The Prognosis and Predictive Value of Estrogen Negative/Progesterone Positive (ER-/PR+) Phenotype: Experience of 1159 Primary Breast Cancer from a Single Institute.

Breast cancer is a serious worldwide public health problem and is currently the most common cancer overall. Its endocrine therapy is related to the expression of the steroid hormones, estrogen receptor (ER), and progesterone receptor (PR). Breast cancers can be presented under multiple profiles of steroid hormones: ER(-)/PR(+), ER(+)/PR(-), double-positive/negative ER, and PR. 2-8% of all breast cancers express only PR (ER-/PR+) which is an abnormal phenotype, with less known about their behaviors and outcomes. Our study was performed on a large and well-characterized database of primary breast cancer from 2012 to 2019, up to 1159 cases. These cases were divided according to ER and PR expression, as we put all of our focus on ER-negative/PR-positive group, more specifically ER-/PR+/HER2+ and ER-/PR+/HER2- gene expressions, to highlight their features and find a pattern that links HR (hormone receptors) profiles and breast cancer subtypes. Out of the informative cases, 94 patients (8%) had ER-/PR+ breast cancers, while 676 (58.4%) had ER+/PR+, 88 (7.6%) had ER+/PR-, and 164 (14.2%) had ER-/PR- tumors. The ER-/PR+ group was statistically correlated with a high risk of recurrence and death in midway between the double-negative and double-positive HR. According to HER2 status, a low DFS was observed in patients ER-/PR+/HER2-, which is closer to the DFS of TNBC cases but worse than ER+/PR any. On the other side, the ER-/PR+/HER2+ showed also a poorer DFS closer to the HER2+ subgroup in between TNBC and ER+/PR any. The clinicopathological features of the ER-/PR+/HER2- and ER-/PR+ HER2+ have distinguished the patients into two groups with a difference in some clinicopathological characteristics: both groups had closer OS estimation, which was worse than ER-/PR any and better than TNBC and HER2. The ER-/PR+/HER2- seems to increase the risk of recurrence than ER-/PR+/HER2+ when compared to ER+/PR any. On the other hand, the ER-/PR+/HER2+ seems to increase the risk of death more than ER-/PR+/HER2- in comparison with ER+/PR any. Our results support that ER-/PR+ tumors really exist and are rare and clinically and biologically distinct subtypes of breast cancer. In addition, our analysis, which was based on dividing the groups according to HER2 expression, has revealed the existence of two distinct groups; this gave the ER-/PR+ subgroup a heterogeneity characterization. Moreover, this breast cancer subtype should not be treated as a luminal tumor but rather according to the HER2 expression status.

Mapping Cell Membrane Organization and Dynamics Using Soft Nanoimprint Lithography.

Membrane shape is a key feature of many cellular processes, including cell differentiation, division, migration, and trafficking. The development of nanostructured surfaces allowing for the in situ manipulation of membranes in living cells is crucial to understand these processes, but this requires complicated and limited-access technologies. Here, we investigate the self-organization of cellular membranes by using a customizable and benchtop method allowing one to engineer 1D SiO2 nanopillar arrays of defined sizes and shapes on high-performance glass compatible with advanced microscopies. As a result of this original combination, we provide a mapping of the morphology-induced modulation of the cell membrane mechanics, dynamics and steady-state organization of key protein complexes implicated in cellular trafficking and signal transduction.

Cancer du sein triple négatif chez la femme enceinte : le pronostic est-il si mauvais dans le contexte africain ?

Le cancer du sein triple négatif est un groupe de carcinome du sein caractérisé par le manque d'expression des récepteurs hormonaux aux oestrogènes et à la progestérone (RE, RP) et HER2. Cette forme se caractérise également par son agressivité, un faible taux de survie et l'absence de thérapies ciblées. Dans cemanuscrit, nous en rapportons deux cas de survenant chez des femmes enceintes ayant bénéficié d'une chirurgie basée sur des mastectomies plus curage axillaire suivie d'une chimiothérapie et radiothérapie adjuvantes. Les deux évolutions ont été marquées par la survenue de métastases cérébrales. À travers ces cas, nous décrivons en mettant un accent sur les caractéristiquesclinique et le mauvais pronostic du cancer du sein triple négatif pendant la grossesse. Nous soumettons également une brève revue de la littérature sur cette situation pendant la grossesse

Intrapartum group B Streptococcus screening in the labor ward by Xpert® GBS real-time PCR.

Group B Streptococcus (GBS) is the leading cause of neonatal infections in industrialized countries. Intrapartum antibiotic prophylaxis (IAP) given to colonized parturients is a key step for the prevention of neonatal early-onset infection. We compared the performances of Xpert® GBS polymerase chain reaction (PCR) (Cepheid, Sunnyvale, CA, USA) as a point-of-care system in labor wards to standard culture for intrapartum GBS detection. Pregnant women with a GBS-positive antenatal screening were prospectively included. A vaginal double swab was collected at the time of delivery for point-of-care Xpert® GBS PCR and GBS culture. A total of 565 pregnant women were included. Valid Xpert® GBS results were obtained for 488 (86.4%) women on the first attempt. Repeat testing improved the PCR success to 516 (91.3%) women. Among the 305 women positive for GBS by culture at delivery, only 238 (78.0%) were positive by Xpert® GBS PCR, cycle thresholds being correlated to culture quantification. Among 260 women negative for GBS culture, 56 (21.5%) were positive by Xpert® GBS PCR, including 50 where IAP was initiated before vaginal sampling. Overall, among the 565 women with GBS antenatal positive culture, only 335 (59.3%) were still positive at delivery whatever the technique used, resulting in unnecessary IAP for 40% of them. This large cohort study comparing intrapartum to antepartum GBS detection provides evidence that (i) Xpert® GBS PCR might be a valuable solution for intrapartum GBS detection compared to culture-based strategies and (ii) laboratory training of non-specialized staff is mandatory to reach the performances required for point-of-care tests.

[Spontaneous pneumomediastinum: A rare complication of dermatomyositis]. / Pneumomédiastin spontané : une complication rare de la dermatomyosite.

Dermatomyositis is a rare connective tissue disease of unknown origin, including inflammatory myopathy and cutaneous manifestations. Several pulmonary complications associated to dermatomyositis were described; especially interstitial lung disease. Some rare and particular pulmonary complications were reported in the literature such as pneumodiastinum and pneumothorax. We are describing here, a case report about a female patient, who presented with dermatomyositis associated to pneumomediastinum as a severe and lethal complication without pneumothorax. It is a novel observation depicting this severe and rare complication. Brutal dyspnea and cervical subcutaneous crackling are alarming signs that should make practitioners think about this complication.

Diabetic kidney disease and vascular comorbidities in patients with type 2 diabetes mellitus in a developing country.

Type 2 diabetes mellitus (T2DM) is associated with an increased risk of progression toward end-stage renal disease and cardiovascular (CV) risk mortality. To investigate this association, we studied 637 patients with T2DM in the Eastern area of Morocco. The mean follow-up period was 42 ± 11 months. At the baseline visit, 22.8%, 59.1% and 18.1% of the patients had normo-albuminuria, micro-albuminuria and macro-albuminuria, respectively. Of all patients, 51.6% had a history of hypertension and 37.4% had hypertension on admission. At the end of follow-up, rapid progression (estimated glomerular filtration rate >5 mL/min/1.73 m² /year) was observed in 24.1% of the cases and the frequency of occurrence of CV events was 5%, 18.5% and 32.5% in the normo-, micro- and macro-albuminuria groups, respectively (P <0.001). In multivariate analysis, arterial hypertension was identified as an independent risk factor related to diabetic kidney disease (DKD, P = 0.04) and occurrence of CV events (P = 0.02), while albuminuria was not identified as an independent risk factor, either for DKD or for the occurrence of CV events. Our study found that hypertension was an independent risk factor for the DKD and the occurrence of the CV events in T2DM patients.

Giant pelvic retroperitoneal epidermoid cyst: a rare case report.

Epidermoid cyst is a frequent benign cutaneous tumor. The pelvic localization does not occur very often. The literature that taps into such cases is very limited in scope. Here is a report of a 27-year-old woman with a giant pelvic retroperitoneal epidermoid cyst. The use of ultrasound exploration and computed tomography has indicated ovarian origins. The surgery also revealed a retroperitoneal epidermoid cyst, uterus and ovaries were all intact. The evacuation of a cyst was found to contain lamellas of keratin. Histology permitted us to confirm the diagnosis. The patient was faring well after two years of followup.

Endometrial stromal nodule: report of a case.

Endometrial stromal nodule (ESN) is the least common of the endometrial stromal tumors. They are rare neoplasms which are diagnosed in most instances by light microscopy. Although such nodules are benign, hysterectomy has been considered the treatment of choice to determine the margins of the tumor required for diagnosis and to differentiate it from invasive stromal sarcoma Whose prognosis is totally different. We report a case of a 45 years old woman, with presurgical diagnosis of adnexal mass or uterine tumor. She underwent a total abdominal hysterectomy. Pathologic examination revealed an endometrial stromal nodule. Through this observation, we insist on the fact that the ESNs are rare and benign entities which must be differentiated from the other invasive malignant stromal tumors; this can change the final prognosis.

Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France.

BACKGROUND: Anakinra treatment has been reported to be effective in some patients with systemic-onset juvenile idiopathic arthritis (SoJIA) or adult-onset Still disease (AoSD). OBJECTIVES: To assess the efficacy and the safety of anakinra treatment in SoJIA and AoSD. METHODS: SoJIA and AoSD patients were treated with anakinra (1-2 mg/kg/day in children, 100 mg/day in adults); we analysed its effect on fever, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, numbers of swollen and tender joints, the assessment of disease activity (by physician and parent/patient) and pain (by parent/patient), and American College of Rheumatology (ACR) pediatric core set criteria for JIA activity. RESULTS: A total of 35 patients were included, 20 with SoJIA and 15 with AoSD. Their mean age (range) at the onset of treatment was 12.4 (3-23) and 38.1 (22-62) years, respectively; disease duration was 7.0 (1-16) and 7.8 (2-27) years, respectively. Active arthritis was present in all cases but one. Of the 20 SoJIA patients, 5 achieved ACR 50% improvement in symptoms (ACR50) response criteria at 6 months. Steroid dose had been decreased by 15% to 78% in 10 cases. A total of 11 of the 15 AoSD patients achieved at least a 50% improvement for all disease markers (mean follow-up: 17.5 (11-27) months). Steroids had been stopped in two cases and the dose was decreased by 45% to 95% in 12 patients. Two patients stopped anakinra due to severe skin reaction, and two patients due to infection: one visceral leishmaniasis and one varicella. CONCLUSION: Anakinra was effective in most AoSD patients, but less than half SoJIA patients achieved a marked and sustained improvement.

[Vogt-Koyanagi-Harada syndrome. Eight cases]. / Syndrome de Vogt-Koyanagi-Harada. A propos de huit cas.

PURPOSE: Vogt-Koyanagi-Harada disease is a rare systemic affection characterised by the association of ocular inflammatory manifestations and extra ocular lesions such as meningismus, tegumentary or auditory findings observed in pigmented population. Auto-immune origin of this syndrome is probable; a T-lymphocyte-mediated autoimmune process is directed against an unidentified antigen associated with melanocytes. METHODS: The aim of this retrospective study is to determine their clinical profile in our country. RESULTS: Eight consecutive cases of Vogt-Koyanagi-Harada disease were studied during a 22-year period. All the patients fulfilled the criteria of American Uveitis Society. All patients were female and had bilateral ocular involvement as panuveitis. Retinal serous detachment was observed in 4 patients, meningitis in 6 cases. 7 patients had hearing loss and 5 patients had cutaneous lesions represented by poliosis. Corticosteroids and/or immunosuppressive therapy were administered in all patients leading to improvement in 5 patients. CONCLUSION: Our series are concording with a usually good ocular prognosis. Immunosuppressive therapy should be used early in posterior segment involvement.

[Treatment of childhood cancer in Africa. Action of the Franco-African childhood cancer group]. / Le traitement des cancers de l'enfant en Afrique. Travaux du groupe franco-africain d'oncologie pédiatrique.

The childhood cancer survival rate is currently 75% in industrialized countries. Rates in developing countries are much lower. The Franco-African Childhood Cancer Group (French acronym, GFAOP) was founded in 2000 with aim of reducing this unfavorable situation in Africa. The GFAOP has developed two forms of action. The main form consists of organizing two- to twelve-month training sessions for physicians and nurses in France and Morocco. The other form involves assessing the feasibility of modern treatment protocols for various cancers in Africa. The first feasibility trials were carried out on nephroblastoma and Burkitt's lymphoma in 12 pilot units in North Africa, West Africa, and Madagascar. In the first study from 2001 to 2005 we treated 306 cases of Burkitt's lymphoma using French LMB protocols adapted to the African setting and achieved a survival rate of 61%. A second study started in 2005 using Endoxan alone achieved a highly satisfactory survival rate of 73% for neuroblastoma in all stages except bilateral. Altogether from 2001 to 2007 more than 1000 cases of nephroblastoma and Burkitt's lymphoma were treated in African hospitals by African doctors and nurses. No patients were transferred to Europe. The GFAOP supplied drugs when necessary and took care of most travel expenses. African and French doctors worked together on protocol design, trial management, and data analysis. These promising results show that the latest therapeutic techniques can be used to treat childhood cancer in Africa by adapting the protocol to conditions in developing countries. Sanofi-Aventis Laboratories in association with the International Union against Cancer has launched a major campaign to improve Pediatric Oncology in developing countries. Projects in four GFAOP units are being financed through this campaign. In 2006 the GFAOP began assessment of two new treatment protocols, i.e., one for acute lymphoblastic leukemia and the other for Hodgkin's disease. Two other projects are being planned, i.e., one for treatment of retinoblastoma and the other for treatment of some types of brain tumors.

[Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]. / Manifestations neurologiques de la maladie de Behçet.

INTRODUCTION: Neurological involvement is well described in Behçet's Disease (BD), with variable prevalence of 5.3 to 30p.cent. The purpose of this retrospective study was to analyze the clinical patterns of neuro-Behçet (NB) and to compare them with different clinical features of 925 BD registered in the same period and the literature. METHODS: All patients of NB fulfilled the International Study Group Criteria for the diagnosis of BD. 93 patients with headache, without other neurological symptoms were excluded. The findings were supported by cerebrospinal fluid, computed tomography scan, magnetic resonance imaging and angiography. RESULTS: NB was present in 16.64p.cent of BD. A sex ratio male/female: 4.31. The mean age of patients with NB was 31.76 years. The average age of onset of NB was 29.83. The mean duration of the NB disease was 3.81 years. The findings were categorised in 2 main types: parenchymal and non-parenchymal involvement. Some patients had features of both types (mixed patterns). 94 patients (61.03p.cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, pseudobulbar syndrome and cerebellar signs. 27 patients (17.53p.cent) without parenchymal CNS which were divided into: intracranial hypertension in 24 patients (15.58p.cent) presenting headache, vomiting and bilateral papilloedema; cerebro-arterial involvement in 3 cases (one of them had cerebrovascular aneurysms). Mixed patterns were observed in 9 cases. Erythema nodosum and vascular involvement were more frequent in intracranial hypertension than in BD. Other clinical features were reported: pure meningeal pattern in 15 cases, pure peripheral nervous system involvement in 4 cases, isolated cranial nerves in 8 cases, chorea in one patient and pseudotumor of cervical medulla in other patient. 6 cases of juvenile BD and one case of familial Behçet were observed. 133 patients were treated (86.36p.cent). Mean duration of treatment was 7.46 month. We used corticosteroids, immunosuppressive agents (cyclophosphamid, azathioprine, chlorambucil) and anticoagulant in intracranial hypertension. The course of disease was good in only 54.13p.cent of cases, and was bad in 18.79p.cent. CONCLUSION: NB occurred frequently in men and is more serious especially in parenchymal CNS involvement: 7 deaths (6 from parenchymal CNS).

[Thymic hyperplasia following treatment for nephroblastoma]. / Hypertrophie thymique après une chimiothérapie pour un néphroblastome.

UNLABELLED: Thymic hyperplasia in response to stress is a well known phenomenon. Thymic hyperplasia has also been described after chemotherapeutic treatment for malignancies in children. CASE REPORT: A three-year-old girl was followed up from the age of 18 months for a left kidney nephroblastoma treated by combination of chemotherapy (vincristin, actinomycin and adriamycin) and surgery. Assessment at the end of treatment was normal. Four months after the end of treatment, pulmonary radiography showed mediastinal enlargement, which was shown to originate in the thymus at thoracic CT scan. A recurrence of the disease was suspected. Biopsy showed thymic hyperplasia without evidence of tumor cells. Mediastinal enlargement then disappeared spontaneously 2 months later. CONCLUSION: Thymic hyperplasia occurring during remission of a cancer treated by chemotherapy is a diagnostic dilemma as it suggests mediastinal reccurence of the disease. Needle aspiration cytology is an appropriate investigation in thymic hyperplasia. No steroid therapy should be used before histologic diagnosis of thymic hyperplasia.

[Langerhans-cell histiocytosis of the orbit. A case study]. / Histiocytose langerhansienne à localisation orbitaire. A propos d'un cas.

PURPOSE: Langerhans' cell histiocytosis is a rare disease representing less than 1% of orbital tumors. METHODS: We report a case of Langerhans cell histiocytosis with orbital involvement in a 9-year-old boy. He presented with an inflammatory swelling if the left lateral orbital wall. The computed tomographic scan revealed an orbital cellular mass with lytic bone lesion within the orbital roof and intracranial enlargement. RESULTS: The cytological study after a biopsy showed infiltrates of histiocytes derived from Langerhans cells. Diagnosis was confirmed by immunohistochemistry, which identified positive staining with anti-S100 and anti-CD1a antibodies. The rapidly expanding orbital tumor, posing a threat of ocular compression as well as intracranial spreading, was treated by chemotherapy (Vinblastine) combined with a steroid. CONCLUSION: A 2-year follow-up showed no evidence of recurrence or systemic involvement. According to this observation, the authors describe the clinicopathological and histological features of orbital involvement in Langerhans cell histiocytosis.