperative follow-up was 9.40 years. There was no operative mortality and malignant or extra-adrenal pheochromocytomas were not present. Twenty-three patients with a unilateral pheochromocytoma and a macroscopically normal contralateral gland underwent unilateral adrenalectomy. A pheochromocytoma developed in the remaining gland a mean of 11.87 years after the primary adrenalectomy in 12 (52%) patients. Conversely, 11 (48%) patients did not develop pheochromocytoma during a mean interval of 5.18 years. In the interval after unilateral adrenalectomy, no patient experienced hypertensive crises or other complications related to an undiagnosed pheochromocytoma. Ten (23%) of 43 patients having both adrenal glands removed (either at a single operation or sequentially) experienced at least one episode of acute adrenal insufficiency or Addisonian crisis, including one patient who died during a bout of influenza. CONCLUSIONS: Based on these data, the treatment of choice for patients with MEN 2A or MEN 2B and a unilateral pheochromocytoma is resection of only the involved gland. Substantial morbidity and significant mortality are associated with the Addisonian state after bilateral adrenalectomy.

Addison Disease↗

Adrenalectomy in adrenal hypertension.

Between 1965 and 1983, a total of 138 patients with endocrine hypertension were treated at the Münster Surgical Clinic. 71 catecholamine-producing tumors were removed in 56 patients, 60 patients had primary aldosteronism; 32 patients with Cushing's syndrome received uni- or bilateral adrenalectomy. The operative access to the adrenals was by anterior abdominal incision which we consider superior to lateral or transthoracic approaches. 35 complications occurred. The operative management of these forms of secondary hypertension, with its low overall rate of complications, is currently seen as the most successful therapeutic approach.

Adenoma↗

Metabolism of C 19 -steroids by homogenates of normal rat and mouse adrenal tissue and of the Snell transplantable rat adrenocortical tumour 494.

C(19)-steroid metabolism in homogenates of adrenal tissue from rats and mice has been studied. Production of these compounds from [7alpha-(3)H]cholesterol by rat adrenal tissue appeared to follow a route independent of pregnenolone. The major products of [7alpha-(3)H]-dehydroepiandrosterone metabolism by rat adrenal tissue were 5alpha-reduced steroids, principally androsterone, epiandrosterone and 5alpha-androstanedione. No differences in metabolism of [7alpha-(3)H]dehydroepiandrosterone or [4-(14)C]pregnenolone were detected between adrenal tissue from Sprague-Dawley, Wistar and Osborne-Mendel rats, but experiments with the Snell rat adrenocortical tumour 494 showed that this tissue had low 5alpha-reductase activity. In contrast, the major products of [7alpha-(3)H]dehydroepiandrosterone metabolism by mouse adrenal tissue were 5beta-reduced steroids. Differences were observed between LACA and NH strains of mice in that there was a lower metabolism of androstenedione by NH mouse adrenal and a considerable difference in the proportions of aetiocholanolone and epiaetiocholanolone produced.

Adrenal Gland Neoplasms↗

Fine-needle aspiration biopsy of adrenal masses in patients with extraadrenal malignancy.

BACKGROUND: Reports evaluating the efficacy of fine needle aspiration (FNA) of the adrenal gland have suggested a possible correlation between size of an adrenal mass and the presence of a primary or metastatic malignancy. These studies have focused on FNAs of all adrenal gland masses regardless of clinical history. This study investigates this relationship in a subpopulation of patients with a known history of primary extraadrenal malignancy. METHODS: All patients who have undergone computed tomographic-guided FNA biopsy of an adrenal mass at Loyola University Medical Center and Hines Veterans Administration Hospital, from 1985 to 1991, were reviewed. RESULTS: If size was assumed to be an independent predictor for presence of metastases, the highest efficiency was obtained with a cutoff value of 3 cm. This value divided the group into 15 (42%) "low-risk" (< or = 3 cm) and 21 (58%) "high-risk" (> 3 cm) subjects. In the "low-risk" group, 87% of the masses (13 of 15) were benign and 13% (2 of 15) were malignant. Within the "high-risk" group, more than 95% of the masses (20 of 21) were malignant, with a single (5%) benign case (p < 0.05). CONCLUSIONS: There is a significant correlation between the size of an adrenal nodule and the presence of metastases in patients with a known primary extraadrenal malignancy. Nodules greater than 3 cm have a very high probability of involvement by metastatic tumor. Nodules 3 cm or smaller are usually benign, but metastatic tumor can still be found in up to 13%. FNA biopsy is useful in evaluating adrenal masses in this setting.

Adrenal Gland Diseases↗

[Metachronous or contralateral adrenal metastases in renal cell cancer].

There are few reports on contralateral or metachronous adrenal gland metastases in renal cell carcinoma (RCC) in the literature. Since sonography has been more frequently used in follow-up examinations these metastases have been found more often. We report on five patients with solitary metastases from RCC to the ipsi- or contralateral adrenal gland.

Adrenal Gland Neoplasms↗

Simultaneous occurrence of multiple neoplasms and hyperplasias in the adrenal and thyroid gland of the horse resembling multiple endocrine neoplasia syndrome: case report and retrospective identification of additional cases.

Neoplastic and hyperplastic disorders that affect multiple endocrine tissues in a single individual are well described in humans but less so in domestic animals. Multiple endocrine neoplasia (MEN) in humans is a genetically determined syndrome characterized by the appearance of benign or malignant proliferations within two or more endocrine glands. The primary endocrine tumors that are characteristic of MEN arise from cells that share the capacity for amine precursor uptake and decarboxylation. Here we describe the case of a 22-year-old Thoroughbred mare that died during an unattended parturition and subsequently was presented for necropsy at the University of California, Davis, Veterinary Medical Teaching Hospital. A C-cell (medullary) thyroid adenoma, pheochromocytoma, and multicentric bilateral nodular hyperplasia of the adrenal medulla were present, findings that are remarkably similar to those of human MEN syndrome. Mortality during pregnancy in women with undiagnosed pheochromocytoma is high (approximately 50%), typically because of hypertension and/or hemorrhage associated with catecholamine release from the tumor. Similarly, the mare in this report died of hemorrhage subsequent to parturition. A retrospective evaluation of endocrine tumors in horses that underwent necropsy at the Veterinary Medical Teaching Hospital from 1987 to 1997 was undertaken to identify additional possible cases of MEN in horses. Data from this retrospective evaluation suggest that coexistence of hyperplasias and neoplasias of the thyroid and adrenal glands, similar to MEN syndrome of humans, also occurs with some frequency in the horse.

Adenoma↗

Fine-needle aspiration cytology of adrenal masses in noncancer patients: clinicoradiologic and histologic correlations in functioning and nonfunctioning tumors.

BACKGROUND: Image-guided, fine-needle aspiration (FNA) cytology is performed currently in patients with malignant disease who have suspected adrenal metastases. The objective of this study was to evaluate the usefulness and safety of FNA cytology in patients with incidental adrenal masses and functioning tumors. METHODS: Computed tomography (CT)-guided or ultrasound-guided aspirates using 21-23-gauge needles were performed successfully in 70 patients with functioning (n = 38 patients) and nonfunctioning (n = 32 patients) adrenal masses (median size, 4 cm; range, 3-12 cm) that were detected previously by CT scans. RESULTS: Definitive histology was available in 68 patients (97.1%), showing 53 benign tumors (77.9%), 11 primitive malignant tumors (16.2%), and 4 unsuspected adrenal metastases (5.9%) in patients with unknown primary tumors. In two patients with aspirate reports that ruled out malignancy, the mass was unchanged on CT scan follow-up; thus, they were considered benign lesions. The benign masses were significantly smaller (P < 0.01), although seven malignant tumors (46.7%) measured 3-4 cm in greatest dimension, and eight benign lesions (14.5%) measured 5-6 cm in greatest dimension. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 66.7%, 96.4%, 83.3%, 91.4%, and 90.0%, respectively, for CT scan and 93.3%, 100%, 100%, 98.2%, and 98.6%, respectively, for FNA cytology. The morbidity rate of image-guided FNA cytology was 4.3% (two patients with self-limited, asymptomatic pneumothorax and one patient with spontaneously resolved adrenal hematoma). CONCLUSIONS: Adrenal FNA cytology represents a safe and specific procedure for evaluating patients with adrenal masses measuring > 2 cm in greatest dimension. FNA is able to reveal malignancies and unsuspected pheochromocytomas and should be performed in all patients with adrenal tumors whenever requested for surgical planning.

Adolescent↗

[Myelolipoma of the adrenals--a chance sonographic finding].

A markedly echogenic and sharply marginated suprarenal mass was found incidentally during an abdominal sonography in the case of three patients. One female patient died due to a mesenterial infarct, in the other cases the tumors were removed surgically. In two cases the histological diagnosis indicated a myelolipoma of the adrenal gland, in one case an angiolipoma attached directly to the adrenal. Myelolipoma is a benign and hormone-inactive tumorous lesion, which most commonly occurs in the adrenal gland. Knowledge of the clinical and sonomorphologic characteristics along with computed tomography helps to distinguish other tumors of the retroperitoneum. Thus, unnecessary surgical intervention can be avoided.

Adrenal Gland Neoplasms↗

Ultrastructure, steroidogenic potential, and energy metabolism of the Snell adrenocortical carcinoma 494. A comparison with normal adrenocortical tissue.

Electron microscope studies were carried out with the adrenocortical carcinoma 494 and normal adrenal cortex tissue. The mitochondria of the tumor cells showed marked differences when compared with mitochondria from fasciculata cells of the normal adrenal cortex. These differences were primarily related to mitochondrial number and crista structure. Corticosterone production in isolated tumor cells was extremely low and neither ACTH nor dibutyryl cyclic AMP had any stimulatory effect. Normal adrenal cells showed at least a tenfold increase under identical conditions. In the presence of corticosteroid precursors the amount of corticosterone produced by the tumor cells was much less than that produced by normal cells. The results indicate a reduced capacity for 11beta-hydroxylation in the tumor mitochondria and a possible reduced capacity for biosynthetic steps before the 11beta-hydroxylation reaction. Glycolysis in isolated tumor cells was also lower than in normal cells. Isolated tumor mitochondria oxidized succinate normally with a good degree of coupling with phosphorylation. However, unlike normal adrenal mitochondria, the tumor mitochondria showed little or no oxygen uptake with other Krebs cycle substrates. These data suggest that the tumor mitochondria may be lacking in the flavoprotein dehydrogenases responsible for the oxidation of NADH and NADPH, although other components of the respiratory chain may be intact.

Adrenal Cortex↗

The role of computed tomography in assessment of tumoral pathology of the adrenal glands.

The findings at computed tomography (CT) of the adrenal glands in 58 patients are presented. Most patients (50) were referred for suspected adrenal dysfunction. In three, adrenal masses were suspected from other radiological studies. Three patients had palpable abdominal masses, and incidental adrenal metastases were found in two. Pathological findings included pheochromocytomas (nine patients), adrenocortical adenomas (six), adrenocortical carcinomas (three), metastases (two), and one example each of myelolipoma, hemorrhagic renal cyst, and probable granuloma. Of 24 adrenal masses, 23 were clearly apparent in the scans and one was suspected. The smallest tumor diagnosed was 0.5 cm in diameter. There was on false positive diagnosis of a small tumor and no known false negatives. Because of their consistent location and unusual shape and because they are usually surrounded by retroperitoneal fat, the adrenal glands are ideal organs for study by CT, a highly accurate method for detecting and excluding mass lesions that may render arteriography and venography unnecessary. Surgery was performed in five patients with tumors following localization by CT only. Limitations and potential diagnostic pitfalls in interpretation are discussed.

Adolescent↗

The radiological diagnosis of adrenal tumours.

The radiological diagnosis of adrenal tumours is reviewed with special reference to the literature and the author's personal experience. The clinical presentation varies widely with the different pathology and radiological investigation is decided largely by clinical assessment. The various methods of investigation are related to the individual problems with special reference to arteriography and phlebography. Thirteen cases of Conn's tumour have been diagnosed by adrenal phlebography and 18 cases of phaeochromocytoma have been investigated by arteriography. These cases are reviewed in detail.

Addison Disease↗

Magnetic resonance imaging of neuroblastoma with a 0.15-T magnet.

Magnetic resonance imaging (MRI) in nine children with neuroblastoma showed that MRI can effectively demonstrate primary and metastatic disease and aid in predicting tumor resectability. MRI can show changes in tumor size and intensity in response to chemotherapy or radiation therapy. Neuroblastomas have an image intensity much lower than that of liver and muscle on inversion-recovery scans. On spin-echo 500/30 scans, the tumors have a greater intensity than muscle and a similar or slightly greater intensity than liver. On spin-echo 1000/60 scans, they are always of greater intensity than liver or muscle. At this stage, the overall accuracy of MRI as compared with other imaging methods is not known.

Adrenal Gland Neoplasms↗

Evaluation of adrenal masses in patients with bronchogenic carcinoma using 18F-fluorodeoxyglucose positron emission tomography.

OBJECTIVE: The purpose of this study was to assess the usefulness of positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) when differentiating benign from metastatic adrenal masses in patients with bronchogenic carcinoma. SUBJECTS AND METHODS: For our prospective study, any patient presenting to our institution with pathologically proven bronchogenic carcinoma and an adrenal mass was eligible. Thirty-three adrenal masses (mean size, 3 cm; range, 1-9 cm) in 27 patients were revealed by CT. PET was performed in all 27 patients and interpreted as positive when FDG uptake in the adrenal mass was greater than background activity or negative when FDG uptake in the adrenal mass was equal to or less than background activity. In addition, semiquantitative analysis was performed by computing a standardized uptake ratio. All studies were reviewed independently by three radiologists and then correlated with biopsy and CT findings. Specificity and sensitivity for determining metastatic disease to the adrenal gland were calculated. RESULTS: FDG uptake was positive (abnormally increased) in 25 adrenal masses. Twenty-three (92%) of the 25 masses were metastatic disease. The mean standardized uptake ratio of these was 6.28 (range, 3.22-14.41). The remaining two masses (8%) that had positive FDG uptake showed no tumor at percutaneous biopsy. The standardized uptake ratio values for these two masses were 3.0 and 3.7. FDG uptake was negative (normal) in eight adrenal masses. All these lesions were benign as proven by biopsy (n = 2) and CT attenuation values of less than 10 H (n = 6). The mean standardized uptake ratio value for these eight lesions classified as benign was 1.77 (range, 0.93-3.70). The sensitivity for detecting metastatic disease was 100%, and the specificity was 80%. CONCLUSION: PET with FDG is an accurate, noninvasive way to differentiate benign from metastatic adrenal masses in patients with bronchogenic carcinoma.

Adrenal Gland Diseases↗

[A case of malignant lymphoma with bilateral adrenal involvement].

Since the adrenal gland is a common site of metastatic disease, it is sometimes difficult to find the primary lesion only by diagnostic imaging. We report a case of non-Hodgkin's lymphoma with bilateral adrenal involvement, in which percutaneous needle biopsy was useful to determine the histology.

Adrenal Gland Neoplasms↗