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[Diagnosis in progressive hirsutism. Catheter technique and results (author's transl)].

Catheterization of adrenal and ovarian veins in combination with hormone concentration analyses was performed in seventy hirsute women. There was suspicion of tumor in 30 cases because of excess androgen production (plasma testosterone more than 1,5 ng/ml and/or plasma dehydroepiandrosterone sulfate more than 7000 ng/ml in peripheral blood samplings). Catheterization and blood sampling was done with a femoro-visceral catheter by the transfemoral approach. Contributary blood flows from many different venous systems were found to be more often to the right ovarian and to the left adrenal veins in comparisons to the other side. Catheterization of the wrong vessels occurred without being recognized immediately (circumaortic renal collar, inferior phrenic vein, ascending lumbar vein). These pitfalls might falsify blood samplings. There were no significant complications. Seven ovarian tumors were localized. Their size ranged from 0,5 to 2,5 cm in diameter. They were not recognized during laparoscopy. There were 10 hyperthecoses, four adrenogenital syndromes, one abortive testis, one adrenal adenoma, one Cushing's syndrome and one Cushing's disease due to an adenoma of the pituitary gland. In five patients a diagnosis could not be made. Tumor was excluded in forty patients. Their virilization was of adrenal origin in 32%, of ovarian origin in 29%, and of both adrenal and ovarian origin in 22%. In 17% a source of the excess androgen production was not found. This differential diagnosis is not important for the patient therapy.

Adrenal Gland Neoplasms↗

Giant adrenal lipoma: a report of two cases and review of literature.

Lipoma of the adrenal gland is extremely rare. We report two cases of adrenal lipomas, one in a 64-year-old female and the other a 78-year-old male. The sizes of these tumours (4.5 cm and 8 cm) were larger than those reported in the literature. Both cases were found incidentally, one at autopsy and the other during ultrasonographic examination for unrelated condition. The histogenesis, clinicopathological features, differential diagnosis and management of these cases are discussed.

Adenocarcinoma↗

[An operated case of multiple endocrine neoplasia type IIb and review of the Japanese literature].

Multiple endocrine neoplasia, type IIb (MEN IIb) is a rare syndrome characterized by the occurrence of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuroma. A 35-year-old male patient with MEM IIb having megacolon, marfanoid habitus and no family history of the disease underwent surgery. Because MTC was present in both lobes, total thyroidectomy and modified neck dissection were performed. Pheochromocytoma was found bilaterally and bilateral adrenalectomy with adrenal autotransplantation in the rectus abdominis muscle was carried out. Postoperative course was satisfactory except for transient hypocalcemia and mild ileus. After the slow corticosteroid weaning process, his adrenocortical function was at the lower level within a normal range. In August 1986 (24 postoperative months), he was maintained by the administration of 10mg of hydrocortisone every three days, and calcitonin and CEA levels in sera were normal. We collected 15 cases reported in Japanese literatures. MTC and mucosal neuroma were found in all cases, whereas pheochromocytoma was present in 9 cases. Bilateral and multicentric occurrences were usual, and total thyroidectomy and bilateral adrenalectomy were, warranted. We believe that autotransplantation following bilateral adrenalectomy is a worthy alternative.

Adrenal Gland Neoplasms↗

[Catecholamine-producing tumors of the chromaffin tissue. Morphological and ultrastructural characteristics].

Histological and ultrastructural studies were carried out in 41 hypertensive patients showing increased urine excretion of catecholamines and vanilmandelic acid due to chromaffinoma of the adrenal and other sites: malignant chromaffinoma of the adrenal, including multiple--55%, border-line chromaffinoma--34% and malignant--11% (total--53 tumors). Ultrastructurally, malignant chromaffinoma showed pronounced aniso- and poikilocytosis, lack of desmosomes, presence of dark and clear cells, irregular and bizarre-shaped nuclei and enlarged nucleoli. An inverse correlation between the level of secretory granules and that of organelles was established for all sites and patterns of growth. The data obtained suggest certain ultrastructural peculiarities which alongside with macro- and microscopic features serve identification of malignity of tumor, functional status of tumor cells and type of secretion.

Adolescent↗

Selective venous catheterization in the evaluation of hyperandrogenism.

Retrograde bilateral ovarian-adrenal vein catheterization was carried out in 16 patients with plasma testosterone levels exceeding 1.4 ng/ml (4.85 nmol/l). While pelvic ultrasonography and computerized axial tomographic scan failed to locate the androgen-producing ovarian tumors, catheterization led to a diagnosis of occult ovarian tumor in 5 patients, based on the observation of an abnormally-high and unilateral ovarian-peripheral vein testosterone gradient, which was subsequently confirmed histopathologically. In one case, unilateral elevation of the adrenal-peripheral vein testosterone gradient was found, complementing the ultrasonographic finding of an adrenal mass and confirming the diagnosis of a virilizing adrenal tumor. In the other 10 patients, gradient analysis ruled out an androgen-producing tumor, leading to the identification of nontumoral hyperandrogeny, such as a severe form of the polycystic ovary syndrome in the 6 premenopausal patients and of ovarian stromal and hilus cell hyperplasia in the 4 menopausal patients. In conclusion, appropriate indication of selective catheterization may considerably reduce the need for exploratory surgery and may help in selecting the adequate surgical approach.

Adrenal Gland Neoplasms↗

[Endoscopic excision of extra-adrenal pheochromocytomas (paragangliomas)].

We describe the successful endoscopic removal of six extra-adrenal pheochromocytomas in three patients. One neoplasia was located retrocavally, two between the aorta and vena cava, two by the para-aortal, and one by the parailiacal. The tumors were removed by the posterior retroperitoneoscopic or by the anterior laparoscopic approach, respectively. The intraoperative and postoperative courses were uneventful.

Adrenal Gland Neoplasms↗

Salivary corticosteroids in the study of adrenal function.

Salivary corticosteroids (SCC) and plasma corticosteroids (PCC) were studied under basal conditions, after dexamethasone (DXM) and in the ACTH stimulation test in a reference group (RG) of 33 adults, in three groups with non-adrenal pathology and in a group of 4 patients with hypercortisolaemia. SCC and PCC were measured using a non-extraction RIA method using [3H]cortisol. The results for SCC in the RG and in the groups with non-adrenal pathology were similar to those obtained for PCC in terms of percentage of decrease in the circadian rhythm or DXM suppression. However, the responsiveness to ACTH in saliva was twice that obtained in plasma. In patients with hypercortisolism, SCC were in closer agreement with the adrenal hyperfunction than PCC. From the previous results the following conclusions may be drawn: (a) SCC differentiate adrenal gland normal function from hyperfunction as clearly or even better than PCC does; (b) SCC were in a closer agreement with the symptomatology of adrenal hyperfunction than were PCC; and (c) the responses to ACTH obtained with SCC were clearly higher than those obtained with PCC.

Adenoma↗

Monensin depletes PC12 pheochromocytoma cells of catecholamines and of chromaffin-type granules.

PC12 pheochromocytoma cell store dopamine in chromaffin-type granules. The carboxylic ionophore momensin depletes the cells of dopamine and of granules PC12 cultures incubated for 30 min with 100 nM monensin lose more than 90% of their dopamine. A small fraction of the dopamine that disappears from the cells is converted to dihydroxyphenylacetic acid. The remainder is presumably converted to other, unidentified metabolites. Monensin-treated cells contain many cytoplasmic vesicles. Some of these vesicles contain amorphous, electron-dense material, which may represent the matrix of disrupted granules. There is no morphological evidence of exocytosis. Thus, monensin appears to promote the intracellular lysis of chromaffin-type granules in PC12 cells.

Adrenal Gland Neoplasms↗

Endoscopic ultrasound (EUS) and EUS-guided fine-needle aspiration of the left adrenal gland.

BACKGROUND: We reported the application of endoscopic ultrasound (EUS) and EUS-guided fine-needle aspiration (FNA) in the local staging of lung cancer. Up to 16% of patients with lung cancer may have adrenal masses. The role of EUS in the characterization of the adrenal gland, as well as EUS-guided FNA of the adrenal gland, has not been described. METHODS: The adrenal gland was characterized by EUS in 30 of 31 (97%) patients. EUS-guided FNA of an adrenal was performed in one patient. Anatomic correlation and ex vivo images were obtained. RESULTS: The average dimension was 2.5 cm (range 1.4 to 3.5) x 0.8 cm (range 0.3 to 1.4) with two morphologic types: "seagull" and "elliptical." One patient with lung cancer and a left adrenal mass with a nondiagnostic CT-guided FNA underwent EUS-guided FNA, which established the diagnosis of metastatic adenocarcinoma. Surgery was avoided. CONCLUSIONS: The left adrenal can be imaged by EUS in almost all patients. EUS-guided FNA may be useful when applied to left adrenal metastatic tumors for tissue diagnosis.

Adenocarcinoma↗

Malignant pheochromocytoma: clinical course and treatment.

We describe 7 patients with 16 malignant pheochromocytomas, with followup for 7 to 21 years. Five patients are free of disease at a mean of 13.5 years and 2 patients died of malignancy at 10 and 13 years. The 3 criteria believed important for a maximum tumor-free interval are: 1) adjunctive lymphadenectomy at the initial operation when 1 or more lymph nodes contain tumor, 2) close followup of all patients with pheochromocytoma by diagnostic biochemical assay for 15 years and 3) an aggressive excision of all single or multiple recurrent pheochromocytomas as soon as a biochemical diagnosis is established.

Adolescent↗