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.
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PMID:Sertoli-Leydig cell tumor of the ovary: a rare cause of amenorrhea. 156 81

A case of an ovarian, poorly differentiated, Sertoli-Leydig cell tumor in a 55-year-old woman is reported. The patient showed no virilization, but did show elevated serum alpha-fetoprotein (AFP) levels. The tumor, measuring 70 x 45 x 50 mm, consisted mainly of a diffuse proliferation of spindle cells. Among this proliferation, some tubules of Sertoli cells and groups of leydig cells were found. Immunoperoxidase studies, using anti-AFP, localized the AFP in the leydig cells.
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PMID:[An alpha-fetoprotein producing Sertoli-Leydig cell tumor--a case report]. 246 36

An 18-year-old girl with virilization and an elevated level of serum alpha-fetoprotein (AFP) was found to have a Sertoli-Leydig cell tumor (SLCT) of the ovary. The tumor had a heterologous element of gastrointestinal type epithelium, retiform pattern, and multilocular cysts, many of which were lined by Sertoli-like cells. Alpha-fetoprotein was detected immunohistochemically in cells with the histologic appearance of Sertoli cells, and testosterone was detected in the Leydig cells. Results of lectin affinity chromatography indicated that AFP produced by this tumor was different from the AFP to be found in the liver.
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PMID:Ovarian Sertoli-Leydig cell tumor with elevated serum alpha-fetoprotein. 246 33

A 21-year-old white woman presented with virilization, hirsutism, and acne of 1.5 years' duration. Endocrine testing demonstrated complete suppression of serum testosterone, from 5.3 to 0.6 ng/mL, and serum androstenedione, from 4.7 to 1.7 ng/mL, after oral administration of 50 micrograms of mestranol and 1 mg of norethindrone for 21 days. No suppression of either steroid was produced by dexamethasone, whereas serum dehydroepiandrosterone sulfate was suppressed from 5.2 to 1.9 micrograms/mL. A left salpingo-oophorectomy was performed for a 3 x 4-cm Sertoli-Leydig cell tumor of intermediate differentiation. Intraoperative studies demonstrated that the tumor secreted testosterone, androstenedione, 17 alpha-hydroxyprogesterone, and estradiol, but not dehydroepiandrosterone sulfate. These findings support the thesis that hormonal manipulation tests cannot differentiate between adrenal and ovarian virilizing tumors. Nor does the oral contraceptive suppression of testosterone secretion exclude an ovarian malignancy. The patient remains free of recurrence after 7 years.
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PMID:Diagnosis and resection of an oral contraceptive-suppressible Sertoli-Leydig cell tumor with preservation of fertility and a 7-year follow-up. 252 30

A case of androblastoma with not only signs of virilization but also manifestations of precocious puberty occurring in a five-year-old girl is presented. The postoperative diagnosis was one of undifferentiated Sertoli-Leydig cell tumor of the right ovary in stage I c (International Federation of Obstetrics and Gynecology). The histopathological findings are precisely described with special reference to occurrence in young females and unique hormonal manifestations.
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PMID:Androblastoma in a five-year-old girl. 254 49

A 24-year-old woman was admitted to hospital because of hirsutism, virilism and amenorrhea, which had appeared 6 months earlier. Endocrinological evaluation showed a slightly elevated serum level of testosterone (1.2 +/- 0.05 ng/ml), normal plasma levels of dehydroepiandrosteronesulfate (DHEA-S) (2,070 +/- 6 ng/ml), androstenedione (1.8 +/- 0.5 ng/ml) and sex hormone-binding globulin (SHBG)(42 +/- 3 nM/L); there was normal urinary 17-ketosteroid (17-KS) excretion (11.7 mg/24 h), low urinary estrogen (E) excretion (3 +/- 0.4 micrograms/24 h), suppressed basal gonadotropin concentrations (LH 0.9 microUI/ml; FSH 3.2 microUI/ml) and an exaggerated response to the LH-RH test. At laparotomy, a monolateral ovarian tumor was found, which was proved histologically to be a Sertoli-Leydig cell tumor. After tumor ablation, a regular menstrual cycle followed and progressive reduction of virilism was noted. This was followed within 4 months by complete normalization of LH, FSH, estrogen and progesterone serum levels. The responsiveness to LH-RH also became normalized. Two years after this operation, the patient had a normal pregnancy. This case of virilization in a woman affected by a benign Sertoli-Leydig cell tumor was primarily characterized by an unusual response of the hypothalamopituitary axis against an endocrinological background of notable alteration of the androgen/estrogen ratio, where the androgens were slightly increased and the estrogens greatly reduced.
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PMID:An unusual hormone pattern in a virilized woman affected by Sertoli-Leydig cell tumor. Report of a case. 269 56

A woman with severe hyperandrogenemia and virilization was found to have a fall in serum testosterone (T) concentration while taking an oral contraceptive containing norethindrone (500 vs. 164 ng/dL). Subsequent surgical exploration revealed an ovarian Sertoli-Leydig cell tumor. In vitro, the tumor secreted T (mean, 1.88 ng/mg X 4 h). hCG did not stimulate T secretion. In addition, norethindrone inhibited T secretion (0.33 ng/mg X 4 h). We conclude that norethindrone directly suppressed T production by the Sertoli-Leydig cell tumor.
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PMID:Norethindrone inhibition of testosterone secretion by an ovarian Sertoli-Leydig cell tumor. 358 96

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

A case of bilateral ovarian Sertoli-Leydig cell tumor with heterologous elements is reported in an 18-yr-old girl with marked virilization. Panhysterectomy was performed, yet the tumor recurred shortly after the intervention. The microscopic picture was one of intermediate to poor differentiation. Despite chemotherapy, the postoperative course was rapidly malignant, and the patient died 4 wk later.
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PMID:Bilateral Sertoli-Leydig cell tumor with heterologous elements: report of an unusual case and review of the literature. 373 87

A 16-year-old girl with virilization and elevated serum alpha-fetoprotein (AFP) levels was found to have a Sertoli-Leydig cell tumor of the ovary. Immunoperoxidase studies using anti-AFP localized AFP in cells with the histologic appearance of Leydig cells. Serum AFP fell to undetectable levels after excision of the tumor. The patient is alive and well 1 year postexcision.
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PMID:Sertoli-Leydig cell tumor of the ovary producing alpha-fetoprotein. 620 58


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