Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042755 (masculinization)
 2,562 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 15-year-old girl presented to a primary care clinic with secondary amenorrhea, masculinization, and rapidly increasing abdominal girth. Evaluation revealed a large pelvic mass, which was found at laparotomy to be a bilateral Sertoli-Leydig cell tumor producing androgens and high levels of alpha-fetoprotein (AFP). The patient underwent bilateral salpingo-oophorectomy. Other pelvic structures were uninvolved. The postoperative course was unremarkable, with testosterone and AFP levels in the normal range. A thorough literature search revealed no cases in which bilateral Sertoli-Leydig cell tumors were associated with high AFP levels. This case was unusual in its pathology, dramatic in its presentation, and valuable in its implications regarding the work-up of amenorrhea in young women.
 ...
PMID:Sertoli-Leydig cell tumor of the ovary: a rare cause of amenorrhea. 156 81

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.
 ...
PMID:Clinical syndromes associated with tumors of the female genital tract. 175 57

Virilizing adrenal adenomas are rare, and Leydig cell adenomas of the adrenal rarer still. Our patient, a 60-year-old virilized woman, was found to have a Leydig cell adenoma. Virilization associated with normal 17-ketosteroid and elevated testosterone levels necessitates excluding an adrenal cause. Although one might expect high testosterone levels to suppress gonadotropins, this is not the case. In addition to other studies recommended for assessing the incidentally discovered adrenal mass, we would add a testosterone assay.
 ...
PMID:Virilization due to a Leydig cell adrenal adenoma. 283 7

A 49-year-old woman with virilization demonstrated biochemical features traditionally ascribed to virilizing ovarian tumors: marked elevation of serum testosterone level with normal urinary excretion of 17-ketosteroids and normal serum dehydroepiandrosterone level. An adrenal cortical adenoma containing neoplastic cells indistinguishable from Leydig cells, including the demonstration of characteristic crystalloids of Reinke, was shown to be the source of the elevated testosterone level. A review of the literature revealed 13 cases with a similar biochemical profile, and of these, two were reported to contain crystalloids of Reinke. Taking cognizance of the fact that these characteristic crystalloids are only found in 40 percent of Leydig cell tumors of the testis, it is concluded that Leydig cells may be present in, and may be active participants in, the pathophysiology of a number of testosterone-secreting adrenal tumors.
 ...
PMID:Testosterone-secreting adrenal adenoma containing crystalloids characteristic of Leydig cells. 300 Jan 78

Leydig cell function in 21 boys with acute lymphoblastic leukemia who had been treated by bilateral direct testicular irradiation (12 X 2 Gy) at 8.4 +/- 0.7 years, was evaluated 3.8 +/- 0.4 years after irradiation. At the time of irradiation all were prepubertal and at evaluation 12 were prepubertal and 9 pubertal. Leydig cell insufficiency, indicated by a low plasma testosterone response to chorionic gonadotrophin and/or an increase in basal level of plasma luteinizing hormone, was observed in 19/21 patients. The children who were the youngest at testicular irradiation were more vulnerable. Spontaneous virilization occurred in 3 of the older children and resulted from compensated Leydig cell dysfunction.
 ...
PMID:Leydig cell insufficiency after testicular irradiation for acute lymphoblastic leukemia. 324 74

A report is given on the rare case of a hormonactive Leydig cell-tumor in a 52-year-old patient. In spite of virilization symptoms of many years' standing, it was only because of multiple troubles with a great uterus myomatosus that she was admitted to the hospital. An ovarian tumor existing at the same time, turned out to be histologically a mere hilus cell-tumor with a strong nucleus polymorphy. In consideration of the danger of a malignant degeneration (borderline case), the patient was included in our tumor follow-up control programme.
 ...
PMID:[Leydig cell tumor with pronounced virilization]. 401 45

Endocrine studies were made on 4 women with ovarian Sertoli-Leydig cell tumors of varying degrees of differentiation. Clinically, all 4 patients showed evidence of increased androgen production, manifested by either hirsutism or virilization. The calculated ratios of steroid hormones between the affected ovarian venous values and peripheral values for testosterone (T), androstenedione (A), and dehydroepiandrosterone (DHEA) in 3 patients, were 1.4-18.6, 4.2-24.4, and 3.7-10.0, respectively. The peripheral levels of the hormones before salpingo-oophorectomy in all the patients were T: 0.97-45.0 ng/ml; A: 2.92-114.0 ng/ml; and DHEA: 13.0-20.9 ng/ml. In the 3 juvenile patients, elevated basal levels of LH and normal or subnormal levels of FSH (high LH:FSH ratio) were found. In a 12-year-old patient with 1,900 g tumor composed predominantly of Sertoli cells with lipid storage, the peripheral serum levels of T and estradiol (E2) were 114 ng/ml and 1890 pg/ml, respectively. The present data suggest that: 1) androblastomas have a biosynthetic capacity for androgen, estrogen, or both in which both the delta 5 and delta 4 pathways are involved, and 2) little correlation exists between the endocrine function and cellular composition of the tumors or their degree of differentiation.
 ...
PMID:Endocrine studies on ovarian androblastomas (Sertoli-Leydig tumors). 609 78

Fifty ovarian stromal tumors that had a predominant pattern of fibroma or thecoma but also contained cells typical of steroid hormone-secreting cells are reported. Forty-six tumors were classified as luteinized thecomas because the steroid cells resembled lutein cells and lacked crystalloids of Reinke. Four were classified as stromal Leydig cell tumors because crystalloids were identified in the steroid cells. The luteinized thecomas occurred at an average age of 46 years and were associated with estrogenic manifestations in 50% and androgenic changes in 11% of the cases. In the remaining cases there was no clinical or pathological evidence of steroid hormone production at the time of diagnosis. Six patients, two of whom were virilized, were pregnant. Four tumors appeared malignant on histologic examination. One of these tumors was rapidly fatal, the outcome is unknown in a second case, the third patient is alive and well at 5 years, and the fourth tumor was diagnosed too recently for evaluation of its behavior. The stromal Leydig cell tumors occurred at an average age of 61 years and were associated with virilization in one case, endometrial hyperplasia in another case, and endometrial hyperplasia with carcinoma in a third case. The fourth tumor was unassociated with endocrine manifestations. Luteinized thecomas and stromal Leydig cell tumors are indistinguishable except for the presence of crystalloids of Reinke in the latter. In view of the prolonged search that is necessary to find these structures in some stromal Leydig cell tumors and their well-known absence in the majority of testicular Leydig cell tumors, it is reasonable to assume that an unknown proportion of tumors in the luteinized thecoma category are unrecognized stromal Leydig cell tumors.
 ...
PMID:Ovarian stromal tumors containing lutein or Leydig cells (luteinized thecomas and stromal Leydig cell tumors)--a clinicopathological analysis of fifty cases. 630 83

An 18-yr-old 46,XY man with primary hypogonadism and a microphallus is described whose Leydig cells appear to be partially insensitive to gonadotropin action. The external genitalia were well differentiated though abnormally small. The mean +/- SE baseline plasma testosterone (T) level was 62 +/- 3.9 ng/dl, and androstenedione was 34.5 +/- 7.3 ng/dl. Plasma levels of dehydroepiandrosterone, dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, 17-hydroxypregnenolone, corticosterone, deoxycorticosterone, and 17 beta-estradiol were all normal. After the im administration of hCG, plasma T increased insignificantly from 71 to 78 ng/dl, and androstenedione increased from 22 to 47 ng/dl; there was no significant change in the levels of precursor steroids. The mean +/- SE serum FSH level was 17.4 +/- 3.6 mIU/ml, and LH was 15.4 +/- 1.1 mIU/ml (normal, 5-20); both responded briskly to iv GnRH. Exogenous T therapy resulted in normal virilization, whereas therapy with hCG was ineffectual. Testicular biopsy revealed Leydig cells in normal numbers, some spermatogenesis, and thickened tubular basement membranes. In vitro binding studies using [125I]hCG were performed with testicular homogenates from the patient and three normal subjects. With 7.4 fmol labeled hCG, the specific binding (mean +/- SD), expressed as femtomoles of hCG per mg protein, was 1.16 +/- 0.44 compared to 2.49 +/- 0.41 in normal subjects (P less than 0.05). These data demonstrate partial resistance to hCG and suggest that the defect in Leydig cell function may be at the LH receptor or postreceptor level.
 ...
PMID:A syndrome of gonadotropin resistance possibly due to a luteinizing hormone receptor defect. 632 57

Seventeen granulosa cell, thirteen Sertoli-Leydig cell and six unclassified sex cord-stromal tumors diagnosed during pregnancy or the puerperium were reviewed. Eleven patients presented with abdominal pain or swelling, five in shock, two with virilization, and one with vaginal bleeding. Three asymptomatic patients were explored because of a palpable mass and one because of an adnexal mass found on ultrasound examination. In thirteen patients the tumor was discovered during a cesarean section; five of them had had dystocia and in eight of them the tumor was an incidental finding. All the tumors were Stage I but 13 of them had ruptured; all but one were unilateral. Hemoperitoneum was present in seven cases. On microscopical examination many of the tumors differed from tumors in the same diagnostic categories occurring in the absence of pregnancy by having a disorderly arrangement of their cells, lacking recognizable differentiation in many areas, showing prominent edema, and containing unusually large numbers of lutein or Leydig cells. The last two features were most obtrusive in tumors removed at term. With one exception the patients were initially treated by conservative surgical procedures. Two of them received chemotherapy and two radiation therapy postoperatively. A hysterectomy and salpingo-oophorectomy was performed at a second operation in eight cases; no residual tumor was found in any of the specimens. Only one patient had a recurrence, which was treated surgically. Follow-up for an average of 4.7 years is available for 30 of the 36 patients; all of them were alive and free of disease at the time of the last examination.
 ...
PMID:Granulosa cell, Sertoli-Leydig cell, and unclassified sex cord-stromal tumors associated with pregnancy: a clinicopathological analysis of thirty-six cases. 673 62


1 2 3 4 5 6 7 8 Next >>