Cases reported "adrenocortical carcinoma"

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1/84. 11-Deoxycorticosterone-producing adrenocortical carcinoma.

    A woman presented with a history of weight loss and muscle weakness. A laboratory test revealed hypokalemia and elevation of plasma 11-deoxycorticosterone (DOC). CT showed a left adrenal mass. A left adrenalectomy was performed. The histological and immunohistochemical diagnosis showed a DOC-producing adrenocortical carcinoma. This cancer is very rare; only 10 cases including the present case have appeared in the literature. ( info)

2/84. Lack of response to octreotide in Cushing's syndrome due to metastatic adrenocortical carcinoma.

    Functional metastatic adrenocortical carcinoma is an uncommon cause of Cushing's syndrome, which rarely responds to conventional treatment. A patient presenting with Cushing's syndrome secondary to adrenocortical carcinoma underwent surgical resection. Postoperatively, she developed metastatic disease resistant to conventional chemotherapy. octreotide, a somatostatin analogue which is effective in the treatment of several types of neuroendocrine tumour, was tried to ameliorate her secretory symptoms, but without any therapeutic effect. ( info)

3/84. Anaesthetic management of a patient with adrenocortical tumour.

    Adrenocortical tumours in children are rare. They produce many changes in haemodynamics and blood chemistry due to hormones of the adrenal cortex. The details of perioperative management and the need for perioperative steroid supplementation are discussed. ( info)

4/84. Peritoneal carcinomatosis following laparoscopic resection of an adrenocortical tumor causing primary hyperaldosteronism.

    A clinical syndrome combining hypertension and hypokalemic alkalosis led to the diagnosis of primary hyperaldosteronism, caused by a right-sided, 2 cm large, apparently benign aldosterone-producing adenoma. The adrenal tumor was completely resected by laparoscopic adrenalectomy. Six months after surgery, the patient exhibited a severe relapse of hyperaldosteronism. Extensive peritoneal metastases of a mixed aldosterone- and cortisol-secreting adrenocortical carcinoma were found at abdominal laparotomy. In the light of this case report, we discuss the possibility that laparoscopic resection of adrenocortical tumors might contribute to their subsequent peritoneal dissemination. ( info)

5/84. Two cases of osteosarcoma occurring as second malignancy of childhood cancer.

    We report on two patients in whom osteosarcoma occurred as second malignancy of childhood cancer. One patient had a malignant teratoma and the other adrenocortical carcinoma as the primary cancer. The emergence of cancer in cured cases and long-term survival cases of childhood cancer may result in an increase in the number of osteosarcomas seen in adolescence occurring as second malignancy. Anti-cancer drugs in large does were used for the treatment of a malignant teratoma in the former. These anti-cancer drugs may be involved in the occurrence of the second malignancy. In the latter, the patient has the germ-line mutation of p53 tumor suppressor gene, so genetic factors are presumably related to the occurrence of the second malignancy. ( info)

6/84. Changes in neoplastic cell features and sensitivity to mitotane during mitotane-induced remission in a patient with recurrent, metastatic adrenocortical carcinoma.

    A 58-year-old man had adrenocortical carcinoma in the right adrenal gland. The tumour secreted excessive cortisol and dehydroepiandrosterone-sulphate (DHEA-S), and had invaded the right hepatic lobe and vena cava. Eleven months after surgical tumour resection, the serum DHEA-S levels again increased. Local tumour recurrence and a metastasis was found in the lung. Eleven months after surgery chemotherapy with mitotane (o,p'-DDD) was initiated. Twelve weeks of mitotane reduced serum DHEA-S levels and caused these tumours to disappear. The patient was then treated with low-dose mitotane (1.5-2.0 g/day) for 2 years. serum levels of mitotane remained at less than 10 microg/ml. Although such low serum levels of mitotane and delayed initiation of mitotane after surgery have been proposed to weaken the antineoplastic effect of mitotane, the patient had a remission for 2 years. However, there was then local re-recurrence with an increase in serum DHEA-S and death 4 months later. The histological features of neoplastic cells were quite different comparing tumour resected at surgery and tumour at autopsy. The latter had more frequent mitotic nuclei. This tumour was initially sensitive to mitotane, but later became insensitive. ( info)

7/84. Adrenal cortical carcinoma metastatic to the brain in a child.

    A 9-year-old girl presented with profoundly decreased vision in both eyes and bilateral optic disc swelling. magnetic resonance imaging showed a large intraventricular mass. Excisional biopsy diagnosed metastatic adrenal cortical carcinoma (ACC). This is, to the best of our knowledge, the first reported case of ACC metastatic to the brain in a child. It also illustrates the importance of differentiating optic neuritis from papilledema in children. ( info)

8/84. Fine-needle aspiration cytology of a case of oncocytic adrenocortical carcinoma.

    We report on the results of fine-needle aspiration cytology of a case of oncocytic adrenocortical carcinoma in a 39-yr-old man. The tumor invaded the inferior vena cava and extended up to the right atrium. Aspirate smears were very cellular and showed a monomorphic population of large polyhedral cells with abundant granular cytoplasm, predominantly distributed singly. Mitotic activity was inconspicuous, and there was no necrosis. Immunohistochemically, the tumor cells were positive for vimentin, cytokeratin, and p53, and negative for synaptophysin, chromogranin, inhibin, and S-100. Ultrastructurally, the cytoplasm of the tumor cells was packed with mitochondria. The patient underwent left radical nephrectomy as well as a combined cardiopulmonary bypass, with atriotomy and resection of the tumor from the right atrium and inferior vena cava. Three months of postoperative follow-up were uneventful. ( info)

9/84. Characterization of a newly established cell line derived from human adrenocortical carcinoma.

    BACKGROUND: ACT-1, a new cell line of human adrenocortical carcinoma, has been established and successfully maintained in culture. This study examined the biological characteristics of the cells. methods: The tumor cells were isolated from a surgical specimen of the tumor thrombus and cultured in monolayer. RESULTS: Histologically, the primary tumor was composed of a solid proliferation of large polygonal cells. A part of the atrophic adrenal cortex remained at the periphery of the tumor. The cultured ACT-1 cells were spindle-shaped in morphology and grew exponentially with an approximate population doubling time of 24 h. A chromosomal analysis revealed a modal number of 61 with consistent structural abnormalities of add(3)(q11), add(9)(p11), and add(16)(ql1). The expression of 3beta-hydroxysteroid dehydrogenase was observed in the ACT-1 cells as well as in normal human adrenal glands. Conclusions: The ACT-1 cell line provides a reproducible model system which gives good insight into the oncogenesis of adrenocortical carcinoma. ( info)

10/84. adrenocortical carcinoma with cerebral metastasis in a child: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: adrenocortical carcinoma (ACC) is rare in the pediatric population, and brain metastasis seldom occurs. CLINICAL PRESENTATION: The authors report a case of metastatic ACC to the brain in a 9-year-old patient who had an adrenal cortex neoplasm removed at 4 years of age, and was free of symptoms for 5 years. Two weeks before admission she complained of blurred vision in both eyes. INTERVENTION: Examination revealed bilateral papilledema, and a magnetic resonance imaging (MRI) of the brain revealed a mass in the left lateral ventricle with extensive vasogenic edema and hydrocephalus. The tumor was removed, and histopathologic examination demonstrated metastatic ACC. CONCLUSION: Although ACC is a rare neoplasm it must be considered in the differential diagnosis of cerebral lesions in patients with a history of this tumor. Periodic long-term brain imaging is suggested as part of the follow up in patients with adrenocortical neoplasms. ( info)
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