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The Journal of Obstetrics and... Oct 2009A pregnancy luteoma represents an unusual response of ovarian stromal cells to the altered hormonal levels of pregnancy. It is a distinctive non-neoplastic lesion...
A pregnancy luteoma represents an unusual response of ovarian stromal cells to the altered hormonal levels of pregnancy. It is a distinctive non-neoplastic lesion characterized by solid proliferations of luteinized cells resulting in a tumor-like ovarian enlargement. Most patients are asymptomatic; the ovarian enlargement is usually discovered incidentally at cesarean section or during postpartum tubal ligation. We report a typical case that we found at cesarean section to be associated with a virilized infant who manifested clitoromegaly and labial fusion. We detected an increased level of testosterone in the maternal patient. We concluded that the ovarian luteoma induced the fetal virilization.
Topics: Adult; Cesarean Section; Female; Humans; Infant, Newborn; Luteoma; Maternal-Fetal Exchange; Ovarian Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Testosterone; Virilism
PubMed: 20149054
DOI: 10.1111/j.1447-0756.2009.01046.x -
Journal of Pediatric and Adolescent... Oct 2009Only 30% of luteinized thecomas are found in women under the age of 30, and they are typically benign. Only 11% of luteinized thecomas show clinical signs of androgen...
BACKGROUND
Only 30% of luteinized thecomas are found in women under the age of 30, and they are typically benign. Only 11% of luteinized thecomas show clinical signs of androgen production. We present an unusual case of a 15-year-old female who presented with secondary amenorrhea and virilization and was subsequently diagnosed with a benign luteinized thecoma of the ovary. This is the youngest nonmalignant luteinized thecoma reported to date.
CASE
A 15-year-old Sri Lankan female presented with increasing hair growth, a hoarse voice, and secondary amenorrhea. On physical examination, there was marked hirsutism and clitoromegaly. Investigations found an elevated free testosterone level and an enlarged, homogenous left ovary with absent normal ovarian architecture. A laparotomy and unilateral salpingo-oophorectomy was performed. The final diagnosis was a luteinized ovarian thecoma, with no evidence of malignancy. Postoperatively, testosterone levels normalized and menstrual cycles resumed. Although laser treatment helped with her hirsutism, her other virilizing symptoms (deepening of voice, clitoromegaly) did not improve postoperatively.
CONCLUSION
This case of a virilizing ovarian luteinized thecoma emphasizes the need for timely evaluation, diagnosis, and treatment of patients presenting with symptoms of excess androgen secretion in order to avoid irreversible unwanted effects. Possible ovarian causes of excess androgen secretion should not be overlooked when considering possible causes of hyperandrogenism and secondary amenorrhea.
Topics: Adolescent; Amenorrhea; Fallopian Tubes; Female; Hirsutism; Hoarseness; Humans; Laser Therapy; Ovarian Neoplasms; Ovariectomy; Thecoma; Tomography, X-Ray Computed; Virilism
PubMed: 19576809
DOI: 10.1016/j.jpag.2008.06.002 -
Best Practice & Research. Clinical... Jun 2006Hyperandrogenism or androgen excess is a common endocrine disorder of women of reproductive-age, with a prevalence of 5-10%. The majority of patients with... (Review)
Review
Hyperandrogenism or androgen excess is a common endocrine disorder of women of reproductive-age, with a prevalence of 5-10%. The majority of patients with hyperandrogenism will have polycystic ovary syndrome. Hyperandrogenism presents a complex diagnostic challenge for both the practicing physician and the clinical investigator. Clinical manifestations of hyperandrogenism include hirsutism, acne, androgenic alopecia, and virilization. Hirsutism, defined as excessive growth of terminal hair in women in a male-like pattern, is the most commonly used clinical diagnostic criterion of hyperandrogenism. The presence of hirsutism is usually determined by using a standardized scoring system of hair growth. Depending on the definition, hirsutism is present in up to 80% of patients with hyperandrogenism. Acne and androgenic alopecia are other common androgenic skin changes, and might be observed without hirsutism in some hyperandrogenic women. However, isolated presence of any of these manifestations is not used as a diagnostic criterion for hyperandrogenism. Virilization is a relatively uncommon feature of hyperandrogenism, and its presence often suggests an androgen-producing tumor. A thorough history and a focused clinical examination are extremely helpful in diagnostic evaluation of patients with suspected hyperandrogenism.
Topics: Acne Vulgaris; Alopecia; Female; Hirsutism; Humans; Hyperandrogenism; Virilism
PubMed: 16772149
DOI: 10.1016/j.beem.2006.02.004 -
Gynecological Endocrinology : the... Aug 2014We report the case of a 36-year-old woman with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, and corticosteroid replacement therapy since birth....
We report the case of a 36-year-old woman with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, and corticosteroid replacement therapy since birth. She manifested persistent virilization and high testosterone levels that were attributed to nonadherence to medical treatment. The patient was referred to our gender unit for genitoplastic surgery. We recommended the patient for left oophorectomy after detecting an ovarian mass. Pathologic findings confirmed an ovarian hilus cell tumor. Testosterone levels fell back to normal and masculinization disappeared but ACTH remained elevated. This case represents a very rare type of primary ovarian tumor that must be considered in persistent virilizing symptoms in women with CAH.
Topics: Adrenal Hyperplasia, Congenital; Adult; Female; Humans; Leydig Cell Tumor; Ovarian Neoplasms; Virilism
PubMed: 24702195
DOI: 10.3109/09513590.2014.907260 -
The Journal of Clinical Endocrinology... Dec 1992We describe a 44-yr-old woman with a 12-yr history of clinical virilization and serum testosterone levels up to 28.1 nmol/L (normal range, 1-3.3 nmol/L) in whom repeated...
We describe a 44-yr-old woman with a 12-yr history of clinical virilization and serum testosterone levels up to 28.1 nmol/L (normal range, 1-3.3 nmol/L) in whom repeated clinical evaluation and surgical procedures failed to reveal the source of androgen production. At the time the patient was referred to the Clinical Center of the NIH, an intrathoracic mass was seen on upper cuts of an abdominal computer-aided tomography scan, confirmed by computer-aided tomography scan and magnetic resonance imaging of the chest. A 6 x 5 x 3.5-cm mass, attached to the posterior pericardium, was removed by thoracotomy. Pathological examination revealed an adrenal cortical neoplasm of uncertain malignant potential that contained testosterone, 11-deoxycortisol, progesterone, and 17-hydroxyprogesterone. After the operation, the patient's serum testosterone levels decreased to the normal range. Ectopic adrenal cortical rests in the thorax and neoplasms arising from these rests are extremely rare, and we are not aware of a similar case previously reported. In women with virilization, radiological studies of the thorax as well as other reported sites of ectopic adrenal cortex should be performed if radiological studies of the abdomen and pelvis fail to locate the source of the neoplasm.
Topics: Adrenal Cortex Neoplasms; Adult; Choristoma; Female; Humans; Radiography; Testosterone; Thoracic Neoplasms; Virilism
PubMed: 1464658
DOI: 10.1210/jcem.75.6.1464658 -
Yonsei Medical Journal Feb 2009A 29-year-old woman presented with secondary amenorrhea, primary infertility, and virilization, which had developed over the past 2 years was suspected to have a...
A 29-year-old woman presented with secondary amenorrhea, primary infertility, and virilization, which had developed over the past 2 years was suspected to have a virilizing tumor at her left ovary. Her serum testosterone level was markedly elevated (380 ng/dL). Left salpingoophorectomy was performed, and histopathological examination revealed a thecoma of the left ovary. The postoperative serum testosterone level returned to 65 ng/dL. The patient did not have regression of virilism soon. However, the patient had a normal menstruation 29 days after surgery and gave birth to a baby 13 months after surgery.
Topics: Adult; Female; Humans; Menstruation; Ovarian Neoplasms; Ovariectomy; Pregnancy; Pregnancy Outcome; Testosterone; Thecoma; Virilism
PubMed: 19259367
DOI: 10.3349/ymj.2009.50.1.169 -
Gynecological Endocrinology : the... Oct 1998Whereas ovarian tumors with overt endocrine manifestations account for less than 5% of all ovarian neoplasms, the incidence of virilizing type tumors in postmenopausal...
Whereas ovarian tumors with overt endocrine manifestations account for less than 5% of all ovarian neoplasms, the incidence of virilizing type tumors in postmenopausal women is even lower since the average age of occurrence is 43 years. Steroid cell tumors not otherwise specified (NOS) are even more rare. We report the case of a 56-year-old woman (age of onset of menopause 43 years) who consulted our service due to a hyperandrogenic syndrome: deepening of the voice, temporal balding, hirsutism and cliteromegaly. Laboratory findings indicated hyperandrogenism in male range. The dexamethasone suppression test did not modify basal values, indicating that adrenal origin was unlikely. Transvaginal ultrasound disclosed multiple microcysts in the left ovary. Abdominal tomography was normal. Suspecting an ovarian tumor, bilateral oophorectomy was performed and a pediculate, 3 cm in diameter, was encountered in the left ovary. Histopathological studies determined it to be a virilizing ovarian tumor NOS. Postoperative recovery was fast; normal hormonal values were reached together with visible clinical improvement. This case is reported because this type of tumor is very infrequent in postmenopausal women, and because in this case it was the functional hormonal test that allowed tumor localization.
Topics: Diagnosis, Differential; Female; Humans; Hyperandrogenism; Middle Aged; Ovarian Neoplasms; Postmenopause; Virilism
PubMed: 9859028
DOI: 10.3109/09513599809012837 -
Cancer Sep 1993Virilizing adrenocortical tumors are uncommon in adult women. These lesions generally secrete dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEAS),...
BACKGROUND
Virilizing adrenocortical tumors are uncommon in adult women. These lesions generally secrete dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEAS), but not testosterone, which usually is produced by ovarian tumors. Exceptionally, adrenal growths may give off testosterone and no other assessable androgen. The detection of the site of excess testosterone yield is paramount for proper surgery. The true nature of the growth often is unpredictable, even at the time the pathologist examines the surgical specimen.
METHODS
The workup in a virilized adult woman relies on biochemical tests such as 24-hour urinary 17-KS and 17-OHCS levels and plasma corticosteroid levels (testosterone, DHEA, DHEAS, and androstenedione), and on modern imaging studies such as ultrasonography, computed tomography, and digital angiography.
RESULTS
Among a series of 190 adrenal tumors collected in the last 30-year period, only 10 virilizing growths (5.3%) were detected. Two cases of virilization mixed with cushingoid features were observed. In two other cases, reported in detail, the tumor secreted testosterone only, without other assessable androgens. Seven of the 10 tumors were malignant.
CONCLUSIONS
In cases of tumors secreting testosterone only, high-resolution imaging has contributed significantly in pinpointing the site of the growth, whereas dynamic hormone testing, using selective stimulation or suppression studies, has been misleading. The malignant nature of the growth may be revealed only by the presence of metastases, because pleomorphism and capsular and vascular invasion have been detected histologically in clinically benign tumors. The prognosis for large tumors usually is dismal.
Topics: Adrenal Gland Neoplasms; Adult; Aged; Carcinoma; Female; Humans; Middle Aged; Testosterone; Virilism
PubMed: 8364880
DOI: 10.1002/1097-0142(19930915)72:6<1997::aid-cncr2820720634>3.0.co;2-1 -
European Journal of Endocrinology Nov 2004In foetuses at risk of virilising congenital adrenal hyperplasia (CAH), prenatal treatment can be offered by administration of dexamethasone (DEX) via the mother, in... (Review)
Review
In foetuses at risk of virilising congenital adrenal hyperplasia (CAH), prenatal treatment can be offered by administration of dexamethasone (DEX) via the mother, in order to suppress foetal adrenal androgen oversecretion and prevent genital malformations. The first treated cases were described 20 years ago, and several hundred pregnancies have been reported since. There is a consensus that the treatment effectively prevents or reduces virilisation, but opinions regarding its safety differ. Rare adverse events have been reported in treated children, but no harmful effect has been documented that can be clearly attributed to the treatment. However, few treated foetuses have been followed until adolescence. Animal studies and epidemiological data point to various adverse effects of excess glucocorticoids on the developing foetus. In order to prevent virilisation effectively in females affected with CAH, the prenatal treatment needs to be instituted in the early first trimester, before prenatal diagnosis is possible. Thus, a majority of treated foetuses will receive DEX unnecessarily. The PREDEX study was initiated in Stockholm in 1999 as an open, controlled, non-randomised, multicentre trial. Participating centres are Stockholm, Bergen, Kuopio, Warsaw, London, Lyon and Barcelona. The study has been approved by the ethics committees in each country. The purpose of PREDEX is to evaluate prospectively the prenatal treatment regarding efficacy in preventing virilisation as well as to study its safety for both mothers and treated children. Children are followed until 18 years of age and a wide range of physiological, metabolic and developmental parameters are considered. In Sweden, treatment is not offered outside the frames of the trial.
Topics: Adrenal Hyperplasia, Congenital; Clinical Trials as Topic; Dexamethasone; Female; Follow-Up Studies; Humans; Maternal-Fetal Exchange; Pregnancy; Virilism
PubMed: 15554888
DOI: 10.1530/eje.0.151u063 -
MMW Fortschritte Der Medizin Jan 2011
Topics: Adult; Cooperative Behavior; Diagnosis, Differential; Female; Hirsutism; Humans; Interdisciplinary Communication; Referral and Consultation; Virilism
PubMed: 22165616
DOI: 10.1007/BF03367698