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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tumors of the female genital tract may be associated with a variety of unusual clinical manifestations. Uncommon endocrine and paraendocrine syndromes include production of human chorionic gonadotropin by tumors other than those of germ cell origin, hyperthyroidism associated with struma ovarii and gestational trophoblastic disease, the carcinoid syndrome, the Zollinger-Ellison syndrome, hypercalcemia, Cushing's syndrome, hypoglycemia, hypertension related to renin or aldosterone production, hyperprolactinemia, inappropriate secretion of antidiuretic hormone, and virilization associated with Nelson's syndrome and placental site trophoblastic tumor. Paraneoplastic syndromes associated with gynecological tumors include disorders of the nervous system, connective tissue, and skin, as well as hematologic abnormalities and the nephrotic syndrome. Heritable and other congenital syndromes associated with these tumors are the Peutz-Jeghers syndrome, the nevoid basal-cell carcinoma syndrome, Ollier's disease and Maffucci's syndrome, hereditary leiomyomatosis, ataxia-telangiectasia, von Hippel-Lindau's disease, thyroid abnormalities associated with Sertoli-Leydig cell tumors, and Carney's complex. Other syndromes associated with tumors of the female genital tract include Meigs' syndrome, hyperamylasemia, uveal melanocytic lesions, and pyrexia.
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PMID:Clinical syndromes associated with tumors of the female genital tract. 175 57

Sexual precocity has important psychosocial implications for the prematurely developing child, as well as being associated in some cases with significant pathology. Conscientious evaluation and initiation of effective therapy can have a significant impact on improving long-term outcome. The differentiation between complete sexual precocity with activation of the hypothalamic-pituitary axis and incomplete sexual precocity without activation of the central reproductive system is of paramount importance. In incomplete sexual precocity, the sex steroids are of exogenous, adrenal, or gonadal origin. Premature adrenarche presents with the early development of pubic hair only and must be distinguished from adrenal hyperplasia or an androgen-secreting neoplasm, which may be associated with accelerated growth, advanced bone age, and virilization. When incomplete sexual precocity involves the ovary, ovarian tumors must be considered. Other causes of incomplete sexual precocity include hypothyroidism and gonadotropin-independent precocity such as McCune-Albright syndrome. Complete sexual precocity or precocious puberty of central origin is diagnosed in girls by gonadotropin-releasing hormone challenge yielding a stimulated luteinizing hormone peak greater than 15 IU/L. Radiologic evaluation of the central axis is necessary. Treatment of precocious puberty relies on the use of potent agonists of gonadotropin-releasing hormone that reversibly suppress the prematurely activated pituitary. Depot preparations are efficacious. Early initiation and careful monitoring of treatment can reduce physical signs of development, improve the likelihood for normal adult height, and postpone normal pubertal progression to a more appropriate age.
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PMID:Recent advances in the management of sexual precocity in girls. 181 19

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

Adrenocortical causes of hypertension are established by examining the mineralocorticoid hormones produced in the zona glomerulosa and zona fasciculata. In the zona glomerulosa, aldosterone excess leads to hypertension, hypokalemia, and suppressed plasma renin activity, with increased concentrations of urinary aldosterone (either as the 18-glucuronide or free aldosterone) as an index of its production. Identifying a tumor by computed tomography scan verifies the diagnosis of a correctable lesion. If no tumor is found, several maneuvers are used to identify primary adrenal hyperplasia, a disorder with autonomous aldosterone production, for which reduction of adrenal mass is curative. The zona fasciculata has two major pathways: the 17-deoxy pathway, where deoxycorticosterone (DOC) and corticosterone are the significant steroids, and the 17-hydroxy pathway, which leads to cortisol production. Tumors of the 17-deoxy pathway, DOC-producing adenomas, have increased concentrations of DOC and its precursor steroids, normal concentrations of cortisol, and suppression of aldosterone production secondary to suppression of the renin system. Two enzymatic defects in the zona fasciculata, 11 beta- and 17 alpha-hydroxylase deficiency, can be first readily identified by the virilization in the former, hypogonadal features in the latter. Steroid patterns are diagnostic. DOC is produced in excess in both deficiencies and is the cause of the hypertension. Deficient or impaired 11 beta-hydroxy steroid dehydrogenase in the apparent mineralocorticoid excess syndrome or after licorice ingestion retards the conversion of cortisol to inactive cortisone in the kidney, leading to mineralocorticoid hypertension; this leads to suppression of the renin system and subsequently of aldosterone.
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PMID:Steroid characteristics of mineralocorticoid adrenocortical hypertension. 191

We studied three cases of adrenocortical neoplasms that were detected incidentally after radiological examination of the abdomen. These cases did not demonstrate any clinical evidence of adrenocortical abnormalities, such as virilization. Macroscopically, the tumors were light to dark tan on cut surface. Light-microscopic examination revealed compact cells with abundant lipid-sparse eosinophilic cytoplasm and occasional enlarged nuclei. In one case, ultrastructural observation demonstrated abundant mitochondria. Immunohistochemical examination of all of the adrenocortical steroidogenic enzymes showed that none of the cases had immunoreactivity. No mitotic activity and no vascular invasion was observed. The postoperative course were uneventful. The follow-up interval varied from 8 to 27 months. These three neoplasms apparently represent the first reported cases of adrenocortical oncocytoma. They can be considered true nonfunctioning adrenocortical neoplasms because steroidogenic enzymes required for corticosteroid biosynthesis were not expressed in the tumor cells.
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PMID:Adrenocortical oncocytoma. A true nonfunctioning adrenocortical tumor. 192 51

A study of 28 adolescents aged 10-20 years admitted with ovarian tumors during a 5-year period revealed that 75% had malignant ovarian tumors, with germ cell tumors being most frequent. The high incidence of malignancy was due to our hospital being a referral centre. One case of mixed germ cell tumor (mainly choriocarcinoma) presented with precocious puberty, and one case of arrhenoblastoma showed signs of virilization. Tumor markers were found useful in diagnosing choriocarcinoma and endodermal sinus tumor. Amongst the malignant ovarian tumors, the best prognosis was noted with dysgerminomas. Tumors were found more frequently on the right side. Optimal surgery at initial laparotomy, followed by adjuvant chemotherapy or radiotherapy were found to offer the best change of cure.
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PMID:Ovarian tumors in the second decade of life. 195 33

An 8-year-old girl exhibited severe, progressive virilization of 2 years' duration associated with markedly elevated circulating testosterone concentrations. Based on her initial clinical presentation and results of a chemical evaluation, she was originally thought to have non-classic 21-hydroxylase deficiency, but her condition did not respond to corticosteroid therapy. Further evaluation confirmed the presence of an ovarian neoplasm. The excised ovary contained an attached gray-brown mass. Light microscopic and ultrastructural examination revealed the mass to be a steroid cell tumor. Because Reinke's crystals were not present, it was designated to be a steroid cell tumor not otherwise specified. This case represents one of 22 reported cases of steroid cell tumor occurring in children described in the literature, most of which have been associated with heterosexual precocity. To our knowledge, steroid cell tumors are benign when they occur in prepubertal children. Although they are rare, steroid cell tumors of the ovary should be considered in cases of childhood virilization.
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PMID:Steroid cell tumor of the ovary in a child. 199 81

The stromal Leydig cell tumour of the ovary is a very rare benign neoplasm which usually occurs in postmenopausal women. Due to a significant production of androgens by the tumour, it is frequently associated with symptoms of virilization. To our knowledge only 7 cases have been reported in the literature, one of them with bilateral manifestation. We report an additional case affecting both ovaries in a 59-year-old nun with long-standing virilism, review the literature, and discuss the histomorphological features for differential diagnosis.
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PMID:Bilateral stromal Leydig cell tumour of the ovary. Case report and literature review. 206 19

Testosterone-secreting adrenal adenoma is rare. We recently experienced a 17-year-old pubertal girl who showed signs of virilization and a high serum level of testosterone. The excised adrenal gland showed a 3.5 x 3 x 3-cm cortical adenoma. Light and electron microscopic findings together with the high serum level and high tumor tissue contents of testosterone and dehydroepiandrosterone (DHA) indicated that the tumor was a testosterone-secreting adrenal cortical adenoma. This appears to be a rather rare tumor from a review of the literature. Interestingly, in this case, the cytoplasm of the tumor cells contained structures resembling spironolactone bodies. From the results of enzyme histochemistry, the steroidogenetic pathways in this tumor were speculated.
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PMID:A case of testosterone-secreting adrenal cortical adenoma with spironolactone body-like inclusion. 215 3

This clinical case describes a 13 year-old pubertal girl suffering from secondary virilization, amenorrhea, and abdominal mass. Plasma testosterone and androstenedione levels were markedly elevated. Upon surgery, an ovarian tumor containing 5 l of liquid was removed. The histologic examination revealed a macrocystic granulosa cell type tumor of non-juvenile type. After removal of the tumor, which included ipsilateral ovariectomy, the contralateral ovary developed polycystic changes. The role of the tumoral hyperandrogeny in inducing this contralateral polycystic ovary is discussed.
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PMID:[Virilizing ovarian tumor in an adolescent]. 215 55


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