Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

A case of tubular Krukenberg tumor in pregnancy with virilization is presented. The pathology is reviewed. This rare tumor must be distinguished from a Sertoli-Leydig tumor. The index case adds to the previously recorded eight cases. All nine cases reviewed presented with progressive virilization between the third and eighth month of gestation, which regressed after surgery. The fetal outcomes of seven cases have been recorded. The fetuses were all female and of these five were virilized. A gastric primary was found in five cases. A primary breast carcinoma was postulated in another. In the remaining cases either no autopsy was performed or no primary tumor was found.
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PMID:Tubular Krukenberg tumor in pregnancy with virilization. 185 Nov 27

A review of a series of 70 Krukenberg tumors seen in consultation disclosed 13 cases with a predominant tubular pattern. Eleven of them had been diagnosed by the referring pathologist as a tumor in the sex cord-stromal category, usually a Sertoli-Leydig cell tumor; no diagnosis was preferred in the other two cases. Three factors contributed to the erroneous diagnoses: a prominent tubular pattern, luteinization of the stroma of the tumor in five cases, and associated virilization in two cases. Each tumor, however, contained typical signet-ring cells that were readily demonstrable with mucicarmine stains. In six cases the tumors were unilateral and in seven, bilateral. Ten patients died of their cancer from 2 to 21 months after the diagnosis had been made. In one case the ovarian tumors were not discovered until autopsy. Two patients are alive 7 and 9 months postoperatively. A primary tumor was found in the stomach in four cases and in the sigmoid colon and appendix in one each. No primary tumor was found in seven cases but an autopsy had been performed in only one of these. The diagnosis of Krukenberg tumor must always be considered in the differential diagnosis of an ovarian tumor with a tubular pattern even though endocrine manifestations are present.
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PMID:Tubular Krukenberg tumor. A problem in histopathologic diagnosis. 626 18

A virilizing adrenocortical carcinoma was treated by excision of the primary tumor and later lobectomy for metastases to the lung. Virilization was abolished, fertility restored, and the patient is without evidence of recurrent tumor 25 years later.
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PMID:Pulmonary lobectomy for metastatic adrenocortical carcinoma: a 25-year survival. 648 3

A 43-year-old female patient is currently being treated by us for a recurrence of adrenal cortical carcinoma associated with Cushing's syndrome and virilization. Despite many relapses of metastatic spread she has not only survived for 25 yr after the removal of the primary tumor but also until now preserved her ability to work in a textile factory.
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PMID:Recurrent adrenal cortical carcinoma with Cushing's syndrome: twenty-five-year follow-up of a patient. 720 88

Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development). Abdominal sonography showed a large adrenal tumor. Echocardiography revealed myocardial hypertrophy with severe subaortic obstruction. Blood tests showed excessive androgen and cortisol serum levels with absent circadian rhythm as well as suppressed corticotropin. Urine catecholamine levels were within the normal range. Tumor resection with general anesthesia was performed after preparation with antihypertensive and anticongestive drug therapy. Continuous intravenous hydrocortisone substitution was started intraoperatively and subsequently tapered and switched to oral administration after 12 days. A gradual reduction in glucocorticoid substitution and its discontinuation after a total duration of 9 months were well tolerated. Histopathologic workup revealed an adrenocortical tumor of intermediate dignity. Postoperative tumor staging excluded both residual primary tumor and metastases. Both a normalization of body weight and myocardial mass were observed. The present article is, to our knowledge, the first to describe severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor and provides novel detailed data on postoperative glucocorticoid management.
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PMID:Hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor. 2383 80