Cases reported "Granulosa Cell Tumor"

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1/11. Masculinizing granulosa cell tumor of the ovary in a postmenopausal woman. A case report.

    BACKGROUND: A total of 32 cases of virilizing granulosa cell tumors of the ovary have been reported. The current case has some unique features not previously reported. CASE: A 78-year-old woman presented with symptoms and signs of masculinization. A large, painless abdominal mass was discovered. Exploration revealed the mass to be originating in the left ovary, and surgical resection resulted in prompt reversal of the clinical and biochemical hyperandrogenic manifestations. Morphologic studies demonstrated a homogeneous granulosa cell tumor. CONCLUSION: This is the oldest patient on record with a masculinizing granulosa cell tumor and also the only masculinizing tumor presenting with advanced, stage III disease. Such tumors, although rare, should be considered in the differential diagnosis in postmenopausal women presenting with masculinizing symptoms.
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2/11. Androgenic adult granulosa cell tumor in a 13-year-old prepubertal patient: a case report and review of the literature.

    We report the clinicopathologic findings of an unusual case of adult granulosa cell tumor with androgenic manifestation in a 13-year-old prepubertal girl. The patient had never had a menstrual period and presented with a 1 year history of hirsutism. physical examination was only remarkable for an increase in facial and abdominal hair, both with a male pattern of distribution. A pelvic ultrasound demonstrated a 6.0 cm right adnexal cyst. plasma testosterone and 17-hydroxyprogesterone levels were elevated. The patient initially was treated with monophasic oral contraceptive pills for 3 months and owing to lack of response, she underwent an exploratory laparotomy in which a left ovarian tumor, 7.0 cm in greatest dimension, and a 6.5 cm right paratubal cyst were found. A wedge biopsy of the left ovary and subsequent left oophorectomy with right salpingectomy were performed. No gross evidence of disease outside the ovary was noted. Microscopic examination of the left ovarian tumor revealed the typical features of an adult granulosa cell tumor. No tumor was seen outside the ovary. Six days after surgery, plasma testosterone and 17-hydroxyprogesterone levels were in the normal range. Nine months postoperatively, the patient shows no evidence of disease. To our knowledge, this represents the first case of a prepubertal patient with an adult granulosa cell tumor with androgenic manifestations reported in the English literature.
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3/11. Ectopic bioactive luteinizing hormone secretion by a pancreatic endocrine tumor, manifested as luteinized granulosa-thecal cell tumor of the ovaries.

    Endocrine pancreatic tumors are rare neoplasms consisting of multipotent cells capable of secreting various bioactive substances causing characteristic clinical syndromes. Ovarian stromal hyperthecosis is characterized by varying degrees of luteinized stromal cell proliferation after sustained LH and/or human chorionic gonadotropin stimulation, clinically manifested by symptoms/signs of virilization resembling the polycystic ovary syndrome (PCOS). We report a case of ectopic bioactive LH production from a pancreatic endocrine tumor in a 33-yr-old woman with rapidly developing symptoms/signs of hyperandrogenism and markedly elevated serum androgen and LH levels leading to hyperthecosis and bilateral luteinized granulosa-thecal cell tumors of the ovaries. Although the patient was initially thought to have either severe PCOS or an LH-secreting pituitary tumor, an LH-producing pancreatic endocrine tumor bearing somatostatin receptors was demonstrated on scintigraphy with [111In]octreotide and abdominal imaging. Symptoms and signs of hyperandrogenism resolved after the resection of the tumor. immunohistochemistry, in situ hybridization, and electron microscopy studies confirmed LH synthesis by the tumor cell. Although extremely rare, ectopic LH production from nonpituitary endocrine tumors should be considered in the differential diagnosis of hyperandrogenism, particularly when associated with highly elevated serum LH levels.
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4/11. Androgenic adult granulosa cell tumor in a teenager: a case report and review of the literature.

    The clinicopathologic findings of the third case of androgenic adult granulosa cell tumor in patients younger than 15 years was presented and discussed in the light of the literature. A patient complaining of secondary amenorrhea and hirsutism with elevated levels of plasma total testosterone, dehydroepiandrosterone sulfate, free androgen index and serum inhibin A, and a left ovarian septated, cystic mass was admitted to the hospital. The inhibin A level was within normal levels in the first month postoperatively. Inhibin A could be a tumor marker of utmost importance particularly in patients with androgenic or hyperestrogenic symptoms, especially in cases where benign criteria are abundant such as young age, nonincreased levels of classic tumor markers, and ultrasonographic appearance without any suspicion of malignancy.
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5/11. Unusual presentation of juvenile granulosa cell tumor of the ovary.

    BACKGROUND: We present a case report of juvenile granulosa cell tumor of the ovary (JGCT) with an unusual clinical presentation and hormonal secretion. CASE: A 16-yr-old girl had developed spontaneous menarche at the age of 12 yr, but after this initial menstrual bleeding she had no further periods for 4 yr. She had no clinical signs of virilization. Endocrinological studies detected high levels of DHEA, 17 hydroxyprogesterone (17OH-P), insulin and PRL, an exaggerated DHEA response after ACTH stimulation, and low FSH and high LH values after GnRH. An ultrasound examination revealed an irregular structure and increased diameters of her right ovary, due to the presence of a cyst. Because exploratory laparoscopy revealed the presence of a right ovarian mass, her right ovary was removed. JGCT was diagnosed. Ten days after surgery, menstrual bleeding initiated. Endocrinological evaluation after the operation showed that 17OH-P, insulin and basal FSH and LH serum values had returned to normal, while DHEA levels had decreased to within the upper limit of the normal range. Only PRL levels remained unchanged. CONCLUSION: Our patient presented some unusual characteristics. She did not have precocious puberty, but secondary amenorrhea. Hormonal secretion consisted mainly of androgens, even though clinical signs of virilization were not present.
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6/11. Malignant sex cord-stromal tumour in a patient with the androgen insensitivity syndrome.

    A malignant sex cord-stromal tumour that occurred in a 56-year-old patient with the androgen insensitivity syndrome is reported. Although hamartomas composed of sex cord cells are common in the testes of patients with this syndrome, unequivocal neoplasms of sex cord type are rare. The tumour described herein most closely resembled, but lacked the overall morphology of, a juvenile granulosa cell tumour, and Charcot-Bottcher filaments, indicative of Sertoli cell differentiation, were seen on electron microscopy. The features of the androgen insensitivity syndrome and the various tumours that have been reported in patients with this syndrome are briefly reviewed.
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7/11. Characteristics of in vitro steroidogenesis in a woman with hyperandrogenism, a granulosa cell tumor and polycystic ovary disease. A case report.

    A 20-year-old woman presented with hyperandrogenism presumed to be due to an androgen-secreting granulosa cell tumor. In order to confirm this unusual presentation, short-term tissue culture steroidogenesis of the granulosa cell tumor and contralateral polycystic ovary was performed. testosterone and androstenedione were produced only by the polycystic ovary, whereas the tumor was producing estradiol and progesterone. The studies of in vitro steroidogenesis prevented an incorrect interpretation of the clinical presentation.
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8/11. Androgenic juvenile granulosa cell tumour. Case report.

    An androgenic juvenile granulosa cell tumour of the ovary in a postmenarcheal girl is presented. Distinctive histological features of this variant are stressed and virilizing effects are explained by an immunohistochemical study of hormones produced by granulosa and theca cells.
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9/11. Androgenic granulosa cell tumors of the ovary. A clinicopathologic analysis of 17 cases and review of the literature.

    We reviewed the clinicopathologic features of 17 androgenic granulosa cell tumors. The patients, two of whom were pregnant, ranged from 2 to 58 years of age (average age, 22.5 years). Fourteen of the patients were masculinized as evidenced by various combinations of facial acne, temporal recession of hair, clitorimegaly, deepening of the voice, and a male escutcheon. Five of these patients had amenorrhea; three of the patients demonstrated hirsutism only. The plasma testosterone level is known to have been elevated preoperatively in nine patients, including the three patients with hirsutism. Four prepubertal patients also demonstrated hyperestrinism as manifested by sexual pseudoprecocity. At laparotomy, all of the tumors were stage lai. Five of the tumors formed unilocular and two of the tumors formed multilocular thin-walled cysts that ranged from 23 to 36 cm in diameter. Nine of the remaining tumors were solid, and one tumor was solid and cystic; these tumors averaged 7 cm in diameter. Eleven tumors were granulosa cell tumors of the adult type, and six tumors were juvenile granulosa cell tumors. The only clinically malignant tumor in the series was a unilocular, cystic, adult granulosa cell tumor that recurred 18 months postoperatively and was fatal within four years.
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10/11. Amenorrhoea and failure to virilize in a patient with a testosterone secreting granulosa cell tumour.

    A 39-year-old woman presented with secondary amenorrhoea in the absence of galactorrhoea, hirsutism or virilization. Investigation revealed a strikingly elevated serum testosterone, dihydrotesterone, free testosterone and LH level. At laparotomy a large granulosa cell tumour was encountered and totally removed. Analysis of the tumour revealed 127 ng of testosterone/mg of tumour tissue. The testosterone was localized immunocytochemically to the granulosa cells, which demonstrated typical ultrastructural characteristics of oestrogen rather than androgen secreting tissue. A study of androgen binding and metabolism prior to tumour resection revealed a normal androgen receptor as evidenced by normal maximum binding, dissociation constant and nuclear uptake. Removal of the tumour resulted in a return to normal of all abnormalities. We conclude that granulosa cell tumours may rarely secret large amounts of testosterone and that in this situation their ultrastructure is unchanged. Furthermore, absence of LH suppression or hirsutism in an amenorrhoeic patient does not dismiss the possibility of a significant androgen-secreting tumour. In this situation, the failure to virilize may be due to a post-nuclear translocational defect.
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