Ovarian dysgerminoma with pseudo-Meigs syndrome: A case report.

RATIONALE: Dysgerminoma is a rare malignant tumor of the ovary, more frequently occurring in young women. The main signs of pseudo-Meigs syndrome (PMS) are ascites and hydrothorax accompanying benign or malignant ovarian tumors (no fibroma or fibroma-like tumor). PATIENT CONCERNS: A 19-year-old woman with fever and chest tightness for 2 days. DIAGNOSES: Pectoral-abdominal computed tomography (CT) scan and contrast-enhanced magnetic resonance imaging revealed a large amount of right pleural effusion, a small amount of ascites, and a huge abdominopelvic mass measuring about 29.2cm × 11.8cm × 8.4 cm in the left ovary. The result of hydrothorax examination was consistent with the diagnosis of exudative pleural effusion. In addition, Rivalta-test showed a positive result and lactate dehydrogenase was elevated. The histopathological diagnosis was a giant germ cell tumor, which was consistent with dysgerminoma in terms of both morphology and immunophenotype. Based on these findings, a diagnosis of malignant ovarian neoplasm with PMS was made. INTERVENTIONS: Surgical resection of the tumor was performed. OUTCOMES: The patient recovered well after operation, and the pleural effusion and abdominal ascites vanished. No recurrence was observed during the 1-year follow-up period. LESSONS: Ovarian dysgerminoma with PMS is a rare malignant tumor of the ovary, which often occurs in young women. It should be considered in differential diagnosis of patients with a pelvic mass, ascites and pleural effusion. Early diagnosis and surgical treatment are beneficial to prolonged survival.

Normal pelvic ultrasound or MRI does not rule out neoplasm in patients with gonadal dysgenesis and Y chromosome material.

INTRODUCTION: Patients with gonadal dysgenesis (GD) with a Y chromosome have an increased risk of gonadal neoplasm. Few data exist on the ability of imaging to detect malignancy in intra-abdominal gonads in these patients. OBJECTIVE: We aimed to determine the correlation between preoperative imaging findings and gonadal pathology in GD patients with Y chromosome material. METHODS: A retrospective review was performed of patients with XY or XO/XY GD who underwent gonadectomy at our institution from 2003 to 2017. Patients were assessed preoperatively with ultrasonography; some additionally underwent MRI. RESULTS: The series consisted of 10 patients, all with female gender and non-palpable gonads. Median age was 13.1 years (range 2.4-18.3 years). Overall, four of the ten patients (40%) had a tumor (gonadoblastoma or dysgerminoma) on final pathology. Four patients had a gonad or gonads that were definitively seen on ultrasonography. All visualized gonads were described as "normal" or "small" with the exception of one patient, who had a normal MRI. Three of the four patients in this group had a tumor on final pathology. The remaining six patients had a gonad or gonads that were not definitively visualized on ultrasound; one patient in this group had a tumor on final pathology. Overall, five of seven gonads (71%) definitively visualized on ultrasound had tumor on final pathology, and two of thirteen gonads (15%) not visualized on ultrasound had tumor on final pathology; this difference was statistically significant (p = 0.012). Three patients were imaged with MRI. Of the gonads that could be visualized on MRI, no definitive abnormalities were seen. All patients imaged with MRI had tumors on final pathology. DISCUSSION: Both ultrasound and MRI are relatively poor at identifying and characterizing intra-abdominal gonads in GD patients. The majority of patients who had a neoplasm had normal imaging findings. Gonads that were definitively visualized on ultrasound were more likely to contain neoplasms that could not be visualized, which perhaps because of tumor growth. No other consistent imaging findings of malignancy were found. Our study included ultrasound evaluations that were completed over 10 years ago and not performed by pediatric ultrasonographers, which may have biased the results. However, results suggest that when discussing gonadectomy with GD patients, one should not be reassured by "normal" imaging findings. Neither ultrasound nor MRI should be relied on for surveillance in GD patients who decide against gonadectomy. CONCLUSION: A normal ultrasound or MRI does not rule out neoplasm in GD patients with intra-abdominal gonads.

Gonadoblastoma bilateral y disgerminoma asociados en un síndrome de Swyer / Bilateral gonadoblastoma and dysgerminoma associated with Swyer syndrome

El síndrome de Swyer es una disgenesia gonadal pura caracterizada por fenotipo femenino y cariotipo XY. Las pacientes presentan unas gónadas disgenéticas y no funcionantes. El riesgo de neoplasia gonadal es alto, entre un 25 y un 30%. El gonadoblastoma y el disgerminoma son los tumores más frecuentes. Presentamos un caso de gonadoblastoma bilateral y disgerminoma asociados en una paciente con este síndrome (AU)

Swyer syndrome is a pure gonadal dysgenesis with female phenotype and 46 XY karyotype. Affected individuals have dysgenetic and non-functioning gonads. The risk of gonadal neoplasia is high at between 25% and 30%. The most frequently reported malignancies are gonadoblastoma and disgerminoma. We report a case of bilateral gonadoblastoma and dysgerminoma in a female patient with this syndrome (AU)

Pure dysgerminoma of the ovary 35 years on: a single institutional experience.

OBJECTIVE: The aim of this study was to evaluate clinicopathologic characteristics, long-term outcome and reproductive function in women diagnosed with pure dysgerminoma of the ovary. METHODS: Sixty-five women with stage IA to IIIC pure ovarian dysgerminoma were identified and included in this retrospective study. Patients were treated at one institution between 1970 and 2005. RESULTS: Median age at diagnosis was 22.2 years (range 8.2-64.1 years). 72.3% of patients presented with stage I, 4.6% stage II and 21.5% stage III disease (1.5% stage unknown). Initial management was surgical for all patients: unilateral oophorectomy in 47 patients (72.2%), bilateral oophorectomy +/- hysterectomy in 14 (21.5%) and cystectomy alone in 3 (4.6%). Seventeen patients received chemotherapy (15 adjuvant, 2 for residual disease), 20 received adjuvant radiotherapy and one patient received both. Recurrence occurred in 6 (9.2%) patients (5 stage IA, 1 stage IIA). All recurrences occurred within 19 months of primary diagnosis. All patients were successfully salvaged with radiotherapy (2 patients), chemotherapy (1 patient) or a combination of surgery and chemotherapy (3 patients). Overall, median follow up from time of recurrence was 22.5 years (range 9.3-31.4 years). Median follow-up of all patients was 10.5 years (range 1.1-31.9 years). Fifteen patients reported an attempt to conceive posttreatment resulting in 12 pregnancies and 12 live births in 8 women. CONCLUSION: The long-term outcome of patients with pure ovarian dysgerminoma is excellent. Recurrences occur within 2 years of diagnosis and are treatable. Patients can be treated with fertility-sparing surgery and can expect good reproductive outcomes.

The role of leptin, soluble leptin receptor, resistin, and insulin secretory dynamics in the pathogenesis of hypothalamic obesity in children.

INTRODUCTION: In this study, we have investigated the role of leptin, soluble leptin receptor(sOb-R), resistin, and insulin secretory dynamics in the development of hypothalamic obesity. MATERIALS AND METHODS: Children who had hypothalamo-pituitary tumor were divided into two groups. First group included obese-overweight (hypothalamic obese = HOB group, n = 23) and second group included non-obese children (hypothalamic non-obese = HNOB group, n = 16). Exogenously obese-overweight children (OB group, n = 22) were included as controls. Basal and second-hour serum glucose and insulin in oral glucose tolerance test (OGTT), basal serum leptin, sOb-R, resistin levels, and homeostasis model assessment (HOMA) indexes were compared between the groups. RESULTS: Age, sex, and pubertal status were similar in study groups. Median and interquartile ranges of body mass index (BMI) z scores were similar in HOB and OB groups (2.0 (1.5-2.1) and 2.1 (1.8-2.3), respectively). Serum leptin levels corrected for BMI were highest and total leptin/sOb-R ratios (free leptin index (FLI)) tended to be higher in HOB than HNOB and OB groups, indicating leptin resistance (leptin/BMI, 4.0 (1.6-5.2), 1.5 (0.8-3.1), and 2.5 (1.8-3.5); FLI, 2.0 (0.8-3.5), 0.6 (0.3-1.2), and 1.5 (1-2.3) in HOB, HNOB, and OB groups; respectively). Serum resistin levels were similar in groups (2.6 (1.9-3.1), 2.8 (1.7-3.4), and 3.0 (2.2-3.5) ng/ml in HOB, HNOB, and OB groups, respectively). Basal serum glucose, basal and second-hour insulin levels in OGTT, and HOMA index were higher in OB group than the HOB and HNOB groups, indicating insulin resistance in simple obesity; however, increment of insulin to same glycemic load in OGTT was highest in the HOB group indicating insulin dysregulation (p < 0.05). CONCLUSION: Hypothalamic obesity seems to be related to both dysregulated afferent (leptin) and efferent (insulin) neural outputs through the autonomic nervous system resulting in energy storage as fat.

Observations of rat ovarian-splenic consecutive transplants.

We examined the results after implantation of ovarian follicles by various modes in a total of 82 cases. One or five ovarian follicles were implanted into spleens in castrated female rats. In 20 cases among these, each five follicles were implanted into native and transplanted spleens after spleen transplantation (double implantation of the ovary). Through consecutive spleen transplantation, we observed the results beyond the rat's life span for a limited period. In many cases, we found a more aggressive form of malignant tumor, i.e., dysgerminoma. We present the results and discuss the modes of implantations and possible pathogenetic mechanisms of tumor formation.

Assessing the hypothalamo-pituitary-adrenocortical axis using physiological doses of adrenocorticotropic hormone.

We compared cortisol responses to 1 microgram adrenocorticotropic hormone (ACTH), 250 micrograms ACTH and insulin-induced hypoglycaemia (IIH), in patients suspected to have secondary hypocortisolism. Twenty-four patients (16 with hypothalamopituitary disorders and 8 on long-term glucocorticoid therapy) and eight healthy controls, underwent all three test protocols, with intervals of one day between each test. Mean cortisol responses to all three tests were comparable in both groups, but were more closely correlated for IIH vs. the 1 microgram ACTH test (r = 0.96) than for IIH vs. the 250 micrograms ACTH test (r = 0.88). Seven patients had discrepant results; all had a normal peak cortisol response to 250 micrograms ACTH (> 550 nmol/l), but a subnormal response to 1 microgram ACTH. Six of these also had a subnormal response to IIH. Cortisol responses to IIH match more closely those for 1 microgram ACTH in individual instances than those for 250 micrograms ACTH. The standard 250 micrograms ACTH stimulation, being supraphysiological, leads to underdiagnosis of the hypocortisolaemic state. The 1 microgram ACTH stimulation test should replace the standard 250 micrograms ACTH stimulation test in assessing the hypothalamo-pituitary-adrenocortical axis in secondary hypocortisolism.

Disgerminoma puro de ovario: presentación de dos casos

Siendo el Disgerminoma el tumor de células germinales más común en mujeres jóvenes, es también de los menos frecuentes de los tumores de ovario, oscilando su incidencia en Colombia del 0.48 a 0.55 por ciento. Se presentan dos casos atendidos en el Departamento de Ginecología y Obstetricia del Hospital Universitario Metropolitano en un período de 10 años

Etoposide y displatino (EP) en el tratamiento de tumores germinales de testículo / Etoposide and cisplatin (EP) in the treatment o germinal testicular tumors: factors which affect tumoral reaction to treatment

Entre 1986 y 1990 se estudió un grupo de 51 enfermos con tumores germinales del testículo, tratados con etoposide y cisplatino, con el propósito de conocer la reacción tumoral a este esquema de tratamiento y establecer que factores clínicos e histopatológicos influyen en ella

Factores clínico-histopatológicos relacionados con la evolución del seminoma: informe preliminar de 15 casos / Clinical-histopathological factors related to the evolution of a seminoma: preliminary report of 15 cases

Se estudió un grupo de 15 pacientes con diagnóstico de seminoma, seleccionado al azar, en el que se relacionaron variables clínicas e histopatológicas con el propósito de establecer que están implicadas en la evolución de este tumor. El grupo de pacientes se dividió en: bajo risgo (estadío clínico I, IIa, IIb) y en alto riesgo (estadío clínico IIc y III). El grupo de bajo riesgo está formado por siete pacientes, todos con seminomas clásicos. El grupo de alto riesgo está formado por ocho individuos, de los cuales, seis son seminomas clásicos y dos anaplásicos. El análisis estadístico de ambos grupos de variables entre los grupos de bajo y alto riesgo no fue significativo

Paternity in a patient with testicular seminoma and contralateral testicular intraepithelial neoplasia.

A 32-year-old patient with unilateral beta hCG-positive seminoma and contralateral testicular intraepithelial neoplasia (TIN; so-called carcinoma-in-situ) with no metastases (clinical stage I) received one course of adjuvant carboplatin therapy. He refused further treatment of TIN in his remaining testis. His wife became pregnant by him 4 months later and delivered a healthy child at term. This case shows that patients with TIN in their remaining solitary testis are not necessarily infertile, and testes afflicted with TIN must also contain tubules that retain normal spermatogenic potential. Surveillance may be an treatment option for patients with TIN in their remaining testis in cases where there is a strong desire for paternity.

[Fertility after treatment for testicular tumor].

Thirty-four patients with stage 1 or stage 2A testicular tumor all survived 5 years after the treatment, whereas eighteen patients with stage 2B or 3 testicular tumor, including only nonseminomatous tumor, had a 5-year survival rate of less than 50%. Spermatogenesis returned to normal in 3 patients surviving 22 months after chemotherapy. Five patients who had undergone retroperitoneal lymph node dissection with division of the inferior mesenteric artery developed impaired ejaculation, whereas 3 patients who had undergone the operation without division of the inferior mesenteric artery had normal ejaculation. In 17 patients with right testicular tumor metastases were found in the para-aortic, paracaval and interaortocaval lymph nodes. On the other hand, in 4 patients with left testicular tumor metastases were limited to the para-aortic nodes. These results indicate that impaired spermatogenesis by conventional chemotherapy is reversible in patients with stage 1 or 2 testicular tumor, and patients with stage 1 tumor or stage 2 tumor with localized para-aortic metastases, not involving the inferior mesenteric artery, should undergo retroperitoneal lymph node dissection without division of the inferior mesenteric artery to preserve postoperative fertility.

Semen quality after treatment for testicular cancer.

During the last 14 years repeated sperm cell analyses and serum hormone analysis were done regularly in patients with testicular cancer before and after treatment. The summarized results are hereby reported. The sperm cell count was highly impaired in 60-70% of testicular cancer patients evaluated 1-4 weeks after unilateral orchidectomy before further treatment. However, improvement of the exocrine gonadal function was regularly observed during the following 2-3 years, provided there were normal or only slightly elevated pre-treatment FSH levels. Standard irradiation and cytotoxic treatment (abdominal radiotherapy 30-40 Gy; < or = 4 cycles of cisplatin-based chemotherapy) delayed this recovery by about one year. Higher doses of chemotherapy or combined irradiation/chemotherapy reduced the chances of rapid improvement of the exocrine gonadal function. Unilateral retroperitoneal lymph node dissection resulted in a slight reduction of the post-treatment ejaculatory volume, even in patients with preserved antegrade ejaculation.

Influence of testicular carcinoma on ipsilateral spermatogenesis.

A histological review of radical orchiectomy specimens was performed to assess the impact of testicular cancer on spermatogenesis. Slides from 28 patients with testicular cancer were available for review, consisting of 14 pure seminomas, 12 embryonal carcinomas and 2 mixed tumors. For each specimen tubules adjacent (less than 3 mm.) to the tumor and distant (more than 3 mm.) from the tumor were evaluated. This study indicates that marked impairment of ipsilateral spermatogenesis is associated with testicular carcinoma, particularly in the vicinity of the tumor. The quality of distant spermatogenesis appears to be influenced by tumor type and not by elevation of known serum tumor markers, such as human chorionic gonadotropin and alpha-fetoprotein, nor by the presence of carcinoma in situ.

A phase II trial of early intensive chemotherapy with autologous bone marrow transplantation in the treatment of poor prognosis non seminomatous germ cell tumors.

Twenty-eight patients with poor prognosis, advanced metastatic non seminomatous germ cell tumors (NSGCT) were treated with early high dose chemotherapy and autologous bone marrow transplantation (ABMT) rescue. The primary tumor was testicular in 19 patients and extragonadal in nine patients. For 19 patients with a testicular primary, the median probability of complete remission (CR) was 0.05 according to our prognostic mathematical model based on pretreatment levels of serum HCG and AFP. The same prognostic model was used for extragonadal primaries. Treatment consisted of two cycles of a modified double dose of cisplatin, vinblastine, bleomycin, VP-16 regimen (mPVeBV) followed by a high dose cisplatin-etoposide-cyclophosphamide regimen (PEC) followed by ABMT. Of the 28 patients, 17 (61%) achieved CR, one of which was surgical CR (sCR), five died of rapidly progressive disease early during the first cycle of mPVeBV, two had treatment-related deaths, three did not respond and one patient refused treatment. Of the 17 patients initially in CR, three relapsed after 4, 4 and 7 months respectively and have subsequently died. Two other patients died while still in CR: one committed suicide and one died of an infectious complication due to transfusion-related AIDS. Twelve patients are alive in CR after a median follow-up of 66 months (range 7-72 months). The non parametric 3-year survival rate is 40%. To demonstrate the effect of intensive chemotherapy with ABMT, a randomized multicenter French study was set up to evaluate the PVeBV regimen with or without high dose treatment and ABMT in poor risk NSGCT patients.

Postradiation lower motor neuron syndrome presenting as monomelic amyotrophy.

Monomelic amyotrophy developed 16 months, nine and 12 years after irradiation of the lumbosacral spinal cord for seminoma in one patient and for Hodgkin's disease in two others. In two patients, involvement was clinically limited to one leg, with a subacute course followed by plateau in the first case and with progressive worsening in the second one. In the third patient, the course was progressive with involvement of the other lower limb occurring five years later. From clinical and electrophysiological data, it seems probable that the disease process was a result of a selective injury to the lower motor neuron in the lower spinal cord.

Elevated intrascrotal temperature and the incidence of testicular cancer in noncryptorchid men.

The possibility that intrascrotal temperature is involved in the etiology of testicular cancer was investigated through a population-based case-control study conducted in western Washington State. A total of 323 men with germ cell tumors of the testis diagnosed between 1977 and 1984 and 658 randomly selected controls were interviewed with regard to type of shorts worn, use of long underwear, heat-resistant clothing, and hot tubs or saunas, and a history of varicocele. No association was found between testicular cancer and having worn tight-fitting underwear (jockey shorts) or having worn heat-resistant clothing on the job. A 50% increase in risk was observed for men who typically wore long underwear for more than three months out of the year, but this association was within the limits of chance given no true association. A smaller fraction of the men with cancer than controls had used a hot tub or sauna in the preceding five years, but the size of the case-control difference was not related to the frequency of use. The relative risk associated with a history of physician-diagnosed varicocele was 1.8 (95% confidence interval 0.9-3.4). There was little difference between men with seminomas and those with other germ cell tumors regarding any of the above exposures. Taken in aggregate, these results provide little or no support for the hypothesis that intermittent intrascrotal temperature elevation, to the degree encountered by US men today, plays a role in the etiology of germ cell testicular cancer. The possibility that risk increases after a continuous temperature increase, such as might result from a varicocele, needs to be evaluated further.

[Hypernatremia due to hypothalamic tumor: ADH response to changes in plasma osmolality].

A case of a patient with the syndrome of chronic hypernatremia and hypodispia due to hypothalamic tumor was studied to evaluate the change of ADH response to plasma osmolality during the clinical course. A 23-year-old man was admitted for investigation of anorexia, hypodipsia and gait disturbance. Examination showed memory disturbance and generalized muscle weakness. Investigation showed marked hypernatremia (177 mEq/l) and hypopituitarism. Water loading test showed that ADH was not stimulated by hyperosmolality but continued to be secreted at a more or less constant level approximating normal basal state. CT scan revealed hypothalamic tumor. The tumor was suspected to be germinoma due to its radiosensitivity and high serum hCG level. After irradiation, the tumor lesion disappeared. ADH secretion came to be responsive to changes in osmolality but the response of the system was markedly reduced compared with the normal response, and hypodipsia and hypernatremia still remained. We conclude that the adipsia and complete destruction of the osmoreceptor in the patient caused marked hypernatremia and the destruction of ADH osmostat improved partially after irradiation. We believe it very useful for analyzing the disturbance of osmoregulatory system to evaluate the relationship of plasma ADH to plasma osmolality.