Complete response to paclitaxel, ifosfamide, and cisplatin therapy in a case of ovarian ependymoma.

There are many reports on paclitaxel, ifosfamide, and cisplatin (TIP) therapy, following standard bleomycin, etoposide, and cisplatin (BEP) therapy, for salvage treatment of testicular malignant germ cell tumors, but there are no reports on its use for ovarian malignant tumors. We report here that a patient with primary ependymoma of the ovary, who was resistant to BEP therapy, achieved a complete response to a combined therapy, including TIP therapy as the second-line chemotherapy and surgery. This important case, combined with published studies, suggests that TIP therapy is effective for both testicular and ovarian malignant tumors and indicates that TIP therapy can be used as an effective second-line therapy for malignant tumors resistant to BEP therapy.

Fibroma of the omentum resembling an ovarian tumor in the pelvis.

We would like to report the case of a patient with fibroma of the omentum that resembled an ovarian tumor in the pelvis. Since primary tumours of the omentum are rare, there is a paucity of information about the biology of such tumors in the basic texts and literature. An ultrasound examination of the patient revealed a mass, likely of ovarian origin, which consisted of liquid and solid components. It was suspected to be a malignant ovarian tumor. However, laparotomy demonstrated it was an omental tumor. This case shows that it can be difficult to pre-operatively diagnose omental fibromas because of their close resemblance to ovarian tumors.

Expression of ovary-specific acidic protein in steroidogenic tissues: a possible role in steroidogenesis.

Ovary-specific acidic protein (OSAP) is a novel molecule discovered from a genomic project designed to identify ovary-selective genes in mice. Whereas public databases suggest extraovarian expression of OSAP, its tissue distribution has not yet been well documented. Thus, the expression profile of mouse and human OSAP was determined by quantitative real-time RT-PCR using RNAs isolated from various tissues. The results demonstrate that the human and mouse OSAP expression profiles are similar; OSAP is prominently expressed in steroidogenic tissues with the highest level of expression observed in the adrenal gland. Placenta served as an exception and possessed minimal level of OSAP mRNA. Immunohistochemical studies show that mouse OSAP localizes almost exclusively to the steroid-producing cells of the ovary, adrenal gland, and testis. Consistent with predictions made by several subcellular localization algorithms, dual labeling studies in Y-1 mouse adrenocortical cells indicate OSAP resides in the mitochondria. Because of its abundant expression in steroidogenic cells and mitochondrial localization, a role for OSAP in steroidogenesis was determined. OSAP silencing by specific small interfering RNAs significantly inhibits 8-bromoadenosine-cAMP-induced progesterone production in Y-1 cells. Reduction in OSAP levels results in mitochondrial fragmentation and a decrease in the cellular content of mitochondrial DNA, indicative of decreased mitochondrial abundance. Lastly, 8-bromoadenosine-cAMP does not regulate OSAP protein expression in Y-1 cells as is the case for other steroidogenic components known to be induced by cAMP. Collectively these results suggest that OSAP is involved in steroidogenesis, potentially through its ability to maintain mitochondrial abundance and morphology.

Occipital scalp hemangioma: prenatal sonographic and magnetic resonance images.

Fetal scalp hemangioma in the occipital region is extremely rare and its accurate diagnosis is essential for perinatal management. We present a case of occipital scalp hemangioma diagnosed by prenatal sonography and magnetic resonance imaging (MRI). An echogenic mass measuring 29 mm x 23 mm x 30 mm was found in the occipital region by sonography at 20 weeks of gestation. Color-flow Doppler sonography depicted vascularity only at the periphery of the mass. The MRI results indicated that the extracranial mass exhibited a heterogeneous appearance of predominantly hyperintensity on T1- and hypointensity on T2-weighted images without evidence of fat signals, suggesting a soft tissue lesion containing blood products. Based on sonographic and MRI findings, the fetus was diagnosed to have occipital scalp hemangioma. Postmortem examination revealed cavernous hemangioma with hemorrhage. The MRI was a valuable adjunct to sonography for prenatal evaluation of an occipital lesion.

Differential zonal expression and adrenocorticotropin regulation of secreted protein acidic and rich in cysteine (SPARC), a matricellular protein, in the midgestation human fetal adrenal gland: implications for adrenal development.

CONTEXT: Matricellular proteins are a group of secreted, multifunctional extracellular matrix glycoproteins that includes thrombospondins (TSPs), tenascin-C, and secreted protein acidic and rich in cysteine (SPARC). They may be implicated in the dynamic developmental processes of the human fetal adrenal (HFA) in which the outer, definitive zone (DZ) cells are postulated to proliferate, migrate centripetally, differentiate, and populate the inner, steroidogenic fetal zone (FZ). OBJECTIVE: The objective of the study was to identify a matricellular molecule that likely plays a major role in HFA development. DESIGN: Studies involved RNA, cryosections, and cell cultures from 14- to 23-wk HFAs and human adult adrenal RNA. MAIN OUTCOME MEASURES: Measures included transcripts encoding matricellular proteins, using real-time quantitative RT-PCR; SPARC localization by immunostaining; and ACTH regulation of SPARC expression and secretion by quantitative RT-PCR and Western blot. RESULTS: SPARC HFA mRNA was 100-, 700-, and 300-fold higher than TSP-1, TSP-2, and tenascin-C mRNA, respectively. HFA SPARC mRNA was 3-fold higher than adult adrenals (P < 0.005), comparable with levels in adult brain (positive control), whereas mRNAs encoding TSP-1 and TSP-2 were lower in fetal than adult adrenals. SPARC immunoreactivity was detected exclusively in the FZ, not DZ. ACTH, a key regulator of HFA growth and function, increased SPARC mRNA (by 1.7-fold at 1 nm, 48 h, P < 0.05) in isolated FZ cells but not DZ cells. ACTH up-regulation of SPARC protein was also detected in FZ cell lysates and culture medium. CONCLUSIONS: Results suggest a possible role for SPARC in development of functional and/or structural zonation of the HFA.

Prenatal diagnosis of hemifacial microsomia by magnetic resonance imaging.

We present a case of hemifacial microsomia, first detected by prenatal sonography and confirmed by ultrafast magnetic resonance (MR) imaging. A 26-year-old patient was referred to our hospital at 20 weeks of gestation because of unilateral right-sided ventriculomegaly and of a possible ventriculoseptal defect (VSD). Our sonographic examination suggested a right orbital hypoplasia and a hemiatrophy of the nose. The ultrafast T2-weighted single-shot fast-spin echo MR imaging, demonstrated a right ear hypoplasia (microtia), a right orbital hypoplasia, and a right renal hypoplasia. Epibulbar dermoid or conjunctival lipodermoid were not recognized. The fetus was prenatally diagnosed as hemifacial microsomia. Termination of pregnancy was performed at 21 weeks gestation, and a male baby weighing 342g was stillborn. The baby showed facial asymmetry, including hemiatrophy of the right nose, and right ear hypoplasia. Autopsy revealed a 2mm area of VSD, agenesis of the right kidney and ureter, pancreatic and renal aberration into right adrenal gland, thymus hypoplasia and an unfixed ascending colon without intestinal malrotation. To the best of our knowledge, this is the first report of prenatal diagnosis for hemifacial microsomia using fetal MR imaging. In our case, fetal MR imaging has evolved into a powerful diagnostic tool, for the accurate prenatal diagnosis.

Pulmonary hypoplasia: prediction with use of ratio of MR imaging-measured fetal lung volume to US-estimated fetal body weight.

PURPOSE: To determine the ratio of fetal lung volume (FLV) to fetal body weight (FBW) by using ultrasonography (US) and magnetic resonance (MR) imaging and to evaluate the usefulness of this ratio in predicting pulmonary hypoplasia (PH) in fetuses at high risk. MATERIALS AND METHODS: MR imaging lung volumetry and US biometry were performed in 90 fetuses at 25-39 weeks gestation. In the control group of 73 fetuses, normal lung development was confirmed at neonatal follow-up and the normative ratio of MR imaging-measured FLV to US-estimated FBW (FLV/FBW) was determined. The high-risk group included 17 fetuses at risk for PH. The FLV/FBW was compared between the control and high-risk groups and with US parameters for predicting the development of PH in the high-risk group. Measurements 2 or more standard deviations below the mean control group measurement were considered abnormal. Comparisons of the FLV/FBW between groups were made by using the Student t test. The association between development of PH and measurement of each parameter was analyzed by using the Fisher exact probability test. RESULTS: In the control group, the FLV/FBW decreased with gestational age during the third trimester and had a normal distribution (mean ratio, 0.028 mL/g; range, 0.015-0.444 mL/g). The mean FLV/FBW for the nine fetuses with PH (0.012 mL/g +/- 0.008) was significantly lower (P <.001) than that for the control group (0.028 mL/g +/- 0.007). Fetuses with abnormal FLV/FBW values were at significantly greater risk (P <.05) for PH development. Abnormal FLV/FBW values had higher diagnostic accuracy than abnormal US parameters. Sensitivity of the FLV/FBW was 89% (eight of nine fetuses); specificity, 88% (seven of eight fetuses); positive predictive value, 89% (eight of nine fetuses); negative predictive value, 88% (seven of eight fetuses); and accuracy, 88% (15 of 17 fetuses). CONCLUSION: The FLV/FBW reflects the adequacy of intrauterine lung growth and can help predict PH.

Hypertensive disorders in Japanese women with gestational glucose intolerance.

We investigated the relationship between gestational glucose intolerance and the development of pregnancy-induced hypertension including gestational hypertension (GH) and preeclampsia. Consecutive Japanese women with singleton pregnancies underwent a standard 1h, 50g oral glucose challenge test (GCT) at 24-27 weeks of gestation, followed by a 75g, 2h oral glucose tolerance test (OGTT) if the GCT result exceeded 130mg/dl. Using criteria of the Japan Society of Obstetrics and Gynecology, gestational diabetes mellitus (GDM) was defined by two or more abnormal OGTT values and mild glucose intolerance by one abnormal value. The normal glucose tolerance group included women with GCT results below 130mg/dl or normal OGTT values. GH was defined as blood pressure of at least 140/90mmHg occurring for the first time after mid-pregnancy, without proteinuria. Preeclampsia was determined as GH with proteinuria. Of 2651 consecutive patients, 49 women were found to have GDM, and 139 showed mild glucose intolerance. Sixty patients showed GH, and 58 developed preeclampsia. The frequency of GH in mild glucose intolerance or GDM was 5.8% or 8.2%, respectively, significantly greater than in normal glucose tolerance (P<0.01). Incidence of preeclampsia was not significantly increased in women with mild glucose intolerance or GDM (2.2% or 4.1%, respectively, compared to those with normal glucose tolerance). Japanese women with gestational glucose intolerance therefore have an increased risk of developing GH.

Successful one-stage radical removal of intravenous leiomyomatosis extending to the right ventricle.

BACKGROUND: Intravenous leiomyomatosis is a rare tumor originating from the uterus that spreads through vessels. This tumor, while histologically benign, can cause fatal cardiovascular symptoms resulting from growth within the heart cavity. CASE: A 51-year-old woman with chronic hypertension was found to have a murmur. Echocardiogram, magnetic resonance imaging, and clinical evaluation revealed an intravenous leiomyomatosis extending to the right ventricle. The appropriate therapy for intravenous leiomyomatosis is complete resection. In this patient, one-stage cardiotomy with laparotomy resulted in successful radical removal of the tumor. CONCLUSION: Our patient underwent successful radical therapy for an intravenous leiomyomatosis extending to the heart, which involved complete resection of the tumor by one-stage cardiotomy with laparotomy.