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)

Thirty-four cases of corticosurrenaloma with clinical onset before 15 years of age have been studied. Higher frequency in girls (65 percent of the cases) and in young age (80 percent before 5 years of age, 43 percent before 2 years), association with personal or familial other tumors and malformations, are noticeable features. Virilism is the major manifestation in children (83 percent of the cases), either isolated or associated with hypercortisolism and/or feminization. Hormonal assays are of little value, and the dynamic adrenal tests are of some help only in small tumors or isolated hypercortisolism. Radiological diagnosis by urography with cavography, arteriography, is easy in most cases. Evaluation of prognosis is very difficult. Clinical and biological data are not significant, histological data are often of little help. Only a very large mass, and occurrence of metastases (liver, lungs) evidence malignancy. This series does not allow to assess definitely the best therapeutic regimen. Surgery has to be performed as soon as possible. The effects of radiations have not yet been ascertained. High doses of op'DDD have led to a sustained remission of the tumor or metastases in 2 patients, and perhaps to a longer survival in 4 others.
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PMID:[Corticosuprarenaloma in children]. 12 62

Extensive hormonal evaluation was performed in a girl with adrenal carcinoma during the primary tumor stage, following adrenalectomy, during the period when metastases were evident and while on treatment with o,p'-DDD. At the age of 14 months a diagnosis of congenital adrenal hyperplasia was made and treatment with dexamethasone (0.125 to 0.25 mg/day) resulted in a fall-off in growth rate, normal advancement in bone age, decrease in virilization and suppression of 17- ketosteroid excretion which continued until 4 3/12 years of age when virilization increased. At five years of age elevated serum and urinary androgen levels unsuppressible with dexamethasone were noted. Following removal of a large right adrenal carcinoma, serum and urinary hormone levels returned to normal. There months following surgery, liver metastases were documented associated with elevated levels of serum androgens. With o,p'-DDD treatment, serum dehydroepiandrosterone sulfate (DS) and urinary 17-ketosteroid (17-KS) excretion fell rapidly while there was a delay in the fall of free androgens. The persistence of free steroid secretion with decreased formation of DS suggests that the o,p'-DDD may have altered sulfatase activity before causing tumor necrosis and total decrease in steroidogenesis.
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PMID:Virilizing adrenal tumor in a child suppressed with dexamethasone for three years. Effect of o,p'-DDD on serum and urinary androgens. 13 87

We have described an unusual case of polyostotic fibrous dysplasia in a 25-year-old white woman who had malignant transformation into a mesenchymal tumor with widespread metastases and features of rhabdomyosarcoma. Masculinization also occurred with high plasma testosterone levels. A mechanism for the development of masculinizing features is discussed.
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PMID:Malignant transformation of polyostotic fibrous dysplasia. 42 32

Adrenal cortical carcinoma is a rare and highly malignant tumor. This retrospective study includes 99 patients (57 males and 42 females) reported to The Cancer Registry of Norway during the 15 year period from 1970 to 1984. The age adjusted incidence was 1.5 per million per year. Eighty-one patients were diagnosed with adrenal cortical carcinoma when still alive. In 18 cases the diagnosis was first made at autopsy. Median age at diagnosis was 54 years (range 2-88 years) with a slight male predominance. Information about initial symptoms was available in 67 patients, of whom 26 patients (18 females and 8 males) presented with clinically functioning tumors, the Cushing syndrome and virilization being most frequently encountered. Only 28 (35%) of 81 patients diagnosed when still alive had tumors confined to the adrenals. Half of the patients had distant metastases, with lungs and liver being the most frequent sites. Sixty-one patients underwent surgery; 51 patients had a radical or debulking operation and 10 patients had laparotomy with tumor biopsy. After a complete follow-up for at least 6 years (range 6-21 years), only 9 patients were still alive. Early stage (Stage I and II) and curative resection had a significant impact on the outcome of this disease. To improve prognosis, early diagnosis and radical surgery, if feasible, are needed.
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PMID:Adrenal cortical carcinoma in Norway, 1970-1984. 141 34

The case of a 57-year-old woman with a history of breast cancer is reported. She presented 10 years later with virilization. Stroma cell hyperplasia was present in the metastatic ovaries. The authors describe hormonal data and discuss the pathogenesis of the stromal activity. Mammary ovarian metastases associated with stroma cell hyperplasia, in the absence of pregnancy, that cause virilization are rare.
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PMID:Mammary ovarian metastases with stroma cell hyperplasia and postmenopausal virilization. 240 Sep 72

The ultrasound appearances of three children presenting with virilization are described. All cases were also examined by computed tomography. The value of ultrasound in the initial detection of virilizing neoplasm and in follow-up studies for localization of tumor recurrence following initial surgery are outlined. The advantages of ultrasound include confirmation of extrarenal and extrahepatic location in longitudinal sections, and clear demonstration of invasion of hepatic veins, the inferior vena cava (IVC), and the right atrium on real-time scanning. Computed tomography is also useful, particularly in the localization of small lesions and the detection of pulmonary metastases.
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PMID:Adrenocortical neoplasm in children. Ultrasound appearance. 303 58

A cortical adrenal carcinoma arising during the neonatal period and associated with virilization recurred after an initial excision and is in complete remission after a follow-up period of 10 years. The authors have performed a review of the medical literature which has yielded 56 neonatal carcinomas including this one. More than half of these cases concerned the thyroid gland (15 cases) and the adrenal cortex (14 cases). These were followed by malignant melanomas (12 cases), carcinomas of the parotid gland (6 cases), oropharyngeal region (3 cases), adrenal medulla (2 cases) and stomach, liver, breast and cutaneous sweat gland (one case each). Six tumors were associated with congenital malformations which included Beckwith-Wiedeman's syndrome; 4 tumors arose from preexisting tissue lesions; in one case, oral contraceptives were taken during pregnancy. The main treatment of neonatal carcinomas is surgery. Local recurrences (7 cases) do not markedly affect the outcome, whereas metastases (12 cases) have a very unfavorable prognosis. Superficial tumors are diagnosed earlier and more frequently cured. Out of 44 cases for which the outcome is known, only 18 children survived.
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PMID:[Neonatal carcinoma. Review of the literature apropos of a case]. 328 44

The clinicopathologic findings in nine patients with ovarian Sertoli-Leydig cell tumor with retiform pattern are described. The patients ranged in age from 11 months to 23 years; and seven patients were 12 years of age or younger. The most frequent presenting sign was the finding of an abdominal mass. This was associated with pain in five patients. In three patients the pain was severe due to torsion, causing an acute abdominal emergency. Slight virilization was observed in one patient only. Two patients had elevated serum alphafetoprotein (AFP), which correlated well with disease activity. The remaining patients had normal serum AFP. All the tumors were unilateral. At laparotomy the tumor was intact in six patients and ruptured in three. The tumors ranged from 8 to 22 cm, were round or oval, and cystic or solid and cystic. Eight tumors were in FIGO Stage I, and one was associated with abdominal metastases and was Stage III. Histologically, the retiform component varied from moderate to predominant in eight of the nine cases. In two tumors a heterologous component composed of striated muscle was also present. Three patients developed metastases. Two of the patients died 11 months and 2 years after diagnosis and the third patient was lost to follow-up with evidence of disease 2 years after diagnosis. The remaining six patients were well and disease-free for periods of 8 months to 6 years. The majority of these tumors were misinterpreted as serous papillary cystadenocarcinoma or endodermal sinus tumor, which are more malignant neoplasms requiring different therapy. This further underlines the importance of recognizing this histopathologic entity.
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PMID:Ovarian Sertoli-Leydig cell tumor (androblastoma) with retiform pattern. A clinicopathologic study. 367 27

Since 1980 we have been carrying out a prospective randomized trial comparing tamoxifen with the combination of tamoxifen plus nandrolone decanoate in advanced breast cancer. The tamoxifen dose is 30 mg daily and the nandrolone decanoate dose 100 mg i.m. once a week for four weeks and thereafter every other week. 98 post-menopausal patients have been evaluated for the response. The number of patients is 49 in both groups. The overall response rates (CR + PR) to tamoxifen and tamoxifen plus nandrolone decanoate were not significantly different; in the tamoxifen group the response rate was 49% and in the combination group 45%. The mean time to progression in tamoxifen group is over 13 months and in tamoxifen plus nandrolone decanoate group over 12 months. Our results do not suggest a synergistic effect from combining tamoxifen and nandrolone decanoate treatments. The response rates to tamoxifen at different sites of metastases were as follows: bones 47%, soft tissues 56%, and viscera 48%. The respective figures with the combination therapy were 36%, 64%, and 40%. Both treatments were well tolerated and in no patient was withdrawal of the therapy necessary. Mild virilization and hoarseness were experienced by all patients treated with nandrolone decanoate. Side-effects associated with tamoxifen were rare, although five patients experienced nausea and two had hot flushes.
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PMID:Nandrolone decanoate added to tamoxifen in the treatment of advanced breast cancer. 397 49

The synthetic non-steroidal antioestrogen nafoxidine (U-11, 100A) was given by mouth to 52 women with locally advanced or metastatic breast cancer, in 85% of whom the disease had become resistant to, or relapsed after, previous endocrine treatment. The objective response rate (complete or partial regression of disease) among 48 cases treated for at least four weeks was 37%. Tumours in soft tissue seemed to respond better than skeletal metastases. The patients in all but one of the 52 cases were postmenopausal. Those who had had an objective response to previous hormone treatment had a greater chance of deriving benefit from nafoxidine than those who had been resistant to hormone treatment.Side effects of nafoxidine were dryness of skin, increased loss of scalp hair, and heightened sensitivity to sunlight. None were serious, and they could be lessened by protection from solar radiation or a decrease in dosage. No obvious depression of thyroid or adrenal function or obvious water retention or masculinization was seen. Cataract was a possible complication.This clinical trial was preceded by laboratory studies in which a transplantable oestrogen-dependent tumour in the Syrian hamster was notably inhibited by the administration of nafoxidine. This experimental model may prove useful in screening potentially useful antioestrogenic agents against breast cancer before a human trial.
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PMID:Antioestrogens in treatment of breast cancer: value of nafoxidine in 52 advanced cases. 436 55


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