Accessory cavitated uterine mass: MRI features and surgical correlations of a rare but under-recognised entity.

OBJECTIVES: To describe MRI features of accessory cavitated uterine mass (ACUM) with surgical correlations. METHODS: Eleven young women with an ACUM at pathology underwent preoperative pelvic MRI. Two experienced radiologists retrospectively analysed MR images in consensus to determine the lesion location within the uterus, its size, morphology (shape and boundaries), and structure reporting the signal and enhancement of its different parts compared to myometrium. The presence of an associated urogenital malformation or other gynaecological anomaly was reported. MRI features were correlated with surgical findings. RESULTS: All 11 lesions were well correlated with surgical findings, lateralised (seven were left-sided), and located under the horn and the round ligament insertion. Nine were located within the external myometrium, bulging into the broad ligament. Two were extrauterine, entirely located within the broad ligament. On MRI, the mean size was 28 mm (range 17-60 mm). Nine lesions were round-shaped, two were oval; all had regular boundaries. At surgery, the ACUM were not encapsulated but were possible to enucleate. On MRI, all lesions were well defined and showed a central haemorrhagic cavity surrounded by a regular ring (mean thickness, 5 mm) which had the same signal compared to the junctional zone. ACUM was isolated in all women, without urogenital malformation, adenomyosis or deep endometriosis. CONCLUSIONS: On MRI, ACUM was an isolated round accessory cavitated functional non-communicating horn-like aspect in an otherwise normal uterus. MRI may facilitate timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. KEY POINTS: • ACUM is rare, with delayed diagnosis in young women with severe dysmenorrhoea. Pelvic MRI facilitates timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. • Quasi-systematically located under the uterine round ligament insertion, ACUM may be intramyometrial and/or in the broad ligament. • On MRI ACUM resemble a non-communicating functional accessory horn within a normal uterus; the mass, most often round-shaped, had a central haemorrhagic cavity surrounded by a regular ring which had the same low signal compared to the uterine junctional zone.

Risk of gestational trophoblastic neoplasia after hCG normalisation according to hydatidiform mole type.

OBJECTIVE: The risk of gestational trophoblastic neoplasia (GTN) after a hydatidiform mole (HM) is well known. However, the risk of GTN after normalisation of hCG in HM is poorly reported. The aim of this study was to evaluate the risk of GTN after normalisation of hCG according to HM types. METHODS: This prospective cohort study carried out between 2000 and 2010 used the database of the French Trophoblastic Disease Centre (FTDC). A total of 2008 registered patients with ascertained types of HM were analysed. Cases of GTN occurring after normalisation of hCG were analysed. RESULTS: A GTN developed in 239 out of 1980 HMs (12.1%) and 6 out of these 239 post-molar GTN (2.5%) were diagnosed after normalisation of hCG. The risk of GTN after normalisation of hCG was 0.34% (6/1747) following a HM, 0% (0/593) after a partial HM (PHM), 0.36% (4/1122) after a complete HM (CHM), and 9.5% (2/21) after a multiple pregnancy with HM. CONCLUSIONS: The risk of post-molar GTN justifies hCG monitoring in all women with HM. However, after normalisation of hCG, monitoring of PHM can be stopped safely while it should be maintained for CHM and more importantly for multiple pregnancies with HM.

Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome.

Although Mayer-Rokitansky-Küster-Hauser syndrome is a rare condition with a reported incidence of 1/4500 female live births, it represents the second most common cause of primary amenorrhea and has psychologically devastating consequences. The radiologist plays a pivotal role in both making the accurate initial diagnosis of this condition and assessing findings that may contribute to treatment planning. The purpose of this article is to provide an overview of the capabilities of ultrasound and magnetic resonance imaging (MRI) for the diagnosis and management of this syndrome with emphasis on the relevant clinical and surgical findings and to describe potential associated abnormalities and differential diagnosis.

Features of pigmented vulval lesions on dermoscopy.

BACKGROUND: The dermoscopic criteria for benign and malignant lesions on the vulva are not well established due to the lack of large series of such lesions. Melanoma should always be included in the differential diagnosis of pigmented lesions on the vulva especially when they are wide, or of recent onset. Elsewhere on the skin dermoscopy plays an important role in the selection of suspicious pigmented lesions, as well as in the selection of the best site to perform the biopsy. OBJECTIVES: To analyse the dermoscopic patterns observed in pigmented lesions of the vulva. METHODS: We analysed a nonselected consecutive series of 68 histopathologically proven cases comprising five melanomas, 16 naevi, 20 lentigos, 12 benign vulval melanoses, 11 cases of postinflammatory pigmentation, three pigmented cases of usual vulval intraepithelial neoplasia (VIN) and one seborrhoeic keratosis seen at our institution. The dermoscope was covered by translucent disposable food wrap and/or antibacterial gel to prevent possible transmission of infections. Descriptive statistics were performed using multiple correspondence analysis. RESULTS: The parallel (37%), ring-like (9%), homogeneous (22%), globular-like (13%) and reticular-like (6%) patterns were observed on benign lesions (naevi, lentigo, vulval melanosis and postinflammatory pigmentation). The cerebriform pattern (6%) was observed only on VIN and seborrhoeic keratosis. The multicomponent pattern (6%) was associated with melanoma (60%). In cases of melanoma we also occasionally observed an irregular pattern, a whitish or blue-whitish veil, irregularly distributed dots and globules and atypical vascular pattern. Using multiple correspondence analysis, we designed a new algorithm for the early detection of vulval melanomas. CONCLUSIONS: Dermoscopy can play a role in the noninvasive classification of vulval melanosis. However, further studies of larger collaborative series are needed to validate our vulval melanoma diagnostic algorithm. VIN and seborrhoeic keratosis share the same dermoscopic features and biopsy should be considered for seborrhoeic-like keratosis. In case of doubt pathological examination of a biopsy remains mandatory.

Predictive values of hCG clearance for risk of methotrexate resistance in low-risk gestational trophoblastic neoplasias.

BACKGROUND: Early identification of patients at high risk for chemoresistance among those treated with methotrexate (MTX) for low-risk gestational trophoblastic neoplasia (GTN) is needed. We modeled human chorionic gonadotropin (hCG) decline during MTX therapy using a kinetic population approach to calculate individual hCG clearance (CL(hCG)) and assessed the predictive value of CL(hCG) for MTX resistance. PATIENTS AND METHODS: A total of 154 patients with low-risk GTN treated with 8-day MTX regimen were retrospectively studied. NONMEM was used to model hCG decrease equations between day 0 and day 40 of chemotherapy. Receiver operating characteristic curve analysis defined the best CL(hCG) threshold. Univariate/multivariate survival analyses determined the predictive value of CL(hCG) and compared it with published predictive factors. RESULTS: A monoexponential equation best modeled hCG decrease: hCG(t) = 3900 x e(-0.149 x t). Median CL(hCG) was 0.57 l/day (quartiles: 0.37-0.74). Only choriocarcinoma pathology [yes versus no: hazard ratio (HR) = 6.01; 95% confidence interval (CI) 2.2-16.6; P < 0.001] and unfavorable CL(hCG) quartile (< or =0.37 versus >0.37 l/day: HR = 6.75; 95% CI 2.7-16.8; P < 0.001) were significant independent predictive factors of MTX resistance risk. CONCLUSION: In the second largest cohort of low-risk GTN patients reported to date, choriocarcinoma pathology and CL(hCG) < or =0.37 l/day were major independent predictive factors for MTX resistance risk.

[Sciatic paralysis following uterine artery embolization]. / Paralysie sciatique transitoire après embolisation de l'artère utérine.

Early postpartum bleeding remains in France the leading cause of maternal mortality in perinatal period. In association with obstetrical and medical measures to control bleeding, uterine arteries embolization constitutes an efficient non-surgical measure whose potential side effects must be kept in mind. We report the case of a patient that presented a popliteal sciatic paralysis in the hours following the procedure. Through this case, we will review the different types of embolization complications.

[Vulvoperineal reconstruction after extended radical vulvectomy: two reconstructive procedures]. / Reconstruction vulvopérinéale après vulvectomie radicale élargie: à propos de deux techniques de lambeau.

Poor perineal healing is often a major complication of extended radical vulvectomy in case of vulvar carcinoma. Procedures of vulvoperineal reconstruction require several criteria of quality for their use. The chosen technique should be: (1) reliable; (2) reproducible; (3) with minimal morbidity; (4) not much invasive with good anatomical and functional results. We describe two procedures of perineal reconstruction that correspond to the previous criteria: a local fasciocutaneous flap with lateral transposition and a regional musculocutaneous flap using the gluteus maximus muscle.

[Utility of fetal ultrasonography in the prenatal diagnosis of Smith-Lemli-Opitz syndrome]. / Apport de l'échographie foetale dans le diagnostic prénatal du syndrome de Smith-Lemli-Opitz.

Smith-Lemli-Opitz syndrome is a rare autosomal recessive genetic disorder which diagnosis is usually made postnatally. We describe a case of a prenatal diagnosis based only on specific ultrasound findings: intra-uterine growth retardation with facial dysmorphia, polydactyly and genital anomalies. We suggest giving more consideration to the ultrasound scanning for the diagnosis of the syndrome in the prenatal period.

[Vaginal hysterectomy using electrofusion: a study of 96 cases]. / Hystérectomie vaginale par électrofusion: étude sur 96 cas.

OBJECTIVE: To compare ligature by electrofusion versus sutures in the practice of vaginal hysterectomy. STUDY DESIGN: This is a retrospective study on 96 patients completed over a period of 47 months. Patients were allocated into two groups: the electrofusion "suture-free" group (n=54) and the "suture" control group (n=42). Designed-end points were operating time, postoperative pain, duration of postoperative hospitalization and perioperative complications. RESULTS: In the electrofusion group, the operating time was significantly reduced (51.3+/-22.6 min versus 67.6+/-20.1 min) as well as the reported postoperative pain (based on the visual analog scale - VAS) (1.9+/-2.0 versus 3.5+/-2.3). The average morphine consumption rate and the timing of postoperative morphine administration were lower in the electrofusion group (22.4+/-31.0mg versus 45.4+/-51.3 mg and 22.4+/-13.4 h versus 29.4+/-18.8 h, respectively). Moreover, in the electrofusion group there was less need for additional analgesics (1.9+/-2.0 versus 3.5+/-2.3) and the hospital-stay was shorter (4.2+/-1.3 days versus 5.0+/-1.0 days). There was no significant difference between the two groups in regard to perioperative complications. CONCLUSION: The use of electrofusion in vaginal hysterectomy appears to be a reliable ligation technique which reduces significantly the operating time, the postoperative pain and the length of postoperative hospitalization.

[Feto-maternal metabolism in human normal pregnancies: study of 73 cases]. / Métabolisme foeto-maternel au cours de grossesses humaines normales: étude de 73 cas.

From 73 normal pregnancies of gestational age between 17 and 41 weeks of gestation (WG), the concentrations of glucose, pyruvate and lactate, free fatty acids, ketone bodies (aceto-acetate and beta-hydroxybutyrate) and cholesterol were assessed on maternal venous blood (MVB) and umbilical venous blood (UVB), sampled by cordocentesis. The objective of this work was to study feto-maternal metabolism, as well as nutritional exchange between maternal blood and fetal blood during the second and third trimesters of pregnancy. Maternal and fetal glycemias, as well as maternal-fetal glucose concentration gradient, were found stable during the studied gestational period; maternal glucose is always higher than fetal glucose, with a mean concentration delta of 0.69+/-0.34 mmol/L. Maternal lactate level (1.26+/-0.38 mmol/L) is lower than fetal lactate level (1.48+/-0.46 mmol/L), whereas maternal blood pyruvate concentration (0.042+/-0.020 mmol/L) is higher than fetal blood pyruvate concentration (0.025+/-0.010 mmol/L). Consequently, mean lactate / pyruvate ratio is found twice lower in maternal blood (31.77+/-9.89) than in fetal blood (64.10+/-17.12). Free fatty acids concentration is approximately three times higher in maternal blood than in fetal blood (respectively 0.435+/-0.247 mmol/L and 0.125+/-0.046 mmol/L). Maternal venous aceto-acetate (0.051+/-0.042 mmol/L) and beta-hydroxybutyrate (0.232+/-0.270 mmol/L) concentrations are significantly lower than those in UVB (respectively 0.111+/-0.058 and 0.324+/-0.246 mmol/L) and the beta-hydroxybutyrate/aceto-acetate ratio is on average 1.7 times higher in MVB (4.75+/-2.5) than in UVB (2.82+/-1.18). Cholesterol concentration is significantly higher in maternal blood (6.26+/-1.40 mmol/L) than in fetal blood (1.66+/-0.34 mmol/L). Our results show the characteristics of oxidative metabolism of the fetus compared with that of the adult. Blood concentration in energy substrates, measured with glucose and free fatty acids levels, is low in UVB and suggests increased energy needs of the growing fetus. Mean high concentrations in aceto-acetate and beta-hydroxybutyrate in UVB, indicate probably fetal ketogenesis. UVB low cholesterolemia suggests high cholesterol consumption in the fetal compartment for cellular membrane synthesis and steroid biosynthesis.

[Evaluation of treatment relating to gestational trophoblastic tumor registered to the French Trophoblastic Disease Reference Center (TDRC) in Lyon from 1999 to 2005]. / Evaluation de la prise en charge des tumeurs trophoblastiques gestationnelles enregistrées au Centre de référence des maladies trophoblastiques de Lyon de 1999 à 2005.

OBJECTIVES: The aim of this study was both to analyse if gestational trophoblastic neoplasia (GTN) registered to the French Trophoblastic Disease Reference Center (TDRC) in Lyon (France) were managed according to the FIGO criteria for diagnosis of GTN and if chemotherapy was adapted to the 2000 FIGO prognostic scoring system. PATIENTS AND METHODS: Retrospective, descriptive analysis of 167 GTN registered to GTC of Lyon between 1999 and 2005. RESULTS: On the one hand, 66% of women (104/158) had a diagnosis of GTN according to FIGO criteria. One third (n=54) of the patients therefore had a premature or erroneous diagnosis of a tumor, when the treatment started. No supporting element of this premature diagnosis has been found out for 26 patients. The identification of lung and vaginal metastasis and histological diagnosis of invasive mole appeared as the most mentioned inappropriate criteria for diagnosis. On the other hand, chemotherapy was adapted to 2000 FIGO scoring in 91, 5% of cases. Twelve low risk GTN were treated with polychemotherapy and two high risk GTN were treated with monochemotherapy. Moreover 29% of the patients received a non adequate treatment due to deviations from the recommended protocol. DISCUSSION AND CONCLUSION: Non respect of FIGO criteria for the diagnosis of GTN can lead to erroneous diagnosis of tumors. Identification of lung or vaginal metastasis or diagnosis of invasive mole should not automatically justify the diagnosis of gestational trophoblastic neoplasia if the decrease in HCG occurs properly. Respect of FIGO criteria for the diagnosis of GTN and adaptation of chemotherapy to 2000 FIGO scoring are necessary to avoid inadequate treatment of gestational trophoblastic neoplasia.

[Lactational breast abscesses: Do we still need surgery?]. / Abcès du sein lactant : et si on ne les opérait plus ?

AIM: To show the effectiveness of ultrasound-guided puncture in the treatment of lactational breast abscess and identify its risk factors. MATERIALS AND METHODS: Retrospective descriptive study at the CHU of Lyon-Sud from December 2007 to December 2013, including patients with lactational breast abscess confirmed on ultrasound and treated with antibiotics and analgesics. Realisation of ultrasound-guided needle under local anesthesia by the radiologist and washing the cavity with physiological serum. RESULTS: Forty patients had lactational abscesses at an average of 10 weeks post-partum. Thirty-four patients were treated by needle aspiration, of which 2 had first surgical drainage. The average size of the abscess was 41.2mm. The success rate of needle aspiration was 91.2%. No cases of recurrence were observed, however, there were 5 fistulisations. In all, 91.2% were treated on an outpatient basis. In 87.8% of cases, breastfeeding was continued on the healthy side and in 48.5% of cases on the affected side. The major risk factor for abscess was mastitis in 91.1% of cases. CONCLUSION: Ultrasound guidance of needle aspiration should be gold standard for the treatment of lactational breast abscesses to continue breastfeeding including the affected side.

Laparoscopic hysterectomy after concurrent radiochemotherapy in locally advanced cervical cancer compared to laparotomy: A multi institutional prospective pilot study of cost, surgical outcome and quality of life.

OBJECTIVE: Laparoscopy allows hysterectomies after chemoradiation to be performed without opening the abdominal wall. We measured the costs and quality of life for locally advanced cervical cancer patients operated on via laparoscopy compared to laparotomy. STUDY DESIGN: We conducted an observational prospective multicenter study on locally advanced cervical cancer patients undergoing an extrafascial hysterectomy after concurrent chemoradiotherapy (CRT). We assessed the costs from the medical visit before surgery up to the first month after surgery from the providers' perspective and measured the quality of life using the EORTC QLQ-C30 and QLQ-CX24 up to six months. RESULTS: Sixty two patients (39 laparoscopy and 23 laparotomy) from December 2008 to November 2011 were included. There was no difference in operative time, or intraoperative and post-operative complication rates between the two groups. Intraoperative transfusion and abdominal drain were significantly lower in the laparoscopy group (respectively, p = 0.04 and p < 0.01), as well as the duration of hospital stay (7.3 d vs. 5.7 d, p < 0.001). All patients who underwent laparoscopic hysterectomy were discharged to home, whereas 4 laparotomy patients used convalescence homes (p = 0.01). Mean costs at one month were €10,991 for laparotomy and €11,267 for laparoscopy (p = 0.76). Sexual activity is better for the laparoscopy group at six months (p = 0.01). CONCLUSION: Laparoscopy for an extrafascial hysterectomy after CRT in locally advanced cervical cancer patients brought better quality of life with similar costs compared to laparotomy, and should therefore be the first choice for surgeons.

Induction of transplantation tolerance in humans using fetal cell transplants.

When engrafted with donor stem cells and lymphoid cells, patients develop transplantation tolerance to donor antigens. We analyzed the mechanism of tolerance induction in immunoincompetent recipients whose immunity has been reconstituted by transplantation of mismatched stem cells. Seven infants or human fetuses received fetal liver transplants as a treatment for severe combined immunodeficiency disease. After reconstitution of immunity by lymphocytes developed from donor stem cells, T-cell clones were produced and analyzed. Because donors and recipients were HLA mismatched, it was easy to demonstrate the donor origin of the T-cell clones. These clones were shown to have developed tolerance to histocompatibility antigens of the stem cell donor via a process of clonal deletion (probably as a result of contact with donor-derived macrophages and dendritic cells). They were also tolerant to histocompatibility antigens of the host but through a different mechanism: many clones recognized these antigens but had no detrimental effect on the target cells exhibiting host antigens, either in vitro or in vivo. Clonal anergy was therefore the cause of this tolerance to host determinants, resulting in a lack of graft-versus-host disease and of autoimmunity. The contact between developing T cells of donor origin and host epithelial cells within the host thymus may explain this colonal anergy. It should be noted that all patients had high serum levels of interleukin-10, which might have contributed to the persistent engraftment and tolerance.

Comparison of HIV detection by virus isolation in lymphocyte cultures and molecular amplification of HIV DNA and RNA by PCR in offspring of seropositive mothers.

An early and accurate diagnosis of HIV infection is needed in the offspring of seropositive mothers. To this end, we have used two techniques for the direct detection of HIV in 12 newborns tested within 2 weeks after birth and 12 children. HIV isolation was carried out in lymphocyte cocultures and compared with detection of DNA and RNA sequences by molecular amplification using the polymerase chain reaction (PCR). In lymphocyte cocultures, HIV was isolated in 8 of 24 cases (33%), including 3 newborns, 3 symptomatic children, and 2 asymptomatic ones. HIV DNA was detected by PCR in twice as many cases, i.e., in 16/24 cases (66%), including 7/12 newborns, 4/4 symptomatic children, and 5/8 asymptomatic ones, 2 of whom became seronegative, HIV RNA was detected in 10 of 16 cases (60%) with detectable HIV DNA, including all of the cases who had a positive HIV isolation. Only children with clinical or biological signs of HIV infection were positive for HIV RNA. Furthermore, signs of HIV infection appeared within 6 months in three of the four newborns who were positive for HIV RNA at birth. These results indicate that HIV DNA detection by PCR is far more sensitive than HIV isolation in culture for the early diagnosis of HIV infection in offspring of seropositive mothers. HIV RNA detection appears to be a useful prognostic marker since it does correlate with disease progression and may serve as a clue for HIV replication in vivo.

Structure and composition of microcalcifications in benign and malignant lesions of the breast: study by light microscopy, transmission and scanning electron microscopy, microprobe analysis, and X-ray diffraction.

Microcalcifications previously located by radiography were extracted from 25 fresh specimens obtained from patients who had undergone tumorectomy or systematized mammary exeresis. Two principal types of microcalcifications were distinguished: Type I microcalcifications were amber in color and generally crystalline on scanning electron microscopy, with only one calcium peak on microprobe analysis; x-ray diffraction revealed that weddellite was involved. Type II microcalcifications were whitish, nonbirefringent under polarized light, and generally ovoid or fusiform, with two peaks, one calcium and the other phosphorus, on microprobe analysis; these microcalcifications were composed of calcium phosphate, the most characteristic form of which is hydroxyapatite, in the form of needles arranged in rosettes on transmission electron microscopy. Type I microcalcifications were observed in four of eight benign breast lesions, in two of three in situ lobular carcinomas, and in no intraductal adenocarcinomas or infiltrating carcinomas. Type II microcalcifications were present in all infiltrating carcinomas and intraductal adenocarcinomas; they were also found in benign lesions (four of eight) and even associated with type I microcalcifications in one in situ lobular carcinoma. There are, therefore, no "benign" or "malignant" microcalcifications; however, the presence of weddellite is a strong indication that a lesion is benign or, at most, an in situ lobular carcinoma.

In utero transplantation of stem cells in humans: technical aspects and clinical experience during pregnancy.

Four fetal patients have received fetal liver cell transplants in utero, at the fertilization ages of 12-28 weeks. Depending on the age, intraperitoneal injection or intravenous infusion into the umbilical vein was used, under ultrasonic guidance. In three of the four cases, engraftment has been obtained and has resulted in cure or significant improvement of the inherited disease.

Fetal liver transplantation: biology and clinical results.

Over the last 18 years, we have developed the transplantation of fetal liver cells to treat severe immunodeficiencies, hematological disorders and inborn errors of metabolism. Post-natally, this treatment is successful in two-third of patients and it is therefore very valuable, especially when there is no perfectly matched donor for a bone marrow transplant. Since 1988 we have carried out these fetal liver transplants (FLTs) in utero, immediately after prenatal diagnosis. Engraftment and reconstitution have been obtained, and several advantages appear to be associated with in utero FLT: increased probability of graft take, ideal isolation of the patient (in the maternal uterus) and optimal environment for the differentiation of the transplanted fetal liver cells (in the fetal host).

New developments in stem cell transplantation with special reference to the first in utero transplants in humans.

Based on the experience acquired in post-natal liver transplantation since 1974, we recently initiated pre-natal, in utero stem cell transplantation from the human fetal liver. The first two fetuses that we treated had immunodeficiencies, the third one had thalassemia major. Donors and recipients were not matched. The fetal cells were infused in the umbilical vein of the first two patients and injected intraperitoneally into the third one, under ultrasonic visualization. The first patient, born in 1988, has both engraftment of donor cells and reconstitution of cell-mediated immunity. This child, who had bare lymphocyte syndrome, has no clinical manifestation of the disease and he lives normally at home. The second child, born in 1989, has not yet developed a significant reconstitution of immunity although donor cell engraftment has been proven (Y chromosome in this female patient). The third patient has also evidence of donor cell take (Y chromosome in a female patient) but the effect on thalassemia has not yet been fully analyzed (donor hemoglobin present in small quantity). In all 3 cases, no side-effect of any kind developed in the mother nor in the fetus. Several advantages appear to be associated with in utero FLT: increased probability of graft take, ideal isolation of patient (in the uterus), optimal environment for fetal cell development (in the fetal host).