Virilization in pregnancy due to a borderline mucinous ovarian tumor

Virilization in pregnancy due to borderline mucinous ovarian tumors is very rare. A case of a 28-year-old patient who was noted at 28 weeks' gestation to have marked virilization with raised serum androgens, ascites and a large complex right adnexal mass is presented. Delivery was carried out by cesarean section and at surgery a large tumor was noted in the right ovary. Histology revealed a borderline mucinous ovarian tumor with stromal luteinization, but there was no evidence of stromal invasion. Serum androgens returned to normal levels following surgery and the maternal virilization had resolved at the 6-week postnatal visit. Stromal changes in borderline mucinous ovarian tumors may result in virilization due to androgen production; surgical removal is associated with an excellent clinical outcome.     

An ovarian steroid cell tumor with virilization and hypothyroidism: a case report

Steroid cell tumors are rare ovarian sex-cord stromal tumors with malignant potential. The majority of these tumors produce steroids with testosterone being the most common. The diagnosis of these rare tumors can be problematic especially in the case of a small nonpalpable ovarian tumor. A 41-year-old woman presented with the gradual onset of defeminization, mild hypothyroidism, and virilization. A physical examination revealed a relatively healthy woman with borderline hirsutism, clitoromegaly and adnexal fullness without a palpable tumor. Elevated serum levels of TSH and testosterone were found preoperatively. Five weeks after an oophorectomy, serum levels of TSH and testosterone returned to normal and menstruation returned. Nonpalpable virilizing ovarian steroid cell tumors may be difficult to diagnose. A careful study of the patient’s history, the development of defeminization followed by virilization, and a “characteristic” ultrasonogram, can be helpful for diagnosis. Hormonal studies including thyroid function should also be considered in an ovarian steroid cell tumor.     

Virilizing ovarian Krukenberg tumor in a 27-year-old pregnant woman. A case report and literature review

A case is reported of a 27-year-old pregnant woman with ovarian tumors, measuring 12 cm and 11.5 cm in the greatest diameter, discovered during investigation for virilization symptoms. Termination of the pregnancy at the 22nd week of gestation and tumorectomy with both adnexa were performed, with the provisional diagnosis of arrhenoblastoma. Pathological examination of the tumors showed typical Krukenberg neoplasms and subsequent upper GI tract endoscopy revealed a gastric cancer that was excised. The pathological examination revealed a diffuse type gastric adenocarcinoma with signet ring morphology, similar to ovarian tumors. In any case of ovarian tumor with unusual hormonal manifestations, in addition to hormonally active sex cord-stromal neoplasms, metastatic ovarian tumors must be considered as well, especially in cases of bilateral tumors.     

Intraoperative venous blood sampling to localize a small androgen-producing ovarian tumor.

BACKGROUND: Among other causes of virilization, ovarian tumors can be the cause of excessive androgen production. We report the case of a Leydig cell tumor of the ovary where diagnostic attempts to localize the source of hyperandrogenism preoperatively failed owing to relatively small tumor size. CASE: A 36-year-old woman presented with clinical signs of severe virilization including progressive balding, increased hirsutism, secondary amenorrhea and enlargement of the clitoris. Extensive work-up included endocrinological tests, pelvic ultrasound, magnetic resonance imaging, chromosomal analysis, norcholesterol scintigraphy and selective venous sampling, without direct localization of the source of hyperandrogenism. Persistently high plasma testosterone prompted an explorative laparotomy. Intraoperative selective blood sampling of the ovarian veins and palpation gave evidence of a right ovarian tumor, which was then removed. Histological examination revealed the presence of a pure Leydig cell tumor. CONCLUSION: Exploratory laparotomy with intraoperative selective blood sampling of the ovarian veins might be a useful approach in patients without accurate preoperative localization of androgen-producing tumors of the ovaries.     

Sertoli cell tumor: a rare case in an elderly patient

Sertoli-Leydig cell tumors constitute < 1% of ovarian tumors, mostly in young women with virilization; however, not all present endocrine manifestations. A 72-year-old female presented with an abdominal mass and no signs of virilization. Total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy and selective pelvic lymphadenectomy was performed. The pathologic diagnosis was poorly-differentiated sex cord-stromal tumor with Sertoli cells. No adjuvant chemotherapy or radiation was administered. At 12-month follow-up the patient showed no evidence of disease.     

Diagnosis of a small,androgenizing Brenner cell tumor in a postmenopausal woman aided by laparoscopic salpingo-oophorectomy. A case report

BACKGROUND: Rapidly progressive hirsutism or virilization in the postmenopausal woman raises the suspicion of an androgen-secreting tumor. Hormonal testing and imaging studies usually rule out an adrenal tumor. However, small, androgenizing ovarian tumors may not be detectable by imaging studies. CASE: A postmenopausal woman presented with rapidly progressive hirsutism and elevated androgens. Imaging studies did not localize the tumor. Bilateral laparoscopic oophorectomy was performed, and a small, androgenizing Brenner cell tumor of the left ovary was demonstrated on histologic examination. CONCLUSION: Because of the low morbidity associated with laparoscopic salpingo-oophorectomy, it may be reasonable to remove the ovaries of postmenopausal women who display virilization or rapidly progressive hirsutism and elevated androgens, even if imaging studies do not detect the ovarian tumor.     

Androgen, estrogen, and progesterone by a lipid cell tumor of theovary.

In a 64-year-old woman with a virilizing lipid-cell tumor of the left ovary, serum progesterons, androgens, estrogens, and cortisol levels in the peripheral and ovarian veins were measured. Although virilization was the only symptom of hormone production by the tumor in this patient, endocrine studies showed that several steroids were secreted by this neoplasm. Of the steroids measured, androstenedione was the principal secretory product. Pregnenolone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, dehydroepiandrosterone, and testosterone were also secreted, but in quantities which were one third to one sixth the amount of androstenedione. The tumor's pattern of hormone secretion was similar to patterns of steroid production by ovarian stromal cells found in previously reported in vitro studies. This case and a review of the literature demonstrate that androstenedione appears to be the predominant secretory product of lipid cell tumors, whereas testosterone is the predominant secretory product of hilus cell tumors.     

Lipid cell tumor in an adolescent girl: a case report

BACKGROUND: Lipid cell tumors of the ovary are rare sex cord neoplasms and account for < 0.1% of ovarian tumors. CASE: A 16-year-old girl sought medical advice because of amenorrhea and virilization, manifested as facial hirsutism and temporal balding. Her total testosterone level was increased in comparison with normal values. Ultrasonography revealed an echogenic left adnexal tumor about 5.7 x 6.3 x 5.5 cm. The patient underwent laparoscopic ovarian cystectomy, and pathology showed a lipid cell tumor. Postoperatively, the serum testosterone level returned to normal. CONCLUSION: The tumor appeared to be the source of hyperandrogenism in this woman.     

Androgenic juvenile granulosa cell tumor of the ovary with cystic presentation: a case report

Granulosa cell tumors account for approximately 1-2% of all ovarian tumors. There are two types: adult granulosa cell tumor and juvenile granulosa cell tumor. Juvenile granulosa cell tumors constitute 5% of this histological subtype, and the prognosis is good because the majority present as Stage I tumors. The treatment can consist of conservative surgery. Androgen production is rare and produces virilization in women. These tumors are usually solid or predominantly solid. We describe the case of a 13-year-old girl with androgenic manifestations and increased abdominal size. Her plasma testosterone level was elevated. A left adnexal cyst (14.4 x 9.1 x 9.7 cm) was revealed at pelvic ultrasonography. The patient underwent an exploratory laparotomy, revealing a left ovarian cystic tumor. Diagnosis was juvenile granulosa cell tumor.     

Ovarian steroid cell tumor as an example of severe hyperandrogenism in 45-year-old woman

Abstract

Approximately, 5% of ovarian tumors have hormonal activity. Steroid cell tumors (SCTs) represent about 0.1% of all ovarian tumors. They cause hyperandrogenism associated with typical virilization. In this case report, we present 45-year-old women with unmalignant ovarian SCT-producing androgens which cause severe virilization and secondary amenorrhea lasting two years. Transvaginal ultrasound, computed tomography of adrenal glands, magnetic resonance imaging of small pelvis, laboratory tests (including serum concentration of FSH, LH, testosterone (T), androstenedione (A), dehydroepiandrosterone sulfate (DHEA-S), as well as ROMA index) were performed. During hormonal evaluation, elevated concentrations of serum T – on admission 1.72?ng/ml and one month later 3.75?ng/ml (normal range 0.08–0.82?ng/ml) and A – 24.90?ng/ml (normal range 0.40–3.40?ng/ml) were found. The ROMA index was within the normal range. Enlargement of the left ovary by solid mass 56?×?43?mm was found during ultrasound examination. Based on small pelvis MRI scan and hormonal finding, patient was qualified for laparotomy. During this procedure, the left salpingo-oophorectomy with removal of the tumor was performed. The histopathological examination identified SCT. During follow-up examination, one day after surgery, we found serum testosterone levels within normal ranges – 0.74?ng/ml (normal range 0.08–0.82?ng/ml). This case shows that hormone-producing ovarian tumors are rare but very important clinical causes of severe hyperandrogenism.     

Mucinous cystadenoma of the ovary with functioning stroma and virilization in pregnancy: a case report and review of the literature

Virilization caused by ovarian tumors with functioning stroma during pregnancy is extremely rare and has been reported in many ovarian tumors. In mucinous cystadenomas with maternal virilization during pregnancy the stromal cells responsible for the hormone secretion resemble lutein or Leydig cells and have been referred to as luteinized stromal cells. We present a case of a 30-year-old, gravida 2, para 1, woman who presented at approximately the 38th week of pregnancy with features of virilization. At the same time, a cesarean section was performed because of fetal distress and a male weighing 3,030 g without any gross abnormalities was delivered. A large tumor of the right ovary was detected and a right salpingo-oophorectomy was performed. Histopathologically, the tumor proved to be a benign mucinous cystadenoma. Masses typically resembling lutein stromal cells or Leydig cells of the testes or ovarian hilus were found in the wall of the cyst below the mucinous epithelium. No crystalloids of Reinke were identified. The stromal component of the tumor was characterized as functioning stroma with luteinized stromal cells. The glandular mucinous epithelium showed focal positivity for human chorionic gonadotrophin. The cytoplasm of the luteinized stromal cells reacted strongly and diffusely with antiserum for vimentin. Also, the cytoplasm of the luteinized stromal cells showed focal intense positivity for synaptophysin, and focal mild positivity for human chorionic gonadotrophin. Staining results for oestrogen and progesterone receptors were negative. In conclusion, we present an unusual case of clinical virilization during pregnancy associated with an ovarian mucinous cystadenoma with functioning stroma. The virilizing manifestations disappeared after removal of the ovarian neoplasm, supporting the perception that the functioning ovarian stroma was responsible for the androgen production.     

Youngest Reported Patient Presenting with an Androgen Producing Sclerosing Stromal Ovarian Tumor

BackgroundSclerosing stromal tumors are extremely rare sex cord stromal tumors of the ovary, with approximately 100 cases reported since first described in 1973. These tumors present predominantly in the 2nd and 3rd decades of life, typically present with pelvic/abdominal pain and tenderness, mass, and/or abnormal menses, and with hormonal activity reported predominantly in postmenarchal females. Only 5 cases of these tumors have been reported in premenarchal girls, with age ranging from 7 months to 12 years. Only 2 demonstrated hormonal manifestations, with vaginal bleeding due to hyperestrogenism in the 7 month old, and virilization in an 11-year-old female.CaseWe report a 9-year-old female who was diagnosed with this ovarian tumor, and who presented with virilization.Summary and ConclusionThis report is remarkable as our patient not only was diagnosed with an ovarian tumor that is extremely rare in this age group but is the youngest reported patient with this tumor who presented with virilization.     

Secretion of prorenin by a virilizing ovarian tumor.

A 53-year-old normotensive, normokalemic female presented with a 6-month history of virilization. Estradiol, LH, FSH, urinary-free cortisol, and DHEA-S levels were normal. Pelvic ultrasound and computerized tomography were also within normal limits. Her serum testosterone (551 ng/dl; nl, 20-70) and plasma prorenin (124 ng AI/ml/hr; nl, less than 50) levels were elevated. At surgery, a lipoid/steroid cell tumor of the right ovary was removed. Postoperative testosterone and prorenin levels were normal. Ovarian tumor cells, in culture, produced large amounts of prorenin. Immunohistochemistry localized prorenin and/or renin to tumor cells. Determining plasma prorenin levels may be a useful adjunct in diagnosing or following patients with nonepithelial ovarian tumors. A larger clinical study of prorenin levels in patients with such tumors is needed.     

Case report: an identical twin with Sertoli-Leydig cell tumor

Our report details the workup and management of a 43-year-old woman with an identical twin who presented with 2 years of virilization and secondary amenorrhea. Serum total testosterone was elevated. An MRI did not identify adnexal or adrenal pathology. Subsequent ovarian vein sampling demonstrated unilateral testosterone elevation. The patient underwent laparoscopic unilateral oophorectomy resulting in the diagnosis of Sertoli-Leydig cell tumor (SLCT). Although SLCT is a rare sex-cord ovarian tumor, it is associated with endometrial hyperplasia and malignancy. Our goals are to review the workup of androgen-secreting tumors and discuss the clinical importance of the DICER1 mutation in the context of SLCT. In this case, an identical twin underwent DICER1 testing which was one of the essential steps in her clinical management.     

Tumor ovárico virilizante mixto de células de Sertoli-Leydig/granulosa (ginandroblastoma) en adolescente de 15 años

Sertoli-Leydig cell tumors constitute less than 5% of ovarian tumors. We report the case of a 15-year-old girl with virilization, amenorrhea, and abdominal pain, who was diagnosed with a left annexal tumor. Laboratory investigations revealed isolated testosterone elevation. Because the tumor was unilateral, the capsule was intact, and the patient wished to preserve her fertility, left salpingooophorectomy was performed. Histopathological examination revealed a mixed Sertoli-Leydig/juvenile granulosa cell tumor with a cartilaginous heterologous component, which could be considered a gynandroblastoma. Symptoms of virilization progressively improved.     

Virilization due to a rare ovarian tumor in a postmenopausal woman

A 60-year-old postmenopausal woman presented with evidence of hirsutism, alopecia and mild virilization. Clinical examination and biochemical abnormalities suggested that the source of androgen excess was ovarian, and an ovarian tumor was confirmed and removed at laparotomy followed by normal endocrine profile in the postoperative period.     

Preoperative localization of virilizing tumors by selective venous sampling

Two postmenopausal patients with virilization had preoperative localization of ovarian tumors by selective blood sampling from both ovarian and adrenal veins and assay of hormone levels. In the first patient, the peripheral concentrations of testosterone (T), androstenedione, and estrone were 936, 1,508, and 73 pg. per milliliter, respectively, levels which are above the ranges found in normal postmenopausal women. The catheterization study showed an increase in the left ovarian vein of all hormones except cortisol. It was predicted that a tumor was present in the left ovary. At operation a 7 by 4 mm. lipid cell tumor was found. In the peripheral blood of the second patient, the T level (4,518 pg. per milliliter) was markedly elevated and the estradiol concentration (73 pg. per milliliter) was increased. At retrograde catheterization the concentration of T in the right ovarian vein was markedly elevated at 120,400 pg. per milliliter. At operation a hilus cell tumor of the right ovary was found. These two cases represent the third and fourth consecutive androgen-secreting tumors from this institution that have been localized by selective ovarian and adrenal vein catheterization and sampling.     

卵巢支持莱狄细胞瘤11例临床分析

目的探讨卵巢支持莱狄细胞瘤的临床特征、治疗和预后。方法 回顾性分析了我院1962—2002年治疗的11例卵巢支持莱狄细胞瘤患者的临床和病理资料。结果11例患者中,病理分化程度:高分化7例,中分化3例,低分化1例。临床分期:Ⅰ a期9例,Ⅱc期及Ⅲc期各1例。临床症状:腹部包块8例;去女性化及男性化表现共6例,其中3例行血清睾酮水平测定,均不同程度升高;月经增多、绝经后阴道出血等女性化表现者5例,其中1例同时具有女性化及男性化表现。合并症:5例合并与雌激素相关的疾病,包括子宫肌瘤、子宫内膜增生等,2例患者先后患乳腺癌。治疗:11例患者均行手术治疗,其中5例肿瘤分化不良或Ⅱ～Ⅲ期者,术后行系统性巩固化疗。随诊:随诊时间为6个月至34年,中位数随诊时间为7年,无一例死于本病。3例患者保留生育功能,均于术后1—3个月恢复月经,其中1例已生育。结论卵巢支持莱狄细胞瘤预后良好。早期、高分化者,可单纯手术治疗。分化不良或晚期肿瘤患者,术后应予巩固化疗。有生育要求者,可保留生育功能。     

A rare ovarian Leydig cell tumor (hilar type) causing virilization in a postmenopausal woman

All patients with virilization signs, increased levels of androgen hormones and rapidly progressive hirsutism should be evaluated for an androgen-producing tumor. The ovarian origin of virilization can be suspected by the presence of elevated levels of circulating androgens, with normal levels of cortisol metabolites and a negative dexamethasone suppression test. A case report of a 50-year-old postmenopausal patient with rapidly progressive hirsutism is presented. After an extensive preoperative investigation a right oophorectomy was performed and a Leydig-hilus cell tumor was diagnosed.     

Virilizing ovarian tumor of cell tumor type not otherwise specified: a case report

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.