Liposarcoma of the extremities: MR imaging features and their correlation with pathologic data

SummaryObjectiveTo describe the MRI features of liposarcomas of the extremities and correlate them with data from the histologic subtypes.Material and methodsRetrospective study of 20 cases of liposarcoma of the extremities identified on MRI, surgically removed and confirmed at pathology. On MRI examination, T1- and T2-weighted fat-suppressed and non-fat-suppressed images were acquired then gadolinium-enhanced fat-suppressed T1-weighted images were obtained in at least two orthogonal planes.ResultsSixteen female and four male patients aged 12 to 77 years old at presentation, in 16 cases, with a slowly enlarging painless mass (demonstrating no associated local or general inflammatory components), located in the lower extremity (16 cases) and predominantly located in the thigh (13 cases). Pathologic examination revealed three cases of well-differentiated liposarcoma, 12 cases of myxoid liposarcoma one of which with round cells, three cases of pleomorphic liposarcoma, one case of dedifferentiated liposarcoma and one case of mixed-type liposarcoma. MR images mostly showed well-circumscribed tumors (19 cases) . Well-differentiated liposarcomas typically demonstrated a very specific diagnostic appearance as a predominantly adipose mass containing nonlipomatous components seen as thick septa that may show nodularity. Other subtypes of liposarcoma demonstrated a small amount of adipose tissue thus producing a marbled textural pattern on T1-weighted images particularly in myxoid liposarcomas (9 cases), or even nonlipomatous elements in high-grade liposarcomas (in round cell liposarcoma and in two out of three pleomorphic liposarcomas). The myxoid subtype has also a relatively characteristic appearance as a low signal intensity noted on T1-weighted images and a marked high signal intensity on T2. The dedifferentiated liposarcoma has a very specific radiologic appearance as a nonlipomatous component within a predominantly adipose mass, simulating that of well-differentiated liposarcomas.ConclusionMRI of extremity liposarcomas is a highly reliable and sensitive method to characterize liposarcomas. Besides its value in the diagnosis of liposarcoma and locoregional extension control, it allows proper identification of the specific histologic subtypes of liposarcoma.Level of evidenceLevel IV. Rétrospective diagnostic study

Intrauterine pregnancy following low-dose gonadotropin ovulation induction and direct intraperitoneal insemination for severe cervical stenosis

BACKGROUND: We present a case of primary infertility related to extreme cervical stenosis, a subset of cervical factor infertility which accounts for approximately 5% of all clinical infertility referrals. CASE PRESENTATION: A 37 year-old nulligravida was successfully treated with ovulation induction via recombinant follicle stimulating hormone (FSH) and direct intraperitoneal insemination (IPI). Anticipating controlled ovarian hyperstimulation with in vitro fertilization/embryo transfer (IVF), the patient underwent hysteroscopy and cervical recanalization, but safe intrauterine access was not possible due to severe proximal cervical stricture. Hysterosalpingogram established bilateral tubal patency and confirmed an irregular cervical contour. Since the cervical canal could not be traversed, neither standard intrauterine insemination nor transcervical embryo transfer could be offered. Prepared spermatozoa were therefore placed intraperitoneally at both tubal fimbria under real-time transvaginal sonographic guidance using a 17 gage single-lumen IVF needle. Supplementary progesterone was administered as 200 mg/d lozenge (troche) plus 200 mg/d rectal suppository, maintained from the day following IPI to the 8(th )gestational week. A singleton intrauterine pregnancy was achieved after the second ovulation induction attempt. CONCLUSIONS: In this report, we outline the relevance of cervical factor infertility to reproductive medicine practice. Additionally, our andrology evaluation, ovulation induction approach, spermatozoa preparation, and insemination technique in such cases are described

Microsolvation of NO + in Ar n clusters: A theoretical treatment

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Structure and stability of Na+Xen clusters

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A simple method for extracting the parameters of the PV cell single-diode model

This paper aims to simplify the parameters' extraction for the solar cell single diode model and to show that there are many solutions. The proposed algorithm considers many pairs of ideality factor and series resistance. For each pair, it finds the other three corresponding parameters using any three arbitrary points of the I-V curve by simply solving 3 ? 3 linear system of equations. Each set of parameters is used to simulate an I-V curve. The deviation of simulated curve from the experimental one is assessed by calculating the root mean square error (RMSE). Finally, the best set of five parameters is selected according to its smallest RMSE value. A large set of combinations of the five parameters that yield quasi-identical I-V curves was obtained. This is well in line with previous studies reporting wide variations in some parameters including unrealistic values. The proposed method was validated with real experimental data, which showed excellent fits to I-V curves measured under various operating conditions. Compared to other methods, the proposed algorithm uses a single I-V curve and does not make any assumptions on the parameters. Moreover, the calculations do not use any slopes and do not depend on specific points of the curve.Scopu

Theoretical investigations of the electronic states of NaXe: A comparative study

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From Mild to Wild Fluctuations in Crystal Plasticity

International audienceMacroscopic crystal plasticity is classically viewed as an outcome of uncorrelated dislocation motions producing Gaussian fluctuations. An apparently conflicting picture emerged in recent years emphasizing highly correlated dislocation dynamics characterized by power-law distributed fluctuations. We use acoustic emission measurements in crystals with different symmetries to show that intermittent and continuous visions of plastic flow are not incompatible. We demonstrate the existence of crossover regimes where strongly intermittent events coexist with a Gaussian quasiequilibrium background and propose a simple theoretical framework compatible with these observations

Diagnostic moléculaire de la maladie de Gaucher en Tunisie

Trabalho desenvolvido na sequência de projeto de colaboração Portugal-Tunisia iniciado no IBMC-UP.[ENG] Gaucher disease is a lysosomal storage disorder caused by a deficiency of the enzyme acid b-glucosidase. In order to determine the mutation spectrum in Tunisia, we performed recurrent mutation screening in 30 Tunisian patients with Gaucher disease. Screening of recurrent mutation by PCR/RFLP and direct sequencing had shown that N370S was the most frequent mutation (22/50 mutant alleles, 44%), followed by L444P mutation, which is found in 16% (8/50 mutant alleles). The recombinant allele (RecNciI)represented 14%. Our findings revealed that the genotype N370S/RecNciI was mosst frequent in patients with childhood onset and it was associated with severe visceral involvement. The screening of these three mutations provided a simple tool for molecular diagnosis of Gaucher disease in Tunisian patients and allowed also genetic counselling for their family members.[FR] Résumé: La maladie de Gaucher est une maladie de surcharge lysosomale due à un déficit de l’enzyme β-glucosidase. Afin d’étudier le spectre mutationnel de cette affection en Tunisie, nous avons recherché les mutations récurrentes chez 30 patients. Le dépistage des mutations récurrentes par PCR/RFLP et séquençage direct a révélé que la mutation N370S est la plus fréquente (44 %, 22/50 allèles mutés), suivie par la mutation L444P qui présente une fréquence de l’ordre de 16 % (8/50 allèles mutés). L’allèle recombinant (RecNciI) a été retrouvé chez 14 % des cas étudiés. Les mutations non identifiées dans cette étude représentent 26 %. En outre, nos résultats ont montré que le génotype N370S/RecNciI est le plus fréquent dans les formes à révélation pédiatrique et il est associé à une atteinte viscérale sévère. La recherche de ces mutations en priorité fournit un outil de diagnostic moléculaire pour la majorité des patients, elle permet ainsi un dépistage des hétérozygotes indispensable pour le conseil génétique. Abstract: Gaucher disease is a lysosomal storage disorder caused by a deficiency of the enzyme acid β-glucosidase. In order to determine the mutation spectrum in Tunisia, we performed recurrent mutation screening in 30 Tunisian patients with Gaucher disease. Screening of recurrent mutation by PCR/RFLP and direct sequencing had shown that N370S was the most frequent mutation (22/50 mutant alleles, 44%), followed by L444P mutation, which is found in 16% (8/50 mutant alleles). The recombinant allele (RecNciI) represented 14%. Our findings revealed that the genotype N370S/RecNciI was mosst frequent in patients with childhood onset and it was associated with severe visceral involvement. The screening of these three mutations provided a simple tool for molecular diagnosis of Gaucher disease in Tunisian patients and allowed also genetic counselling for their family members

Spectre mutationnel de la maladie de gaucher en Tunisie: forte fréquence des hétérozygotes composites N37OS/REC Ncil

European Neighbourhood Policy covering the Mediterranean Partner Countries[Article in French]Gaucher disease is the most common lysosomal storage disorder, it results from the inherited deficiency of the enzyme glucocerebrosidase, the accumulation of its substrate causes many clinical manifestations. Since the discovery of GBA gene, more than 200 different mutations have been identified, but only handful mutations are recurrent (N370S, L444P and c.84insG). In order to determine the mutation spectrum in Tunisia, we performed recurrent mutation screening in ten unrelated Tunisian children with Gaucher disease. Screening of recurrent mutation by PCR/RFLP and direct sequencing, has shown that N370S is the most frequent mutation (6/20 mutant alleles, 30%), followed by recombinant allele (RecNciI) which is found in five patients (5/20 mutant alleles, 25%), the L444P mutation represent 20% (4/20 mutant alleles). Our findings revealed that five among ten studied patients, were compound heterozygous N370S/RecNciI (50%). The screening of these mutations provides a simple tool for molecular diagnosis of Gaucher disease in Tunisian patients and allows also genetic counselling for their family members.EU-supported R&D programm