Ovarian hyperstimulation syndrome and pregnancy luteoma mimicking malignant ascites: a rare case report.

BACKGROUND: During pregnancy, both ovarian hyperstimulation syndrome (OHSS) and pregnancy luteoma could manifest as massive ascites, enlarged ovaries, or elevated serum levels of cancer antigen 125 (CA125), and atypical cells may be found in the ascitic fluid of OHSS patients. Whether this should be treated aggressively as peritoneal carcinomatosis is controversial. CASE PRESENTATION: A 35-year-old G2P1A1 woman with secondary infertility had a successful pregnancy after one cycle of assisted reproductive technology. The patient complained of lower abdominal distension, oliguria, and poor appetite 19 days after embryo transplantation. She was diagnosed with late-onset OHSS. Although the size of the ovaries decreased bilaterally to the normal range at 12 weeks of gestation after prompt medical care, the ascites increased again after an initial decreasing trend. Elevated serum levels of CA125 (191.1 IU/mL), and suspected adenocarcinoma cells were observed in the ascitic fluid. Although further magnetic resonance imaging examination or diagnostic laparoscopy was recommended, the patient was provided with supportive treatment and closely monitored upon her request. Surprisingly, her ascites diminished, and serum level of CA125 started to decline at 19 weeks of gestation. During cesarean section, pathological examination of the solid mass in the right ovary revealed pregnancy luteoma, which was presumably the other cause of the intractable ascites. CONCLUSIONS: Caution should be exercised in cases of suspicious malignant ascites during pregnancy. This may due to OHSS or pregnancy luteoma, in which abnormalities usually regress spontaneously.

A case of excision of ovarian torsion necrosis due to luteoma in a female who conceived a twin pregnancy through in vitro fertilization misdiagnosed with acute appendicitis.

The aim of this study was to describe a case of ovarian torsion necrosis due to delayed treatment of luteoma in a woman with IVF twin pregnancy who was misdiagnosed with acute appendicitis. We summarized the clinical manifestations and diagnosis of luteoma in a woman with IVF twin pregnancy and reviewed the relevant literature. We report the case of a 34-year-old Asian female who became pregnant through in vitro fertilization (IVF) and had acute right underbelly pain at 26 weeks and 6 days of gestation, which was misdiagnosed as acute appendicitis. Written consent was obtained from the patient. After fasting for a short duration and receiving anti-inflammatory and intravenous fluid replacement treatment after admission, the woman experienced increased right lower abdominal pain without relief and decreased fetal movement. B-ultrasound and CT examination showed that luteoma torsion was possible, and we performed emergency surgical laparotomy. During the operation, it was found that the right ovary was enlarged, the fallopian tube was twisted 720 degrees, and there was no lesion in the appendix. The right necrotic ovary was removed, and pathological results showed a hemorrhagic corpus luteum in the right ovary. The patient's abdominal pain was relieved after the operation and the pregnancy was continued. This is a typical case of ovariectomy associated with a luteoma of pregnancy. Whether in vitro fertilization and related procedures increase the risk of luteoma and whether they increase the risk of ovarian torsion necrosis in the case of luteoma formation are questions that need further study.

Female pseudohermaphroditism associated with maternal steroid cell tumor, not otherwise specified of the ovary: a case report and literature review.

Maternal virilization in pregnancy with or without fetal female pseudohermaphroditism has several etiologies. Of these, pregnancy luteoma is the most common cause of maternal virilization during pregnancy, and approximately 20 cases have been reported in recent years. Moreover, four cases of pregnancy luteomas with female pseudohermaphroditism have been reported. However, the extremely rare steroid cell tumor, not otherwise specified (NOS), has been reported only once as a cause for maternal virilization. Herein, the authors report the first case of maternal virilization with female pseudohermaphroditism associated with steroid cell tumor-NOS along with the clinical course, pathological features, and a review of the literature.

Ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal hirsute woman / Deficiência de 21-hidroxilase ovariana intratumoral na virilização da mulher pós-menopausa

Virilising ovarian tumours are a rare cause of hyperandrogenism in women, accounting for less than 5% of all ovarian neoplasms. It occurs most often in - and postmenopausal women. We report a case of a 64 year-old woman with signs of virilisation that had started 3 years before. Blood hormone analysis revealed increased levels of testosterone, and 17-hydroxyprogesterone. The tetracosactin test revealed 21-hydroxylase deficiency. Radiological imaging demonstrated a nodule in her left ovary. The patient was submitted to bilateral laparoscopic oophorectomy, and histopathological examination revealed a luteoma of the left ovary. Postoperative serum testosterone level and 17-hydroxyprogesterone returned to normal levels in one month. Virilism regressed within six months. Our patient also showed an elevation in 17-OHP serum levels. Normalization of 17-OHP after oophorectomy suggests a case of intratumoral 21-hydroxylase deficiency. To our knowledge, this is the first description of ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal woman. Arq Bras Endocrinol Metab. 2012;56(9):672-6.

Tumores ovarianos virilizantes são uma causa rara de hiperandrogenismo em mulheres, contabilizando menos de 5% de todos as neoplasias ovarianas. Esses tumores ocorrem mais comumente em mulheres em peri ou pós-menopausa. Relatamos aqui o caso de uma mulher de 64 anos de idade com sintomas de virilização que começaram 3 anos antes. O perfil hormonal revelou níveis aumentados de testosterona e de 17-hidroxiprogesterona (17-OHP). O teste de tetracosactin demonstrou deficiência de 21-hidroxilase. Exames radiológicos mostraram um nódulo no ovário esquerdo. A paciente foi submetida à ooforectomia laparoscópica bilateral e o exame histopatológico revelou um luteoma no ovário esquerdo. A concentração sérica de testosterona e de 17-hidroxiprogesterona após a cirurgia retornou aos níveis normais em um mês. A virilização regrediu em 6 meses. Nossa paciente também revelou uma elevação dos níveis séricos de 17-OHP. A normalização da 17-OHP após a ooforectomia sugere um caso de deficiência de 21-hidroxilase intratumoral. Esta é a primeira descrição de deficiência de 21-hidroxilase intratumoral em uma mulher na pós-menopausa. Arq Bras Endocrinol Metab. 2012;56(9):672-6.

Luteoma estromal ovárico como causa de virilización en la mujer posmenopáusica / Ovarian stromal luteoma as a cause of postmenopausal virilization

Objetivo. Se presenta el caso de una paciente diagnosticada luteoma estromal de ovario con efecto virilizante. Material y métodos. Mujer de 67 años que presenta cuadro de virilización. A pesar de que no fue posible detectar mediante pruebas de imagen ningún tumor productor de andrógenos, se decidió someter a la paciente a una histerectomía y doble anexectomía debido a la fuerte sospecha clínica y analítica de un tumor productor de andrógenos de origen ovárico. Resultados. El estudio anatomopatológico de la pieza quirúrgica demostró la presencia de un luteoma estromal de ovario como causa del cuadro de virilización que presentaba la paciente. Conclusiones. El luteoma estromal de ovario es una rara neoplasia ovárica que se presenta habitualmente en mujeres posmenopáusicas y puede comenzar con síntomas virilizantes o derivados de un ambiente hiperestrogénico. En un 20% de los casos, se diagnostica como un hallazgo incidental (AU)

Objective. We report the case of a patient diagnosed with a stromal luteoma of the ovary with a virilizing effect. Material and methods. A 67-year-old woman presented with symptoms of virilization. Although no androgen-producing tumor was detected on imaging tests, the patient underwent hysterectomy and double oophorectomy due to strong clinical and laboratory suspicion of an androgen-producing tumor of the ovary. Results. Pathologic study of the surgical specimen showed the presence of an ovarian stromal luteoma causing the patient's virilization. Conclusions. Ovarian stromal luteoma is a rare ovarian neoplasm that usually occurs in postmenopausal women and may present as virilization or hyperestrogenism. In 20% of cases, the diagnosis is incidental (AU)

Ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal hirsute woman.

Virilising ovarian tumours are a rare cause of hyperandrogenism in women, accounting for less than 5% of all ovarian neoplasms. It occurs most often in - and postmenopausal women. We report a case of a 64 year-old woman with signs of virilisation that had started 3 years before. Blood hormone analysis revealed increased levels of testosterone, and 17-hydroxyprogesterone. The tetracosactin test revealed 21-hydroxylase deficiency. Radiological imaging demonstrated a nodule in her left ovary. The patient was submitted to bilateral laparoscopic oophorectomy, and histopathological examination revealed a luteoma of the left ovary. Postoperative serum testosterone level and 17-hydroxyprogesterone returned to normal levels in one month. Virilism regressed within six months. Our patient also showed an elevation in 17-OHP serum levels. Normalization of 17-OHP after oophorectomy suggests a case of intratumoral 21-hydroxylase deficiency. To our knowledge, this is the first description of ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal woman.

Luteoma-induced fetal virilization.

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.

Boy born after gender preselection following successive gestational androgen excess of maternal luteoma and female disorders of sex development.

OBJECTIVE: To present male gender preselection after successive gestational hyperandrogenism and female pseudohermaphroditism by pregnancy luteomas. DESIGN: Case report. SETTING: University-based teaching hospital. PATIENT(S): We describe herein a gravida who presented two successive 46, XX disorders of sex development with apparent female genitalia with an enlarged clitoris due to maternal androgen excess from pregnancy luteoma. The maternal hyperandrogenemia returned to a normal level and bilateral luteoma regressed spontaneously postpartum. After gender preselection by Y-bearing spermatozoa for intrauterine insemination at the third gestation, a healthy boy was born in spite of the exaggerated maternal androgen production of pregnancy luteoma to avoid 46, XX disorders of sex development. INTERVENTION(S): Y-bearing spermatozoa for intrauterine insemination as male gender preselection. MAIN OUTCOME MEASURE(S): Boy born after gender preselection for intrauterine insemination and cesarean section. RESULT(S): Healthy boy born without disorder of sex development after antenatal maternal androgen excess of ovarian luteoma. CONCLUSION(S): Successive pregnancy luteomas associated with maternal hyperandrogenism may cause female disorders of sex development. Male preselection ameliorates the insult of external genitalia ambiguity by antenatal androgen excess in this matter of concern.

Maternal virilization due to luteoma associated with delayed lactation.

OBJECTIVE: To report on a patient with virilization during pregnancy who experienced delayed lactation secondary to elevated maternal androgens. DESIGN: Case report and review of the literature. SETTING: University hospital. PATIENT(S): A 32-year-old pregnant woman presented with virilization at 32 weeks' gestation. INTERVENTION(S): Laboratory evaluation, ultrasound examination, magnetic resonance imaging, cesarean section for fetal indication, nipple stimulation to facilitate lactation. MAIN OUTCOME MEASURE(S): Case report. RESULT(S): Postpartum normalization of serum T levels and patient ability to breastfeed exclusively after delayed initiation of lactation. CONCLUSION(S): Maternal virilization during pregnancy is rare and is often due to androgen-secreting tumors that are benign. Therefore, careful evaluation of these patients is important to avoid inadvertent oophorectomy.

Pregnancy luteoma presenting as ovarian torsion with rupture and intra-abdominal bleeding.

We report a case of pregnancy luteoma, which had undergone torsion in a 33-year-old Indian woman, who presented with severe abdominal pain and decreasing haemoglobin levels at 33 weeks gestation. Ultrasonography showed a right adnexal mass, probably ovarian in origin, with suspicious intratumoral bleed. The pain was treated symptomatically, and the symptoms improved. A successful induction of labour was then performed at 36 weeks gestation. The pain recurred almost immediately after the delivery, and she experienced another intra-abdominal bleed. A diagnostic laparotomy and a right salpingo-oophorectomy were performed, and the diagnosis of luteoma was made based on histology. We discuss the clinical presentation of this unusual tumour, though often asymptomatic, can rarely present with severe abdominal pain from complications like torsion with rupture, leading to massive intra-abdominal bleeding. We also discuss the possible radiological investigations which can be done during pregnancy.

An ovarian stromal tumor with luteinized cells: an unusual recurrence of an unusual tumor.

Sex cord-stromal tumors (SCSTs) of the ovary are uncommon. Their behavior is unpredictable, often with late recurrence, making counseling, management, and prediction of prognosis challenging. A 65-year-old woman presenting with a SCST underwent a bilateral oophorectomy, the histology was unusual but likely to be a luteinized thecoma with suspicious features for invasion. Two years later following a gastrointestinal bleed, a metastasis within the small bowel mucosa was detected. This represents probable isolated hematogenous or lymphatic spread, which is highly unusual especially in the absence of concurrent peritoneal disease. This is the first reported case of a SCST recurring in small bowel mucosa, mimicking a primary colorectal tumor. This highlights the diverse nature and behavior of these tumors.

Stromal luteoma: report of a rare case.

Steroid cell tumours (SCTs) account for less than 0.1% of all ovarian tumours. Three major categories ofSCTs include (1) stromal luteoma, (2) steroid cell tumour not otherwise specified and (3) Leydig cell tumours that do not have another component. Stromal luteomas constitute 20% of SCTs. They usually occur in postmenopausal women and about 60% present with estrogenic manifestations. We report a case of stromal luteoma in an elderly lady who presented with postmenopausal bleeding secondary to endometrial hyperplasia. An interesting finding in our case was the presence of many eosinophilic hyaline globules scattered throughout the tumour, the significance of which remains to be determined.

Maternal and female fetal virilization caused by pregnancy luteomas.

OBJECTIVE: To present the maternal and female fetal virilization caused by excessive androgen secretion of pregnancy luteomas. DESIGN: Case report. SETTING: University-based teaching hospital. PATIENT(S): A nulligravida woman suffering from bilateral hydronephrosis and recurrent acute pyelonephritis caused by bilateral solid ovarian tumors presented virilization during the third trimester. Without prompt surgical intervention, the maternal hyperandrogenemia returned to a normal level and regression of bilateral ovarian tumors occurred spontaneously after a female fetus with clitoral hypertrophy and temporal hyperandrogenemia was delivered. In spite of lacking histology, the clinical course is compared to that of pregnancy luteomas. INTERVENTION(S): Noninvasive imaging assessment. MAIN OUTCOME MEASURE(S): Maternal and female fetal virilization. RESULT(S): Regression of androgen-secreting pregnancy luteomas and hyperandrogenism during the puerperium but apparent female fetal clitoral hypertrophy. CONCLUSION(S): The maternal and female fetal virilization was caused by exaggerating androgen secretion of bilateral ovarian solid tumors. Spontaneous regression of ovarian tumors and hyperandrogenemia during the puerperium is the natural course of pregnancy luteomas, not true neoplasms.

MR imaging of pregnancy luteoma: a case report and correlation with the clinical features.

We report here on a 26-year-old pregnant female who developed hirsutism and virilization during her third trimester along with a significantly elevated serum testosterone level. Abdominal US and MR imaging studies were performed, and they showed unique imaging features that may suggest the diagnosis of pregnancy luteoma in the clinical context. After the delivery, the serum testosterone level continued to decrease, and it returned to normal three weeks postpartum. The follow-up imaging findings were closely correlated with the clinical presentation.

MR Imaging of Pregnancy Luteoma: a Case Report and Correlation with the Clinical Features

We report here on a 26-year-old pregnant female who developed hirsutism and virilization during her third trimester along with a significantly elevated serum testosterone level. Abdominal US and MR imaging studies were performed, and they showed unique imaging features that may suggest the diagnosis of pregnancy luteoma in the clinical context. After the delivery, the serum testosterone level continued to decrease, and it returned to normal three weeks postpartum. The follow-up imaging findings were closely correlated with the clinical presentation.

Prenatal diagnosis of female pseudohermaphroditism associated with bilateral luteoma of pregnancy: case report.

Female pseudohermaphroditism associated with luteoma of pregnancy (LP) is a rare condition characterized by varying degrees of masculinization of a female fetus. We describe a case, diagnosed at 13 weeks gestation. Transvaginal ultrasound at 5 weeks of gestation revealed a normal intrauterine gestational sac and an enlarged maternal right ovary. Re-examination at 13 weeks showed a fetus with male external genitalia. Cytogenetic investigation on amniotic fluid revealed a normal female karyotype 46,XX. Follow-up sonography confirmed the previous assignment of male external genitalia and a second amniocentesis was negative for the SRY gene. High levels of androgens were found in the maternal blood. A diagnosis of female pseudohermaphroditism associated with bilateral LP was made. A healthy girl was born by Caesarean section with complete masculinization of external genitalia (Prader V). Histology confirmed a bilateral LP. To the best of our knowledge this represents the first case of prenatal diagnosis of female pseudohermaphroditism associated with LP and demonstrates the feasibility of diagnosis by sonography from 13 weeks gestation. This is also the first case described of Prader V masculinization associated with LP.