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1/111. Primary aldosteronism with aldosterone-producing adrenal adenoma in a pregnant woman.

    A 30-year-old pregnant woman complained of muscle weakness at 29 weeks' gestation. She was hypertensive with severe hypokalemia. Lower plasma renin activity and higher aldosterone level than the normal values in pregnancy suggested primary aldosteronism. A cesarean delivery was performed at 31 weeks' gestation because of pulmonary congestion. The neonatal course was uncomplicated. The laparoscopic adrenalectomy for a 2.0-cm right adrenal adenoma resulted in normalizing of her blood pressure and serum potassium level. Although primary aldosteronism is rare, especially during pregnancy, it should be always considered as one of etiologies of hypertension in pregnancy.
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keywords = adrenal
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2/111. myocardial infarction and death after caesarean section in a woman with protein s deficiency and undiagnosed phaeochromocytoma.

    We describe the case of a 36-year-old woman, with a previous history of recurrent abortion due to protein s deficiency, undergoing an elective Caesarean section at 39-weeks gestation. During pregnancy no signs of hypertension or cardiovascular disease were reported, but at the end of the surgical procedure, the patient developed acute hypertension, leading to myocardial infarction, severe heart failure and death. The autopsy revealed a 2-cm undiagnosed phaeochromocytoma in the right adrenal gland. Clinical diagnostic features of phaeochromocytoma during pregnancy as well as the main therapeutic approaches suggested in the literature are discussed.
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ranking = 0.50954733725632
keywords = adrenal gland, adrenal, gland
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3/111. pheochromocytoma and sub-clinical Cushing's syndrome during pregnancy: diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy.

    The coexistence of pheochromocytoma and primary adrenal Cushing's syndrome of the same adrenal gland has rarely been reported. We describe here the case of a female patient presenting with mild Cushing's stigmata, hypertension and diabetes mellitus in whom we diagnosed a pheochromocytoma of the left adrenal gland with coexisting non-ACTH-dependent cortisol hypersecretion. While hormonal work-up was still in progress, the patient became pregnant and wanted to carry her pregnancy to full-term. A laparoscopic adrenalectomy in the 17th week of gestation was decided upon and the patient accordingly prepared for surgery by pre-treatment with phenoxybenzamine. Successful surgery--the first ever reported laparoscopic resection of a pheochromocytoma in pregnancy--without perioperative complications was performed under general anesthesia, with the patient receiving peri- and post-operative hydrocortisone substitution. Pathohistological examination revealed a pheochromocytoma with positive immunostaining for interleukin-6 (IL-6) and negative immunostaining for ACTH, vasoactive intestinal polypeptide (VIP) and cytochrome P450, and with no signs of malignancy. A paracrine stimulation of the ipsilateral adrenal cortex by IL-6 produced by the pheochromocytoma, leading to cortical hyperplasia and subclinical Cushing's syndrome, is suggested by the positive immunostaining for IL-6 and the MRI findings. Post-operatively, secondary adrenal insufficiency ensued, necessitating continuing hydrocortisone replacement over 12 months. hypertension resolved after surgery, and diabetes after the uncomplicated vaginal delivery at term.
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ranking = 2.352428007846
keywords = adrenal gland, adrenal, gland
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4/111. diagnosis and management of pheochromocytoma during pregnancy: a case report.

    pheochromocytoma is known to increase morbidity and mortality. We describe a case of pheochromocytoma during pregnancy. A patient was transferred to our hospital during gestational week 15 with severe hypertension, acute pulmonary edema, and cardiomyopathy. One day after transfer, she had a spontaneous abortion of the fetus. One week after hospital transfer, she developed acute dyspnea, supraventricular tachycardia degenerating into ventricular tachycardia, and respiratory failure requiring mechanical ventilation. pheochromocytoma caused by a right adrenal mass was diagnosed. The patient was treated with titrated doses of phenoxybenzamine, intravenous nicardipine, and metyrosine over a period of 3 weeks with resultant stabilization of her blood pressure. She underwent a successful right adrenalectomy 1 month after her initial presentation. Four months after surgery, all antihypertensive medications were discontinued and her blood pressure remained stable 1 year after the surgery. This case describes the maternal morbidity and fetal mortality that may be associated with pheochromocytoma during pregnancy.
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keywords = adrenal
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5/111. Primary aldosteronism caused by aldosterone-producing adenoma in pregnancy--complicated by EPH gestosis.

    pregnancy in conjunction with primary aldosteronism is an unusual occurrence. We report a 28-year-old woman who presented with mild hypertension and hypokalemia as manifestations of primary aldosteronism caused by an aldosterone-producing adenoma in the left adrenal gland during pregnancy. Although the diagnosis was straightforward, the patient refused to undergo the proposed operation during the second trimester of her pregnancy. She was not admitted to hospital until she developed EPH gestosis in the 27th week of gestation, which had an unfavourable outcome for the infant who died nine days after delivery. The patient underwent a laparoscopic adrenalectomy which resulted in normalization of blood pressure and blood potassium levels. In cases of aldosterone-producing adenoma, surgery in the second trimester is the most appropriate option to avoid a poor obstetric outcome.
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ranking = 0.67621400392299
keywords = adrenal gland, adrenal, gland
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6/111. Congenital adrenal hyperplasia associated with maternal pregnancy luteoma and the Antley-Bixler syndrome.

    The authors report on a child with indifferent external genitalia consisting of severe micropenis with penile urethra leading to the tip of the glans and bilateral cryptorchidism. Diagnostic workup findings showed a female karyotype, homozygous 21-hydroxylase deficiency, and excessive testosterone exposure prenatally as a consequence of maternal pregnancy luteoma, altogether causing this unusual phenotype. In addition, the girl suffered from skeletal anomalies consistent with the diagnosis of Antley-Bixler syndrome. Our case shows that, although the association of congenital adrenal hyperplasia with other syndromes is rare, and even if other possible reasons for in utero virilization are present, complete diagnostic workup including karyotyping and hormonal status should be done in all patients with ambiguous genitalia, especially in cases of an unusual phenotype. The authors report on the diagnostic procedures and discuss the surgical approach in this particular case, never described before in the literature.
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ranking = 0.83333333333333
keywords = adrenal
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7/111. pheochromocytoma with negative urinalysis in pregnancy.

    We present a rare case of adrenal pheochromocytoma in pregnancy, with serial 24-h urine specimen collections showing normal concentrations of catecholamine metabolites. The diagnosis was based on clinical presentation, abdominal ultrasound, and magnetic resonance imaging, and was confirmed on post-operative pathohistological examination. Clinical suspicion of pheochromocytoma in pregnancy should be sufficient to implement adequate therapeutic measures, regardless of urine catecholamine concentrations.
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ranking = 0.16666666666667
keywords = adrenal
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8/111. Laparoscopic adrenalectomy for primary hyperaldosteronism during pregnancy.

    Laparoscopic adrenalectomy was performed early in the second trimester of pregnancy in a woman with an aldosteronoma causing hypertension (254/154 mm Hg). The patient was later delivered of a healthy baby. With suitable precautions and timing, major laparoscopic surgery can be performed safely during pregnancy.
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ranking = 0.83333333333333
keywords = adrenal
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9/111. Adenocarcinoid tumor of the ovary diagnosed during pregnancy. A case report.

    BACKGROUND: Adenocarcinoid tumors are uncommon neoplasms with dual morphology, showing components of a neuroendocrine tumor with carcinoid features and an adenocarcinomatous component composed of glands lined with mucin-containing cells, some of which are goblet type. CASE: A 36-year-old woman had a left adnexal mass found during the second week of pregnancy. Sonography showed it to be increasing in size and eventually to become associated with pelvic pain. During the 20th week of gestation, an exploratory laparotomy was performed, and the left ovary and fallopian tube were excised. A diagnosis of adenocarcinoma was rendered by intraoperative frozen section. A staging procedure was then performed that included removal of the contralateral adenexa, pelvic lymph node sampling, peritoneal biopsies and partial omentectomy. The vermiform appendix and gastrointestinal tract appeared unremarkable. The patient was discharged. Permanent sections of the left ovary revealed an adenocarcinoid tumor. CONCLUSION: While reports detail ovarian metastases of adenocarcinoid neoplasms from primary appendiceal and other gastrointestinal sites, this case, in the setting of a normal appendix and negative workup for an extraovarian origin, is the fourth documented one of a primary ovarian adenocarcinoid tumor and first diagnosed during pregnancy.
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ranking = 0.006043702670663
keywords = gland
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10/111. Solitary fibrous tumour of the adrenal gland associated with pregnancy.

    Solitary fibrous tumour (SFT), first described as a pleural lesion, has been reported in several extrathoracic sites over the past 10 years. We describe a SFT of the left adrenal gland incidentally discovered in a 23-year-old, 22-week pregnant woman and characterised by a rapid growth during the third trimester of pregnancy. Elevated serum and urinary levels of cortisol and elevated blood levels of delta 4 androstendione and 17-OH progesterone were observed. After spontaneous delivery, the patient underwent laparoscopic resectioning of the mass and of the left adrenal gland from which the tumour was apparently originating. The kidney was not involved, and no other abdominal tumours were found. Histological and immunohistochemical features were typical of SFT of pleura and other locations. Only one case of adrenal SFT is on record, and the adrenal gland is to be added to the long list of extrathoracic locations of SFT. The association with pregnancy was a previously unrecognised event in SFT. The focal expression of progesterone receptors in the tumour cells may be related to pregnancy. This observation prompted an analysis of steroid hormone receptors in SFT of classical sites (pleura). Two of five cases had focal progesterone receptors too, a finding which deserves further investigations in a much larger series of SFTs.
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ranking = 3.7334980274609
keywords = adrenal gland, adrenal, gland
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