Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present with signs of steroid hormone excess (e.g. Cushing's syndrome, virilization) or an abdominal mass. Tumour size at presentation (mean diameter at diagnosis > 10 cm) is the most important indicator of malignancy. In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media. Magnetic resonance imaging (MRI) is equally effective as CT and is particularly helpful to visualize invasion into large vessels. Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC. Despite tumour resection for cure most patients will eventually develop local recurrence or distant metastases. Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy). In tumour recurrence re-operation should always be considered. In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy. Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l. Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions). Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.
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PMID:Management of adrenocortical carcinoma. 1500 91

A 50-year-old woman underwent surgical resection of a left adrenocortical carcinoma in April 2000. Bilateral pulmonary metastases and abdominal lymph node metastasis were detected in June 2001. After radiation therapy for the abdominal lymph node metastasis, a pulmonary metastatic lesion was thoracoscopically resected. The specimen was subjected to histoculture drug response assay (HDRA), and results revealed that this tumor was sensitive for cisplatin. We therefore performed concurrent chemoradiotherapy including cisplatin for the residual pulmonary metastatic lesion; a complete response was then obtained. Standard protocols of chemotherapy are often absent for malignant tumors, such as in this case, with low incidences. HDRA seems useful for chemotherapy agent selection in cases of rare malignant tumors.
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PMID:[Histoculture drug response assay guided concurrent chemoradiotherapy for lung metastasis from adrenocortical carcinoma--a case report]. 1504 57

Adrenal cortical carcinoma (ACC) is a rare and highly malignant tumour with up to 70% of the patients diagnosed at an advanced clinical stage, up to 40% presenting with metastases. Even after complete surgical excision, up to 80% of the patients show locoregional recurrence or metastases. We report a case of a 62-year-old woman with a non-functional ACC of the left adrenal gland (T2N0M0 classified as stage II). After the initial resection, 3 operations for metastasis of the contralateral adrenal gland and 4 operations for metastasis of the lungs were carried out, allowing survival for more than 28 years with a good quality of life. This case report emphasises the need for careful clinical and radiographic follow-up. A repeat surgical approach should be adopted whenever possible providing long-term survival over decades.
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PMID:Long-term survival over 28 years of a patient with metastatic adrenal cortical carcinoma--case report. 1527 73

Oncocytic adrenocortical carcinoma is rare. To our knowledge, only 6 cases have been previously reported. We describe an additional case of oncocytic adrenocortical carcinoma. A 54-year-old man presented with a right subcostal mass. Computed tomography demonstrated a massive tumor in the right abdomen. Because renal or adrenal cancer was suspected, right adrenalectomy and nephrectomy were performed. Histologic examination revealed an oncocytic adrenocortical carcinoma. Five months postoperatively, multiple metastases had developed and were treated with surgical resection, chemotherapy, vascular embolization, and radiotherapy. At last follow-up, the patient was alive with pulmonary and adrenal metastases and undergoing treatment with mitotane.
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PMID:Oncocytic adrenocortical carcinoma. 1530 3

In this article we review the management of benign and malignant adrenal tumors, with an emphasis on oncologic concerns. Concise, logical guidelines for the diagnosis and operative treatment of incidentalomas, aldosteronomas, adrenal Cushing syndrome, virilizing and feminizing adrenal tumors, isolated adrenal metastases, and adrenocortical carcinoma are provided. We also discuss the choice of optimal surgical approach for performing adrenalectomy (laparoscopic, open, hand-assist).
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PMID:From incidentaloma to adrenocortical carcinoma: the surgical management of adrenal tumors. 1571 74

Aldosterone-producing adrenocortical carcinoma (APAC) is a rare cause of hypertension often diagnosed late because of paucity of information. Thus, we delineated its clinical course and survival rates based on two cases referred to us that featured diverging clinical courses, and on a scrutiny of the literature since 1955 when the first case of APAC was identified. Data on demography, imaging results, hormonal assessment, histology, and clinical course were extracted independently by the investigators. We included in our database 58 cases, most presenting with Conn's syndrome. Plasma aldosterone levels were on average increased 14-fold; plasma renin activity was suppressed in 55% of cases. The tumor showed extremely variable size and weight, and no gender or side preference. Metastases were present in 10% of all cases at initial diagnosis and in an additional 48% of cases at follow-up. Median survival was 546 days (95% confidence interval (CI): 240-851); median time to either recurrence or death was 212 days (95% CI: 29-395). No clinical or histological signs predicted survival with Cox regression analysis. We concluded that, although an ominous course with a poor survival rate is common, no sign accurately predicts the course of APAC. Thus, molecular studies to identify diagnostic markers of survival are mandatory.
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PMID:Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn's syndrome with an ominous clinical course. 1578 46

A 37-yr-old man presented with the classic signs of mineralocorticoid excess hypertension and hypokalemia. The cause was not aldosterone excess, but elevation of plasma 11-deoxycorticosterone (DOC). Computed tomography (CT) scans showed a large right adrenal mass without signs of metastatic disease. The tumor was removed by open laparotomy, and histology revealed an adrenocortical carcinoma. Two yr after diagnosis, the patient is in good general condition and there is no sign of recurrence or metastatic disease, despite the large tumor size. DOC producing adrenocortical carcinomas causing mineralocorticoid hypertension are very rare, so far only 10 cases have been described in the literature.
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PMID:Adrenocortical carcinoma producing 11-deoxycorticosterone: a rare cause of mineralocorticoid hypertension. 1581 73

Adrenocortical carcinoma (AC) is a rare tumor of poor prognosis. Its treatment by o,p'DDD remains a reference after initial surgery. Two galenic forms were recently available in France: Lysodren and Mitotane AP-HP. As Lysodren got a European registration in april 2004, Mitotane is no more produced. O,p'DDD is an adrenolytic and cytotoxic agent. It also reduces the hormonal secretion in AC. Its blood level must reach a therapeutic window (14-20 mg/l) to be effective and to limit toxicity. It is given orally three times a day (3 grams a day for Lysodren and 6-12 grams a day for Mitotane. Its posology is adapted according to serum levels of o,p'DDD and tolerance. Side effects are essentially gastrointestinal (GI), neurologic and hepatic. Five patients (four with AC and one with a metastatic Leydig cell tumor of the testis) were treated by Lysodren. Three patients had early Lysodren discontinuation due to toxicity (skin rash, weight loss, GI toxicity). Studies of combination with other treatments as chemotherapy and targeted drugs are warranted. Surgery is an important part of metastatic disease treatment.
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PMID:[Practical use of o,p'DDD in adrenocortical carcinoma]. 1582 Sep 22

Sheehan's syndrome frequently causes hypopituitarism either immediately or after a delay of several years, depending on the degrees of postpartum ischemic pituitary necrosis. A 55 year-old woman whose last child was born 27 yr ago with massive hemorrhage was diagnosed as postpartum hypopituitarism. She had deficiency of growth hormone, prolactin, gonadotropins and thyrotropin. However, she interestingly had apparent hypercortisolism without suppression response to the dexamethasone tests. We found an adrenal mass with distant metastases to the liver and lung while investigating the origin of the hypercortisolism. Hyperandrogenism and very high levels of 17alpha hydroxyprogesterone were present. Accordingly, the patient was diagnosed as hypopituitarism due to Sheehan's syndrome accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma.
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PMID:A case of postpartum hypopituitarism accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma. 1586 51

We report a rare case of primary aldosteronism due to an adrenocortical carcinoma. A 61-year-old woman with a history of hypertension and hypokalemia was referred for evaluation of a 4.2 cm measuring adrenal mass without secondary signs of malignancy. Endocrinological testing was consistent with primary aldosteronism. The patient underwent surgical resection of the adrenal mass; histology revealed an adrenocortical carcinoma. Postoperatively blood pressure, serum potassium, and aldosterone returned to normal. Four months after adrenalectomy, the patient presented again with hypokalemic hypertension and was found to have metastatic disease. Endocrinological investigation revealed primary aldosteronism and subclinical autonomous glucocorticoid hypersecretion. Careful hormonal investigation should be obtained in patients with adrenal masses causing excessive aldosterone secretion. In uncertain cases of primary aldosteronism, we would suggest to measure 18-hydroxycortisol levels, as excessive amounts may indicate adrenocortical carcinoma.
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PMID:Steroid profile in an adrenocortical carcinoma producing aldosterone. 1589 61


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