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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In reviewing the experience of a number of authors and investigators, it is clear that early diagnosis of adrenocortical carcinoma is essential for cure. Of all the modalities of therapy currently available, surgical resection holds the most promise for cure or prolonged survival. Treatment for extensive local disease or metastatic disease has been discouraging, and the prognosis for reasonable, comfortable survival is poor. Unfortunately, the toxicity of mitotane, an adrenolytic agent and currently the most effective drug available, is often unacceptable and may militate against its use. Because many of the debilitating side effects of these tumors are related to hormone production, newer drugs that result in hormonal blockade may add considerably to the comfort of the patient. The development of less toxic chemotherapeutic agents presents a challenge for both the oncologist and the endocrinologist.
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PMID:Adrenocortical carcinoma. 266 72

The records of 110 patients (58 females and 52 males) with histologically proven adrenal cortical carcinoma were studied. Fifty-six of 110 (50.9%) patients had abdominal symptoms and 33 (30.0%) had a palpable abdominal mass. Surgery for localized and regional disease was associated with a disease-free survival time of at least 2 years in 28 of the 50 (56%) patients. Abdominal radiotherapy, systemic chemotherapy, and 1,1 dichloro-2 (O-chlorophenyl)-2 (P-chlorophenyl) ethane (OPDDD) were effective in three of 19 (15.8%) patients, three of 31 (9.7%) patients, and 21 of 72 (29.2%) patients, respectively. Combined radiotherapy and OPDDD did not confer any additional benefit over OPDDD alone. Six of seven patients who received OPDDD as adjuvant treatment are still alive at 1 to 4 years. Distant metastases occurred most commonly in the lungs, liver, peritoneum, lymph nodes, and bones. Only 23% of the patients survived 5 years or more. We conclude that early diagnosis and complete surgical excision offer the best prospects for long-term survival and that the efficacy of adjuvant OPDDD needs to be evaluated further.
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PMID:Adrenal cortical carcinoma. 274 69

A 47-year-old female presented with hypertension, hypokalaemia, low plasma renin, high plasma aldosterone and was found to have a left adrenal tumour 4 cm in diameter by computerized tomography. Detailed biochemical studies showed high plasma levels of 11-deoxycorticosterone and corticosterone in addition to aldosterone and 18-hydroxycorticosterone. Basal 11-deoxycorticosterone levels were particularly high. Corticosterone, 18-hydroxycorticosterone and aldosterone concentrations were abnormally sensitive to infusions of ACTH and angiotensin II. Plasma cortisol and assays for sex hormones were normal although there was evidence that cortisol derived from the neoplasm. At operation a well-differentiated adrenocortical carcinoma weighing 50 g (56 X 30 X 36 mm) was removed. There was no evidence of metastases following surgery. Adrenal function returned to normal. Review of the literature suggests that adrenocortical carcinoma should be suspected in patients who otherwise have typical features of Conn's syndrome, but whose tumours are more than 3 cm in diameter. Measurement of steroids such as 11-deoxycorticosterone in addition to aldosterone is recommended since abnormally high values may also help to distinguish between hyperaldosteronism due to adenoma and carcinoma. Previously reported cases of isolated aldosterone production by a carcinoma cannot be substantiated.
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PMID:Hypermineralocorticoidism due to adrenal carcinoma: plasma corticosteroids and their response to ACTH and angiotensin II. 282 95

Between 1936 and 1987, 82 patients with adrenal cortical carcinoma were seen at our clinic. Of these patients 49 (72 per cent) have been seen during the last 25 years. A total of 40 patients (48.8 per cent) presented with a hormonally functional tumor and 42 (51.2 per cent) had a nonfunctional tumor. Forty patients (48.8 per cent) presented with localized disease, 12 (14.6 per cent) with regional disease and 30 (36.6 per cent) with distant metastases. Complete surgical removal of all gross tumor was achieved in 49 patients. Over-all 3 and 5-year patient survival rates in this series were 37.5 and 25.1 per cent, respectively. Survival was significantly improved (43.9 per cent at 5 years, p equals 0.0001) in patients with localized disease that was completely removed surgically; postoperative adjuvant therapy with op'-DDD was of no benefit in these patients. Survival in patients with metastatic disease was poor and was not improved by treatment with op'-DDD, cytotoxic chemotherapy or radiation therapy.
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PMID:The Cleveland Clinic experience with adrenal cortical carcinoma. 291 42

A right adrenocortical carcinoma (weighing 978 g) was removed from a 45-year-old man in April 1986, the tumour bed then being irradiated with 40 Gy. Subsequently discovered multiple lung metastases were treated with cisplatin, etoposide and bleomycin, without improvement. Treatment with mitotane (Lysodren) was also without effect and had to be discontinued because of severe side effects. Treatment with suramin (Germanin) was begun in August 1987. After a loading dose of 10.7 g for six weeks the lung metastases regressed almost completely. But lung metastases were again demonstrated in January 1988 during a low-dose maintenance regimen of suramin. Increased dosage arrested further growth, but achieved no regression of the metastases. The patient died unexpectedly in April 1988 of acute circulatory failure. Suramin administration had been discontinued six weeks earlier because of bronchopneumonia and general deterioration. Thrombocytopenia, coagulation disorders and moderate proteinuria were the side effects of suramin treatment.
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PMID:[Treatment of metastatic adrenal carcinoma with suramin]. 292 93

Three patients with advanced adrenocortical carcinoma were treated with a combination of intermittent streptozocin and continuous o,p'DDD. Two patients were treated preoperatively and the primary tumors, initially considered as inoperable, could be resected after 19 and 5.5 months, respectively. In the patient with the longer treatment (35 months), lung and lymph node metastases disappeared and she has no evidence of recurrent disease 6.5 years after start of therapy. One patient was followed by magnetic resonance imaging (MRI) and urinary steroid secretion. The MRI gave a good visualization of the tumor. Measurements of relaxation times showed a significant decrease in T1 values. The urinary steroid profile showed an increased secretion of 3 beta-hydroxy-5-ene steroids and tetrahydro-11-deoxy-cortisol. Treatment with streptozocin and o,p'DDD initially increased 16-oxygenation of dehydroepiandrosterone and androst-5-en-3 beta,17 beta-diol, followed by a decrease in the secretion of all urinary steroids. The third patient received postoperative treatment with no effect on metastatic disease in the lungs, she died 9 months after start of treatment. The therapeutic approach with the combination regimen of streptozocin and o,p'DDD pretreatment plus aggressive surgery has to be further evaluated, as well as MRI and urinary steroid profile as methods to monitor the effect of therapy.
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PMID:Treatment of hormone-producing adrenocortical cancer with o,p'DDD and streptozocin. 294 24

During 1980 and through 1985, a total of three adult patients with adrenal cortical carcinoma were patients at the University of Mississippi Medical Center. All three of these patients with large tumors were evaluated with CT abdominal scans. The CT findings are described in each case. One patient had the unique findings of four separate tumors at the left upper quadrant, i.e. an adrenal cortical carcinoma, an ipsilateral renal cell carcinoma, an adjacent para-aortic neurofibroma, and a gastric leiomyoma. The only known cure for adrenal cortical carcinoma is complete excision. CT scanning should be utilized in all cases for diagnosis, for characterizing the tumor, for showing the presence of metastatic disease, for determining the overall extent of disease, and for determining the relationship to other organs. CT should be utilized in the follow-up with these patients after treatment, i.e. surgery and/or chemotherapy.
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PMID:CT of adult adrenal cortical carcinoma: six years experience. 338 62

Adrenal cortical carcinoma is an uncommon neoplasm in children. Only a handful of congenital adrenal cortical carcinoma cases have been described. A newborn who had metastatic adrenal cortical carcinoma (skin metastases and cerebral lesions) is described. This patient underwent surgical resection of the right adrenal primary, but no further treatment was given. Hemihypertrophy developed in this patient by 2 months of age, and at 4 months of age spontaneous regression of all skin nodules and central nervous system (CNS) lesions was observed. Follow-up at 1 year shows the patient to be alive, well, and disease-free. Evaluation of the tumor included DNA ploidy analysis that showed the tumor to be polyploid, a pattern recently associated with nonmetastasizing adrenal cortical neoplasm. The observation of apparent metastatic disease that regressed spontaneously highlights the prognostic value of DNA ploidy analysis and raises the possibility of an adrenal tumor with properties similar to those of Stage IV-S neuroblastoma.
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PMID:Spontaneously regressing adrenocortical carcinoma in a newborn. A case report with DNA ploidy analysis. 339 Jul 90

Experience with the surgical treatment of 12 patients with carcinoma of the adrenal cortex is presented. They were 6 men and 6 women. Their ages ranged from 23 to 66 years old (mean 38 years). Four carcinomas had detected hormonal activity. The location of the carcinoma involved the right adrenal in 8 cases and the left in 4 cases. The mean diameter of the mass was 15 cm. Excision of the adrenal cancer with the ipsilateral kidney was the usual procedure. Lymph nodes were involved in 5 cases and interestingly the kidney was only involved in 3 cases. One patient died during hospitalization from pulmonary embolus, whereas another one required re-exploration for postoperative hemorrhage. Nine patients developed pulmonary, hepatic and bone metastasis within 3 to 10 months, postoperatively. Radiation treatment and chemotherapy had poor results. Nevertheless, one patient in whom left adrenalectomy, splenectomy and partial pancreatectomy was performed, is doing well, despite the presence of hepatic metastases, with the addition of o,p' DDD, 24 months postoperatively. Another patient underwent reoperation and excision of recurrent local disease 12 months after adrenalectomy/nephrectomy. She is now alive 16 months following her second surgery. Based on the above, an aggressive surgical approach is advocated in the management of adrenocortical carcinoma.
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PMID:The surgical management of adrenal cortical carcinoma. 373 53

A 9 year old Japanese girl was admitted complaining of left hypochondrial pain and a large upper left abdominal tumor. There were no clinical or laboratory signs of hormonal abnormality. Intravenous pyelography showed marked compression and deformity of the kidney by a tumor. This tumor was excised together with the left kidney. The pathological diagnosis was adrenocortical carcinoma. Postoperatively, the child was given neither irradiation nor chemotherapy. Twenty-one months after the surgery, there was a hepatic metastasis, and she died 40 months after surgery from a combination of hepatic metastases and local tumor recurrence.
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PMID:Nonfunctioning adrenocortical carcinoma in a young girl. 383 2


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