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[Treatment of pheochromocytoma: changes in diagnosis and therapy].

Evident progress has been made in the treatment of pheochromocytoma. The results of hormone analysis became very accurate, the method for tumor localisation are non invasive and safe: with 131-I-MIBG, ultrasonography and CT-scan an exact preoperative localisation is possible without serious risks. Patients are prepared for the operation with alpha- and beta-blocking agents. Modern methods of anaesthesia with continuous monitoring of blood pressure, pulmonary pressure and cardiac output and a standardized operative procedure are essential. From 1965 to 1987 71 patients with a total of 87 catecholamine producing tumors have been operated. In all cases a transabdominal access was chosen. Biadrenal tumors were removed in 8 patients, multiple (7) tumors in 2 patients. The comparison of the 2 time intervals 1965 to 1976 and 1977 to 1987 showed a significant decrease of serious intra- and postoperative complications. Surgical specimens of 36 patients with pheochromocytoma were used for immunohistologic evaluation. Marked positivity was found in 44% of cases for calcitonin. The reaction for vasoactive intestinal polypeptide (VIP) was positive in 28% of cases. Somatostatin was not detected in any case, neuron-specific enolase (NSE) in all cases. 6 patients with malignant pheochromocytoma were treated with high doses of 131-I-MIBG, 4 other patients received a combined chemotherapy.

Adrenal Gland Neoplasms↗

Prospective evaluation of unilateral adrenal masses in patients with operable non-small-cell lung cancer. Impact of magnetic resonance imaging.

UNLABELLED: We designed a prospective study to evaluate the accuracy of magnetic resonance imaging in distinguishing a benign from a malignant adrenal mass in patients with otherwise operable non-small-cell lung cancer. METHODS: Potentially operable non-small-cell lung cancer was prospectively staged. If a unilateral adrenal mass was found by computed tomographic scanning, respiratory compensated and cardiac gated thin section magnetic resonance imaging of the adrenal glands was done. One radiologist interpreted the magnetic resonance imaging scan blinded and, on the basis of the relative signal strengths of the T1- and T2-weighted images, judged whether the adrenal mass was benign or malignant. The patients then underwent a percutaneous needle biopsy of the adrenal mass, if technically feasible. If the result of the needle biopsy was nondiagnostic or if the biopsy was not feasible, an adrenalectomy through a posterior approach was performed. RESULTS: Twenty-seven patients with a unilateral adrenal mass entered the study-11 men and 16 women whose ages ranged from 42 to 75 years (median 58 years). Four patients had epidermoid and 23 adenocarcinoma of the lung. The clinical locoregional stage was I in 9, II in 1, IIIA in 16, and IIIB in 1. Twenty-five completed the magnetic resonance imaging procedure. Five adrenal masses (19%) were metastatic non-small-cell lung cancer (adenocarcinoma = 4, epidermoid = 1); 22 masses (81%) were benign (adenoma = 20, hyperplasia = 2). There were no significant differences in age, sex, histologic type, or locoregional stage between those with a benign versus a malignant mass. However, the malignant masses were significantly larger (3.8 +/- 1.9 cm; range 2.5 to 7.1; median 3.1) than the benign masses (2.0 +/- 0.4 cm, range 1.2 to 2.8; median 2.0) (p < 0.001). Among those having magnetic resonance imaging (n = 25), the technique correctly predicted a malignant mass in the four patients with a histologically confirmed metastasis from non-small-cell lung cancer. However, among the 21 histologically benign masses, the magnetic resonance imaging was interpreted as benign in 5, malignant in 14, and indeterminate in 2. Therefore, although the false-negative rate was 0%, the false-positive rate was 67%. CONCLUSION: Most adrenal masses in patients with otherwise operable non-small-cell lung cancer are benign. Currently available magnetic resonance imaging methods cannot replace biopsy.

Adrenal Gland Diseases↗

[Magnetic resonance techniques used in the differentiation of adrenal tumors].

Noninvasive differentiation of adrenal masses by imaging studies as computed tomography and magnetic resonance imaging is an accepted diagnostic procedure in patients with extraadrenal neoplasm and discovered on imaging studies adrenal mass or in patients with incidentally discovered adrenal mass. Significant impact on possibilities of differentiation of adrenal masses with magnetic resonance imaging was due to introduction to adrenal imaging such techniques as chemical shift imaging and dynamic imaging with paramagnetic contrast agent. Differentiation of adrenal masses with magnetic resonance imaging involves following parameters: tumor diameter, signal intensity of adrenal mass on T2 weighted images, lipid contents in adrenal tumor evaluated with chemical shift imaging and washout of paramagnetic contrast agent from adrenal mass evaluated with dynamic examination. The best results, reaching 100% sensitivity and 100% specificity in differentiation adrenal adenomas from non-adenomas, can be achieved by combined analysis of all mentioned parameters. In regard to availability and costs computed tomography not magnetic resonance imaging is the examination of choice in visualization of adrenal glands and their pathology and in differentiation of adrenal masses. Adrenal tumors on non-enhanced images with density below 0 Hounsfield units can reliably be diagnosed as adenomas, whereas adrenal tumors with density above 20 Hounsfield units can reliably be diagnosed as non-adenomas. Tumors with density in 0-20 Hounsfield range are ambiguous in character, that in many cases can be determined in magnetic resonance imaging.

Adrenal Gland Neoplasms↗

Comparative and quantitative study of L-dopa decarboxylase mRNA in rat neuronal and non-neuronal tissues.

The L-DOPA decarboxylase mRNA levels were determined by a sensitive S1 nuclease method in four organs and one tumor of adult rat. S1 mapping analysis, with probes corresponding to the mRNA coding region, showed that this region is conserved in all L-DOPA decarboxylase mRNA of neuronal and non-neuronal tissues. The mRNA was not very abundant; its representation varies approximately from 0.00035% of total RNA in the mid brain to 0.013% of total mRNA in the pheochromocytoma. A strong correlation between mRNA level and enzyme amount was observed (correlation coefficient = 0.99). The results indicate that the level of mRNA is a primary factor determining the L-DOPA decarboxylase level.

Adrenal Gland Neoplasms↗

[Surgical therapy of sporadic and familial pheochromocytoma].

From 1975 through 1987, 54 patients underwent operations for pheochromocytoma (PCC): 37 patients for sporadic unilateral adrenal PCC, 9 for bilateral and familial PCC, and 4 patients for paraganglioma. In 4 additional patients, laparotomy failed to discover the reason for hypercatecholaminemia. There were no operative mortality and a low morbidity for unilateral (n = 43) as well as for bilateral adrenalectomy (n = 6). To avoid hormone replacement therapy after bilateral total adrenalectomy at least for some years, unilateral instead of bilateral adrenalectomy was preferred to be the initial surgical procedure of choice for familial and MEN-IIA-associated unilateral adrenal PCC. Autologous transplantation of the adrenal cortex were performed in 2 patients after bilateral adrenalectomy. 6 months postoperatively, the transplants were vital as proved by light-microscopy, but sufficiently functioning only in 1 patient.

Adolescent↗

[Long-term observations after adrenal surgery].

Long-term observations after adrenal surgery show different results according to indications for operations. The longest period of observation shows 8 patients with adrenal hyperplasia. In 3 of 4 bilaterally adrenalectomised patients no satisfactory results were obtained despite substitution treatment; 4 one-side adrenalectomised patients are in good condition. Operative results of 7 patients with benign phaeochromocytoma are satisfactory. Two patients with malignant phaeochromocytoma developed a local recidive and distant metastases. Carcinoma of the adrenal gland, if not hormonally active, were not diagnosed in early stage, 3 patients died from metastases within 3 years. A 2-years-old girl, who was operated on with hormonally active carcinoma, is in good condition 21 years later.

Adolescent↗

Non-Hodgkin's lymphoma with histological features of neoplastic angioendotheliosis.

Three cases of non-Hodgkin's lymphoma, B-cell type, identical histologically to so-called neoplastic angioendotheliosis (NAE), are reported. All showed a rapidly progressive clinical course and were not properly diagnosed before death. The lymphoma cells were distributed in many vessels of systemic organs with a tendency to form aggregates. The primary sites in the three cases were probably the adrenal gland, lymph node, and spleen, respectively. In particular, in one case it was suggested that the lymphoma cells had spread from the adrenal tumor to other organs, showing the histological features of NAE. In the literature, the primary sites of NAE have been scarcely mentioned. However, gross tumors were present in some cases. In such cases, it is possible that the tumors could be the primary sites of NAE. We conclude that some non-Hodgkin's lymphomas can exhibit the features of NAE during their course, particularly in the terminal stages.

Adrenal Gland Neoplasms↗

Class III beta-tubulin isotype (beta III) in the adrenal medulla: II. Localization in primary human pheochromocytomas.

BACKGROUND: The Class III beta-tubulin isotype (beta III) is expressed specifically in central and peripheral nervous system neurons at various stages of neuronal differentiation. We have shown previously that beta III is expressed in a differentiation-dependent manner in human neuroblastomas arising in the adrenal medulla and sympathetic chains (Katsetos et al., Clin Neuropathol 13:241-255, 1994). The neuronal distribution of beta III in the developing and mature human adrenal medullae is detailed in the companion article (Katsetos et al., 1998A). METHODS: We have compared the localization of the neuronal beta III to S-100 protein, a sustentacular cell marker, in 14 formalin-fixed, paraffin-embedded primary human pheochromocytomas of the adrenal medulla and 14 adrenocortical tumors (adenomas and carcinomas). RESULTS: In pheochromocytomas, beta III staining was present in all tumors, but the number of stained cells varied in the two neural neoplastic phenotypes. Although the majority of chromaffin-like cells were beta III-positive, there was a lack of beta III in one-third of the tumor cells. Compared to chromaffin-like phenotypes, neuronal (ganglion-like cells) were invariably beta III-positive. Stromal sustentacular cells, stromal fibroblasts, and tumor blood vessels were beta III-negative. Sustentacular cells in pheochromocytomas were S-100 protein-positive, but beta III-negative. Primary adrenocortical tumors were beta III-negative with the exception of rare beta III-positive cells demonstrated in one case. CONCLUSIONS: The distribution of beta III in human pheochromocytomas of the adrenal gland is differentiation-dependent, closely recapitulating chromaffin cell and neuronal phenotypes of the normal adrenal medulla. Our findings indicate that beta III may be used as one of the adjuvant neural markers in the differential diagnosis of adrenal tumors, i.e., pheochromocytoma versus adrenocortical carcinoma. The occurrence of rare beta III-positive cells in cortical carcinomas is exceptional and probably represents the acquisition of a divergent neuroendocrine phenotype. The significance of the latter is unclear, although it may constitute a marker for malignancy.

Adrenal Cortex Neoplasms↗