Incidental finding of Zygomyceteslike hyphae in the placenta. A case report

Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism. We present 5 cases with PMDS (2 cases associated with testicular malignancy) and discuss the diagnosis and management. Management strategies of PMDS have changed. Whereas in the past, removal of the müllerian remnants was targeted together with orchidopexy or -ectomy, this is no longer recommended. However, testicles that cannot be descended at an early stage are at a high risk of malignancy and should, therefore, be removed. If this is necessary on both sides, there is the additional problem of lifelong testosterone substitution which requires efficient patient monitoring and good patient compliance. In cases where this cannot be achieved, compromises, such as temporarily delayed orchidectomy, may be considered.     

Sexually dimorphic development of the mammalian reproductive tract requires Wnt-7a

An important feature of mammalian development is the generation of sexually dimorphic reproductive tracts from the Müllerian and Wolffian ducts. In females, Müllerian ducts develop into the oviduct, uterus, cervix and upper vagina, whereas Wolffian ducts regress. In males, testosterone promotes differentiation of Wolffian ducts into the epididymis, vas deferens and seminal vesicle. The Sertoli cells of the testes produce Müllerian-inhibiting substance, which stimulates Müllerian duct regression in males. The receptor for Müllerian-inhibiting substance is expressed by mesenchymal cells underlying the Müllerian duct that are thought to mediate regression of the duct. Mutations that inactivate either Müllerian-inhibiting substance or its receptor allow development of the female reproductive tract in males. These pseudohermaphrodites are frequently infertile because sperm passage is blocked by the presence of the female reproductive system. Here we show that male mice lacking the signalling molecule Wnt-7a fail to undergo regression of the Müllerian duct as a result of the absence of the receptor for Müllerian-inhibiting substance. Wnt7a-deficient females are infertile because of abnormal development of the oviduct and uterus, both of which are Müllerian duct derivatives. Therefore, we propose that signalling by Wnt-7a allows sexually dimorphic development of the Müllerian ducts.     

Immunophenotypic analysis of ovarian and testicular Müllerian papillary serous tumors

Intratesticular Müllerian papillary serous tumors lacking stromal invasion are uncommon neoplasms whose immunophenotypic properties have not been studied extensively. We present such information here and compare it with information from a group of ovarian papillary serous tumors of low malignant potential ("borderline serous tumors") that are morphologically identical. We compared the histologic features of our index case of intratesticular Müllerian papillary serous tumor with those of nine ovarian papillary serous tumors. We then evaluated both the index case and the ovarian tumors with antibodies against carcinoembryonic antigen, LeuM1, CA125, estrogen receptors, progesterone receptors, cytokeratin 7, and cytokeratin 20, by use of established immunohistochemical techniques. The testicular and ovarian tumors were morphologically indistinguishable. The intratesticular Müllerian papillary serous tumor expressed LeuM1, CA125, estrogen receptors, progesterone receptors, cytokeratin 7, and weak cytokeratin 20; carcinoembryonic antigen was not expressed. All of the ovarian papillary serous tumors expressed CA125, estrogen receptors, and cytokeratin 7. Eight of nine expressed progesterone receptors. Five of nine stained with LeuM1. Two of nine were focally weakly positive with cytokeratin 20. LeuM1 expression helps distinguish testicular papillary serous tumors from mesothelial proliferations, which might seem morphologically similar. The immunophenotype of intratesticular and female genital papillary serous tumors is similar; this similarity extends to expression of estrogen and progesterone receptors, which is rare in neoplasms in men, especially among testicular neoplasms.     

Are the wolffian anomalies in males the phenotype corresponding to the müllerian anomalies in females?

The Wolffian and Müllerian ducts are embryologically closely related. This survey was aimed to determine whether, as has been suggested, Wolffian duct anomalies in males are the phenotype corresponding to Müllerian anomalies in females. This was done by analysis of the phenotype of the males in associations and syndromes in which Müllerian duct anomalies are frequent, and of the phenotype of the females in families in which anomalies of the Wolffian ducts are found. It appears that the only relation between the 2 types of defects seems to be a developmental one. In cases of early insult, anomalies are seen in males and females (e.g., in the association of renal agenesis with Müllerian or Wolffian defects). On the other hand, in all the other associations or syndromes, no relation was observed. This suggests that Wolffian anomalies in males are not the phenotype corresponding to Müllerian anomalies in females.     

Programmed cell death in the Müllerian duct induced by Müllerian inhibiting substance

The importance of a secretory product of the fetal testis, Müllerian Inhibiting Substance, in determining the fate of the Müllerian duct in the developing male fetus has been well documented. The present investigation has examined the mechanism of action of Müllerian Inhibiting Substance in the male rat fetus during the course of Müllerian duct degeneration. The action of Müllerian Inhibiting Substance mimics the general morphogenetic phenomenon of "programmed cell death," although important diferences were found compared to the majority of other studies of cell death. The initial morphological event in the degenerating Müllerian duct is an increase in lysosomes within the duct cells. Following loss of polarity and orientation, the duct cells are subsequently removed by invading macrophages from the surrounding mesenchyme. Concommitant with the differentiation of these macrophages, the adjacent mesenchyme assumes a characteristic whorled pattern around the degenerating Müllerian duct. Cytochemical localization of acid phosphatase confirmed the presence of newly formed lysosomes within the Müllerian duct cells and subsequently within the cytoplasm of the invading macrophages.     

Serum levels of müllerian inhibiting substance in preterm and term male neonates

PURPOSE: Müllerian inhibiting substance, also called anti-müllerian hormone, is responsible in the embryo for the regression of the müllerian structures. During the second and third trimesters the physiological functions that müllerian inhibiting substance may have after the period of müllerian duct regression are poorly understood. We obtained information on müllerian inhibiting substance levels in the male newborn during this period of gestation. MATERIALS AND METHODS: Müllerian inhibiting substance was measured by an enzyme immunoassay in cord blood obtained at birth in 27 preterm (25 to 36 weeks of gestation) and 92 term (37 to 42 weeks) male neonates. RESULTS: Cord serum müllerian inhibiting substance concentrations were relatively high from 25 to 31 weeks (mean plus or minus standard deviation 86.4 +/- 36.1 ng./ml.) and then they decreased from 32 weeks to term (mean 24.2 +/- 14.0 ng./ml.). CONCLUSIONS: The decline early in the third trimester may be consistent with müllerian inhibiting substance having a function during the second but a diminished role in the third trimester.     

Predator discrimination error and the benefits of Müllerian mimicry

Müllerian mimicry has traditionally been thought to benefit both unpalatable mimic and model species but recently its existence has been questioned. Even if both mimic and model species are unpalatable, they are unlikely to be equally unpalatable. It has been argued that the more unpalatable species will suffer a cost of increased predation because the presence of the more palatable mimic will increase its perceived palatability (by the predator), similar to that experienced by a model in Batesian mimicry. Yet, previous models of Müllerian mimicry have assumed that a predator can discriminate perfectly between available prey. We argue that this is not the case and that discrimination error is an important factor in determining the nature of mimetic relationships. Using computer simulations we show that the nature of a mimetic relationship will depend on the trade-off between the cost of an increase in perceived palatability and the benefits of a reduction in predator discrimination error. We show that mimicry can be unequivocally Müllerian, with both species benefiting, and propose that palatability should no longer be used as the sole defining characteristic of a mimetic relationship. Copyright 1998 The Association for the Study of Animal Behaviour. Copyright 1998 The Association for the Study of Animal Behaviour.     

Scrotoschisis associated with contralateral meconium periorchitis

Scrotoschisis, a congenital defect of the scrotal wall associated with extracorporeal testicular ectopy, has been previously reported only twice. Meconium periorchitis is another rare scrotal anomaly indicative of an antenatally healed gastrointestinal perforation. The authors present a third case of scrotoschisis and the first associated with meconium periorchitis. Several hours after birth of an otherwise-normal term baby boy, a scrotal exploration was performed with orchidopexy and primary closure of the scrotal wall defect. At 4 months of age the baby underwent a contralateral inguino-scrotal exploration with excision of a paratesticular mass of calcified meconium. The role of a normally developed scrotum in testicular descent and causes of calcified scrotal masses in infants are discussed.     

Detection of congenital müllerian duct anomalies using three-dimensional ultrasound

PURPOSE: The purpose of this study was to assess the value of 3-dimensional sonography in the diagnosis of congenital müllerian duct anomalies, which cause infertility, preterm labor, and first trimester abortion. METHODS: A prospective study was undertaken in which 40 patients with histories of repeated spontaneous abortions or infertility were first examined using conventional 2-dimensional sonography or hysterosalpingography. Three-dimensional transvaginal sonography was then performed. RESULTS: Twenty-eight women had müllerian duct abnormalities, and 12 women had normal uterine anatomy. Müllerian duct defects detected in this study were unicornuate uterus (3), bicornuate uterus (3), complete or partial septate uterus (12), arcuate uterus (9), and didelphic uterus (1). The diagnosis of müllerian duct anomalies in these patients was confirmed by laparoscopic and/or hysteroscopic examinations. Three-dimensional sonography demonstrated all congenital uterine abnormalities with a sensitivity and specificity of 100%. Separate uterus and bicornuate uterus could be correctly diagnosed using 3-dimensional sonography in 11 (92%) of 12 cases and 3 (100%) of 3 cases, respectively. These 2 abnormalities were commonly confused with each other using hysterosalpingography and conventional sonography. CONCLUSIONS: Three-dimensional sonography with image reconstruction is less expensive and less invasive than hysterosalpingography for the assessment of uterine anatomy and diagnosis of müllerian duct abnormalities. The ability to visualize both the uterine cavity and the myometrium on a 3-dimensional scan facilitates the diagnosis of uterine anomalies and enables the differentiation of septate from bicornuate uteri for preoperative surgical planning.     

Genital anomalies in human and animal models reveal the mechanisms and hormones governing testicular descent

A systematic examination of the conditions characterized by the presence of genital anomalies in humans, noting in each condition the position of the gonad, the nature of the gubernaculum and cranial suspensory ligament can provide valuable information regarding the mechanisms controlling the final position of the gonads. In conditions where MIS is absent, the gubernaculum is "feminized', resulting in a testis in the position normally occupied by an ovary or an abnormally mobile testis that can prolapse to the inguinal region. In conditions of androgen insensitivity the testis is located in the inguinal region, indicating that the first phase of descent is normal but that inguinoscrotal descent has failed to occur. Ovarian descent fails to occur in congenital adrenal hyperplasia, despite exposure of the developing fetus to high levels of androgens, indicating that androgen alone does not control gonadal descent. Moreover, ovarian descent fails to occur despite androgen-dependent regression of the cranial suspensory ligament. The correlation between the degree of Müllerian duct retention and scrotal position in mixed gonadal dysgenesis further strengthens the hypothesis that the first stage of testicular descent is controlled by MIS. The study of genital anomalies suggests that MIS controls the swelling reaction in the male gubernaculum, which is responsible for the first phase of testicular descent to the inguinal region. The second or inguinoscrotal phase of descent is androgen-dependent. Regression of the cranial suspensory ligament is also androgen-dependent: however, it is the gubernaculum and not the presence or absence of the cranial suspensory ligament which controls testicular descent. A combined knowledge of the hormonal basis controlling sexual differentiation and the biphasic model of testicular descent enables the clinician to accurately predict the internal anatomy of these complex sexual anomalies.     

Exstrophy of the cloaca in a 47,XXX child: review of genitourinary malformations in triple-X patients

Cloacal exstrophy, unilateral renal agenesis, and Müllerian anomalies occurred in a liveborn infant with a 47,XXX chromosome constitution. The patient extends the range of genitourinary anomalies reported in triple-X patients. Screening asymptomatic patients for urinary tract abnormalities may be useful in searching for silent malformations potentially associated with this karyotype.     

Epithelial metaplastic changes in ovarian endometriosis

Little is known concerning epithelial metaplastic changes (metaplasia) in ovarian endometriosis. Three hundred fifteen consecutive cases of ovarian endometriosis between 1987 and 1995 were retrieved from hospital files and clinicopathologically analyzed. Two hundred fifty-seven cases were not associated with malignant ovarian epithelial tumor or atypia Four cases were atypical endometriosis. The remaining 54 cases were associated with malignant ovarian epithelial tumor, including borderline tumor. Metaplasias in ovarian endometriosis were observed in 162 (63%) cases not associated with malignant epithelial tumor or atypia. Ciliated cell and eosinophilic metaplasias were the most common (44%, respectively), followed by hobnail (13%) and mucinous (4%) types. Metaplasias in endometriosis were observed in all of the four atypical endometriosis cases and all of the 54 cases with malignant ovarian epithelial tumor. Among these cases, ciliated cell and eosinophilic metaplasia were also the most common. There was no correlation between types of carcinoma and types of metaplasia in endometriosis, but in all of the four Müllerian mucinous borderline tumors, ovarian endometriosis with mucinous metaplasia and hyperplasia was contiguous or intimately associated with the tumor. Metaplasia was often observed in ovarian endometriosis and most frequently was associated with malignant ovarian epithelial tumor or atypia. Metaplastic changes in ovarian endometriosis should not be interpreted as neoplastic features. Mucinous metaplasia and hyperplasia in ovarian endometriosis might be closely associated with the development of Müllerian mucinous borderline tumors.     

Diagnosing the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction

PURPOSE: We describe the differential points in the diagnosis of the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction. Various imaging studies and urological procedures were performed. We report our experience in detecting these anomalies in 10 girls and review the literature. MATERIALS AND METHODS: Ten girls, 7 to 13 years old, with this combination of anomalies were identified in the last 10 years. Imaging studies as well as urological procedures were selectively performed, especially at puberty following menarche. Patients received long-term followup with ultrasound. RESULTS: Cystic dilation of Gartner's duct protruded into the bladder and presented as a ureterocele in 5 patients and posterior to the bladder in 5. Surgical removal of a partial portion of a Gartner's duct cyst was performed in 5 patients for alleviation of urinary symptoms. Unilateral müllerian duct obstruction was demonstrated in all 10 patients. Excision of the vaginal septum was performed in 6 patients for relief of genital obstruction. CONCLUSIONS: When cystic dilatation of the pelvis, especially a ureterocele-like cyst without ureteral dilatation, is found in girls with ipsilateral renal dysgenesis, the possibility of a Gartner's duct cyst should be considered. For early detection and treatment of unilateral obstruction of duplicated müllerian ducts pelvic sonography should be performed at puberty, especially just after menarche, in girls with renal dysgenesis and ipsilateral Gartner's duct cyst.     

Fatty acids metabolism of rat liver after extra-lethal gamma-irradiation

OBJECTIVE: To determine if any preoperative or intraoperative factors can reliably predict malignancy in patients with Hürthle cell neoplasms. SUMMARY BACKGROUND DATA: Most experienced surgeons recommend total thyroidectomy for Hürthle cell carcinomas and reserve thyroid lobectomy for Hürthle cell adenomas. However, delineation between Hürthle cell adenoma versus carcinoma often cannot reliably be made either before or during surgery. METHODS: Medical records from 57 consecutive patients who underwent thyroid resections for Hürthle cell neoplasms between October 1984 and April 1995 at The Johns Hopkins Hospital were analyzed to determine if any factors were predictive of malignancy. RESULTS: Of the 57 patients with Hürthle cell neoplasms, 37 had adenomas and 20 had carcinomas, resulting in a 35% prevalence of malignancy. Patients with adenomas did not differ from those with carcinoma with respect to age, sex, or history of head and neck irradiation. However, patients with Hürthle cell carcinomas had significantly larger tumors (4.0 +/- 0.4 cm vs. 2.4 +/- 0.2 cm, p < 0.005). Furthermore, although the incidence of malignancy was only 17% for tumors 1 cm or less and 23% for tumors 1 to 4 cm, tumors 4 cm or greater were malignant 65% of the time (p < 0.05). Both fine-needle aspiration and intraoperative frozen section analysis had low sensitivities in the detection of cancer (16% and 23%, respectively). With up to 9 years of follow-up, there has been no tumor-related mortality. CONCLUSIONS: These data demonstrate that the size of a Hürthle cell neoplasm is predictive of malignancy. Therefore, at the time of initial exploration for large Hürthle cell neoplasms (>4 cm), definitive resection involving both thyroid lobes should be considered because of the higher probability of malignancy.     

Loss of inwardly rectifying potassium currents by human retinal glial cells in diseases of the eye

We compared the inward K+ currents of Müller glial cells from healthy and pathologically changed human retinas. To this purpose, the whole-cell voltage-clamp technique was performed on noncultured Müller cells acutely isolated from human retinas. Cells originated from retinas of four healthy organ donors and of 24 patients suffering from different vitreoretinal and chorioretinal diseases. Müller cells from organ donors displayed inward K+ currents in the whole-cell mode similar to those found in other species. In contrast, this pattern was clearly changed in the Müller cells from patient retinas. In whole-cell recordings many Müller cells had strongly decreased inward K+ current amplitudes or lost these currents completely. Thus, the mean input resistance of Müller cells from patients was significantly increased to 1,129 +/- 812 M omega, compared to 279 +/- 174 M omega in Müller cells from healthy organ donor retinas. Accordingly, since the membrane potential is mainly determined by the K+ inward conductance in healthy Müller cells, a large amount of Müller cells from patient retinas had a membrane potential which was significantly lower than that of Müller cells from control eyes. The mean membrane potentials were -37 +/- 24 mV and -63 +/- 25 mV for patient and donor Müller cells, respectively. The newly described membrane characteristic changes of Müller cells from patient eyes are assumed to interfere severely with normal retinal function: (1) the retinal K+ homeostasis, which is partly regulated by the Müller cell-mediated spatial buffering, should be disturbed, and (2) the diminished membrane potential should influence voltage-dependent transporter systems of the Müller cells, e.g., the Na(+)-dependent glutamate uptake.     

Mesna, doxorubicin, ifosfamide, and dacarbazine chemotherapy for ovarian mixed müllerian sarcoma: report of four cases

Four patients with metastatic ovarian mixed Müllerian sarcoma (2 homologous, 2 heterologous) were treated with mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) chemotherapy. Two of four patients had optimal debulking. Three of four patients responded to chemotherapy, with two complete responses of 34- and 46-month duration. The MAID regimen appears to be active in patients with ovarian sarcoma.     

Molecular cloning of an avian anti-Müllerian hormone homologue

Anti-Müllerian hormone (AMH) is responsible for regression of the Müllerian ducts in males during embryonic development. This peptide hormone of the transforming growth factor-beta family is also believed to play a broader role in sex determination, affecting differentiation and morphogenesis of the testes. Accordingly, in mammals, AMH is produced at much higher levels in male fetuses than in female fetuses. In contrast, in birds, both male and female embryonic gonads produce AMH at high levels, although in males it is still responsible for regression of the Müllerian ducts. Its persistent expression by the embryonic ovaries and its role in female sex determination in birds is not understood. We have cloned an avian homologue to AMH. Avian AMH cDNA encodes a 644 amino acid protein that is 42% identical to human AMH overall with increased identity at the carboxyl terminus. Similarities to human AMH include motifs of sequence identity, a conserved putative plasmin cleavage site and cysteine alignments, and similar genomic intron/exon structure. Antibodies to recombinant avian AMH cross-react with recombinant human AMH and were used to show that avian AMH is glycosylated as has been shown for the human form. The avian AMH gene is transcribed in both male and female gonads but not in liver, heart, kidney or muscle.     

Standard versus individually planned irradiation field in radiotherapy of infradiaphragmatic lymph node sites]

PURPOSE: The aim of this study was to determine the utility of scrotal sonography in the evaluation of patients with scrotal pain or swelling following inguinal hernia repair. METHODS: From our database, we identified patients who were referred for sonographic evaluation because of persistent scrotal pain or swelling after inguinal hernia repair between July 1994 and February 1996. Sonograms and medical charts were reviewed retrospectively. RESULTS: Eight patients were included in this study. Doppler sonography demonstrated evidence of testicular infarction in 2 patients and absence of intratesticular diastolic flow in 1 patient. Five patients had postoperative fluid collections with sonographically normal testes. CONCLUSIONS: Scrotal sonography can diagnose testicular infarction following hernia repair and distinguish postoperative fluid collections from recurrent hernias.     

Laparoscopic management of persistent Müllerian duct remnants associated with an abdominal testis

In recent years, laparoscopy has evolved from a purely diagnostic procedure in the management of nonpalpable testis to a definitive therapeutic intervention. Additional genital malformations occur in association with cryptorchidism, but reports of laparoscopic management of such entities do not exist. Herein, we describe the laparoscopic removal of persistent Müllerian duct remnants (uterus and round ligament) in combination with an orchiectomy of an abnormally small abdominal testis. This technique expands the versatility of laparoscopic management of cryptorchidism to include the resection of associated congenital anomalies.     

隐睾下降固定术后睾丸恶变临床分析

Objective: We summarized the relationship between the descent of a testicle into the scrotum and testicular cancer. Methods: Twenty-eight patients with testicular cancer after surgical treatment of cryptorchidism were retrospective analysis. Results:All patients were performed surgical treatment of cryptorchidism from 2 to 28 years old (median, 12 years;average, 16 years). Testicular cancer age ranged from 19 to 53 years (median, 33 years; average, 36 years). Malignant transformation occurred from 3 to 25 years of operation time (average, 18 years). Twenty-seven cases of malignant cryptorchidism ipsilateral, contralateral malignancy in 1 case, 27 cases were underwent radical resection of testicular cancer. Pathology diagnosis was mainly seminoma. Retroperitoneal lymph node dissection was done in 3 cases, 18 cases were chemotherapy and radiotherapy in 3 cases. Conclusion: The undescended testicle is the most common genital malformation in boys. When diagnosed, it should be treated as early as possible, but successful treatment appears not to lessen the risk of testicular cancer, patients must be closely monitored follow-up.