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1/56. 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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2/56. 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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3/56. 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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4/56. 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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5/56. 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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6/56. Term delivery following conservative treatment for villoglandular papillary adenocarcinoma of the uterine cervix: report of a case and analysis of the literature.

    BACKGROUND: Villoglandularpapillary adenocarcinoma (VPA) of the cervix is often indolent, and surgical treatment has a favorable outlook. risk factors include depth of invasion, lymphovascular invasion, and the presence of other histologic types of cancer. CASE: An amputation of the cervical portio was required to satisfactorily resect a 2.5-cm ectocervical lesion in a 28-year-old nulligravida. A diagnosis of pure VPA with a depth of invasion less than 2 mm was established. During a subsequent pregnancy, second trimester ultrasound showed extreme effacement of her cervix and an abdominal cerclage was placed. The pregnancy continued until delivery of a healthy infant at 36 weeks. CONCLUSIONS: In cases of tumor invasion less than 3 mm, and in the absence of lymphovascular space involvement, extrauterine spread of pure VPA has not been described. When conservative treatment is planned, amputation of the cervical portio may be better suited than conization to the achievement of an adequate margin of resection. Cervical cerclage may be needed to offset the extensive cervical surgery.
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7/56. Massive bleeding from an ectopic lingual thyroid follicular adenoma during pregnancy.

    Ectopic thyroid is a rare developmental anomaly. It can be found anywhere between the foramen cecum and the normal position of the thyroid gland. Massive bleeding from an ectopic lingual thyroid is unusual and occasionally fatal. We present a case of a 22-year-old woman who had a large mass at the base of the tongue for over 6 years. In the sixth month of pregnancy, the patient experienced massive bleeding from the tongue base mass. A thyroid scan revealed that this mass was the only functioning thyroid tissue. Because of massive bleeding and her shock status, the patient received an emergent embolization of the bilateral lingual arteries. Then the huge lingual thyroid was subsequently excised via a mandible swing approach to prevent further episodes of bleeding. pathology analysis indicated ectopic thyroid tissue with follicular adenoma. She delivered without complications in the 36th week and had a normal baby. This case was a very rare one in our review. Although ectopic lingual thyroid usually is not managed surgically, excision of ectopic lingual thyroid can be life-saving when it is causing bleeding or airway obstruction.
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8/56. Carcinoma adenoides cysticum as a primary neoplasm most probably derived from biliary ducts--case report.

    Adenoid cystic carcinoma is a typical tumor of large and small salivary glands and sometimes of the breast. The aim of this report is presentation of a case of primary liver cancer with adenoid cystic cancer histology in 21-year old female patient. The tumor was discovered accidentally during pregnancy. The patient did not complain of any ailments. In biochemical tests only slightly elevated bilirubin, alkaline phosphatase and GGT concentrations were observed. No focal lesions in other organs were found in imaging examinations. Huge tumor of 30-cm diameter was found during surgery, encompassing almost whole left and right lobe of the liver. The biopsy taken from the tumor revealed histological picture typical for adenoid cystic carcinoma. Additional stainings and immunohistochemical examinations pointed to biliary ducts as possible place of origin of the neoplasm. SUMMARY: Among primary liver tumours of atypical clinical course the possibility of adenoid cystic carcinoma, deriving most probably from the biliary ducts, should be considered.
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9/56. Androgen-secreting adrenal adenomas.

    BACKGROUND: The androgen source in women with hirsutism and signs of virilism may be the ovary or adrenal gland. CASES: Three patients with androgen excess are reported. Two had hyperandrogenemia and cushing syndrome with an adrenal mass greater than 5.5 cm; the third had a small adrenal adenoma secreting only testosterone and responsive to human chorionic gonadotropin. In all cases, the pathologic report from surgery and the long-term resolution of symptoms confirmed the benign nature of the tumors. CONCLUSION: Basal and dynamic hormonal tests cannot precisely differentiate ovarian from adrenal tumors. Adrenal adenomas must be considered as a cause of hyperandrogenic syndrome.
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10/56. Lacrimal gland tumors in a medical center.

    Between 1971-2001, there were 9 cases of lacrimal gland tumors in the hospital medical records, 3 adenocystic carcinomas, 1 adenosquamous, 3 benign mixed tumors and 2 malignant mixed tumors. Only 4 had complete clinical histories: 1 benign mixed tumor and 3 adenocystic carcinomas. Among the 4 patients, 3 of them had the tumors removed by transeptal techniques and only I had complete removal of the tumor in a single mass. The histopathology proved to be a benign mixed tumor and was completely cured. The other 2 were adenocystic carcinomas which could not be completely removed, resulting in recurrences of the tumors and distant metastases. Both of them died from intracranial extensions. In 1 case of adenocystic carcinoma, the tumor was removed easier by the lateral orbitotomy technique. The nature of this kind of tumor must be known for accurate diagnosis, proper plan of the management and prognosis of the patient.
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