[Actinomycosis revealed by ulceration of the palate and gingiva]. / Actinomycose révélée par une ulcération du palais et de la gencive.

BACKGROUND: Actinomycosis is a chronic and extensive granulomatous, bacterial infection. Revelation by oral ulceration is rare. PATIENTS AND METHODS: A 76-year-old patient with diabetes was treated with dabrafenib for stage IV melanoma. A follow-up visit revealed two ulcerated, infiltrated and hyperalgesic lesions of the palate and gingiva. There were no associated signs. The laboratory findings were normal. The possibility of squamous cell carcinoma occurring with BRAF inhibitors was discussed, despite the rarity of such cases in the literature. Histological examination showed an actinomycotic grain. A scan of the facial mass showed no osteitis. Antimicrobial therapy was initiated with amoxicillin for four months, with a favorable outcome. DISCUSSION: Actinomycetes are Gram-positive filamentous saprophytic bacteria of the oral cavity and the gastrointestinal tract. They can become pathogenic under the influence of several factors. Cervicofacial involvement in the form of a peri-mandibular inflammatory nodule with secondary fistulation on the skin or in the mouth is the classic presentation. To our knowledge, no cases of opportunistic infection under BRAF inhibitors have been described. Only two cases of tuberculosis have been reported with sorafenib. The initial presentation led to suspicion of squamous cell carcinoma. In our patient, poor oral hygiene and diabetes were the two key factors considered. Moreover, this is the first case reported under dabrafenib, which does not appear to be a favoring factor. We would stress the importance of mucosal examination in patients treated with BRAF inhibitors.

Alterations in bone mineral density and bone turnover markers in newly diagnosed adults with lymphoma receiving chemotherapy: a 1-year prospective pilot study.

BACKGROUND: Bone mineral density (BMD) loss is poorly defined in lymphoma patients. The aim of this study was to measure the extent of BMD loss in newly diagnosed lymphoma patients receiving chemotherapy. PATIENTS AND METHODS: This was a prospective, single-center study conducted in patients aged≥18 years with previously confirmed lymphoma treated by chemotherapy. Patients with low baseline BMD defined as Z/T-score less than or equal to -2.5 and/or history of osteoporotic fractures were excluded. BMD was measured at baseline before initiating chemotherapy and 1 year later. Predictive factors of BMD loss were investigated. RESULTS: Forty-one lymphoma patients (31 males and 10 females) receiving chemotherapy were enrolled. The median age at diagnosis was 59 (range: 19-86) years. Histological subtypes were predominantly diffuse large B-cell lymphoma (58%), mostly stage III-IV (54%). All patients received chemotherapy and 22% of patients received second-line treatment due to relapse or progressive disease. Thirty-two patients were evaluable at 1 year. The mean BMD changes were: -2.7%±3.9% for lumbar spine (P<0.001), -2.2%±7.6% for femoral neck (P<0.01) and -2.6%±4.5% for total hip (P<0.0001). In multivariate analysis, predictive factors of BMD loss at baseline were (i) at lumbar spine: female gender (P=0.01), higher lactate dehydrogenase level (P=0.04) and lower creatinine clearance (P=0.01); (ii) at total hip: lower albumin (P=0.01), higher corrected serum calcium (P<0.01), lower alkaline phosphatase (AP) (P<0.01) and autologous stem cell transplant (P=0.03); and (iii) at femoral neck: higher corrected serum calcium (P=0.02) and lower bone AP (P=0.01). CONCLUSION: Adult patients with known lymphoma receiving chemotherapy experienced significant BMD loss at 1 year.

[Bladder tumor targeting by Hexvix(®) fluorescence: 4 years results after prospective monocentric evaluation]. / Ciblage diagnostique par Hexvix(®) des tumeurs urothéliales de vessie : résultats après quatre ans d'évaluation prospective monocentrique.

PURPOSE: Prospective evaluation of bladder tumor targeting by Hexvix(®) fluorescence. PATIENTS AND METHODS: From September 2008 to April 2012, 107 patients have been evaluated using Hexvix(®) technique (blue light) for typically papillary bladder tumor or suspected bladder tumor. Clinical and pathological data have been collected prospectively and patients have been classified using EORTC score. RESULTS: From the 107 patients, 67 have been identified with bladder cancer and 328 samples have shown positive fluorescence in blue light. Compared to white light, 13 additional tumors have been diagnosed by Hexvix(®) for 11 patients: Cis (n=6), LMP (n=3), pTa low grade (n=3), pT1 low grade (n=1) (P=0.003). The false positive rate for Hexvix(®) was 53.4% versus 52% for white light. Previous TCC history, multifocality and EORTC score for recurrence and progression have been associated with better bladder cancer targeting by Hexvix(®) (P=0.007; P=0.01; P=0.03; P=0.04). CONCLUSION: In our experience, Hexvix(®) targeting was associated with better diagnosis for bladder cancer with 9% (13/144) of new positive samples (P=0.003). In our study, false positive rate is a critical point (53.4%). Multifocality, previous TCC, and EORTC score for recurrence and progression might allow selecting patients to be targeted by Hexvix(®).

Perfusion MRI as a neurosurgical tool for improved targeting in stereotactic tumor biopsies.

OBJECTIVE: Stereotactic biopsies are subject to sampling errors (essentially due to target selection). The presence of contrast enhancement is not a reliable marker of malignancy. The goal of the present study was to determine whether perfusion-weighted imaging can improve target selection in stereotactic biopsies. METHODS: We studied 21 consecutive stereotactic biopsies between June 2009 and March 2010. Perfusion-weighted magnetic resonance imaging (MRI) was integrated into our neuronavigator. Perfusion-weighted imaging was used as an adjunct to conventional MRI data for target determination. Conventional MRI alone was used to determine the trajectory. RESULTS: We found a linear correlation between regional cerebral blood volume (rCBV) and vessel density (number of vessels per mm(2); R = 0.64; p < 0.001). Perfusion-weighted imaging facilitated target determination in 11 cases (52.4%), all of which were histopathologically diagnosed as glial tumors. For glial tumors, which presented with contrast enhancement, perfusion-weighted imaging identified a more precisely delimited target in 9 cases, a different target in 1 case, and exactly the same target in 1 other case. In all cases, perfusion-selected sampling provided information on cellular features and tumor grading. rCBV was significantly associated with grading (p < 0.01), endothelial proliferation (p < 0.01), and vessel density (p < 0.01). For lesions with rCBV values ≤1, perfusion-weighted MRI did not help to determine the target but was useful for surgical management. CONCLUSIONS: For stereotactic biopsies, targeting based on perfusion-weighted imaging is a feasible method for reducing the sampling error and improving target selection in the histopathological diagnosis of tumors with high rCBVs.

[Prospective monocentric evaluation of bladder tumor targeting by Hexvix® fluorescence: preliminary results]. / Évaluation prospective monocentrique du ciblage diagnostique par hexaminolevulinate (Hexvix®) des tumeurs urothéliales de vessie: étude préliminaire.

PURPOSE: Prospective evaluation of bladder tumor targeting by Hexvix(®) fluorescence. PATIENT AND METHODS: From September 2008 to September 2009, 28 patients have been evaluated using Hexvix(®) technique (blue light) for typically papillary bladder tumor or suspected bladder tumor. Clinical and pathological data have been collected and patients have been classified using EORTC score. RESULTS: From the 28 patients, 14 have been identified with bladder cancer (31 tumors) and 90 samples have shown positive fluorescence in blue light. Compared to white light, four additional tumors have been diagnosed by Hexvix(®) for three patients (p=NS): two cis, one LMP, one pTa low grade. The false positive rate for Hexvix(®) was 65.5 versus 58.5% for white light. EORTC score for bladder cancer was associated with better bladder cancer targeting by Hexvix(®) (p<0.05). CONCLUSION: In our experience, Hexvix(®) targeting was associated with better diagnosis for bladder cancer. Thirteen percent of new positive samples have been found for 10.7% of patients. In our study false positive rate is a critical point (65.5%). EORTC score for recurrence and progression might allow to select patients to be targeted by Hexvix(®).

Pathological features of temporal arteries in patients with giant cell arteritis presenting with permanent visual loss.

BACKGROUND: Permanent visual loss (PVL) is the most feared complication of giant cell arteritis (GCA), and its risk factors are still unclear. OBJECTIVES: The aim of our study was to assess the pathological features predictive of PVL on temporal artery biopsy (TAB) specimens in patients with GCA. METHODS: The slides of 391 TAB specimens from patients with GCA were reviewed by two pathologists without clinical information. RESULTS: A total of 29 patients (26 females and 3 males, mean age 78.3 years) presented with unilateral PVL at the onset of the disease, and 362 patients (258 females, 104 males, mean age 74.7 years), did not. The pathological features strongly predictive for PVL were the presence (p = 0.003), number (p = 0.001) and aggregates of giant cells (p = 0.001), presence of plasmocytes (p = 0.002), thickened intima (p = 0.007), neoangiogenesis (p = 0.001) and degree of arterial occlusion (p = 0.006). Presence of neutrophils, eosinophils, parietal necrosis, calcification in the arterial wall and disruption of the internal elastic membrane were similar in both groups. Total obstruction of the arterial lumen by a thrombus, intensity of the inflammatory cells infiltration and inflammation of small vessels, nerves and veins surrounding the temporal artery were not associated with blindness. In multivariate analysis, only giant cells remained significantly associated with PVL. CONCLUSION: Giant cells are strongly associated with PVL, with a significant gradient between great risk and large number of giant cells. However, PVL was neither associated with the intensity of the inflammatory infiltrate, nor with the presence of arterial thrombosis.

Myogenic Disease and Metabolic Acidosis: Consider Multiple Acyl-Coenzyme A Dehydrogenase Deficiency.

BACKGROUND: Multiple acyl-coA dehydrogenase deficiency (MADD) is a rare, inherited, autosomal-recessive disorder leading to the accumulation of acylcarnitine of all chain lengths. Acute decompensation with cardiac, respiratory or hepatic failure and metabolic abnormalities may be life-threatening. CASE PRESENTATION: A 29-year-old woman presented with severe lactic acidosis associated with intense myalgia and muscle weakness. The clinical examination revealed symmetric upper and lower limb motor impairment (rated at 2 or 3 out of 5 on the Medical Research Council scale) and clear amyotrophy. Laboratory tests had revealed severe rhabdomyolysis, with a serum creatine phosphokinase level of 8,700 IU/L and asymptomatic hypoglycemia in the absence of ketosis. Electromyography revealed myotonic bursts in all four limbs. The absence of myositis-specific autoantibodies ruled out a diagnosis of autoimmune myositis. Finally, Acylcarnitine profile and gas chromatography-mass spectrometry analysis of organic acids led to the diagnosis of MADD. A treatment based on the intravenous infusion of glucose solutes, administration of riboflavin, and supplementation with coenzyme Q10 and carnitine was effective. Lipid consumption was strictly prohibited in the early stages of treatment. The clinical and biochemical parameters rapidly improved and we noticed a complete disappearance of the motor deficit, without sequelae. CONCLUSION: A diagnosis of MADD must be considered whenever acute or chronic muscle involvement is associated with metabolic disorders. Acute heart, respiratory or hepatic failure and metabolic abnormalities caused by MADD may be life-threatening, and will require intensive care.

Prevalence of HPV genotypes determined by PCR and DNA sequencing in cervical specimens from French women with or without abnormalities.

BACKGROUND: Epidemiological data on human papillomavirus (HPV) are needed to estimate potential changes in type distribution induced by recent HPV vaccination strategies. OBJECTIVES AND STUDY DESIGN: The epidemiological distribution of HPV in 669 cervical specimens from French women with and without cytological abnormalities was evaluated using type-specific PCR or sequencing. The results were compared with those obtained using the Digene high-risk Hybrid Capture 2 (HR-HC2) assay. RESULTS: The overall prevalence of HPV was high (45.3%) in our study population. 285 of the 291 HPV-positive samples were typed. The distribution frequency concerned 34 different genotypes, with HPV16 being the most prevalent (32.6%). Other genotypes present were HPV31 (7.4%), HPV18, HPV 52 (both 6.0%), HPV6 (5.3%) and HPV66 (4.2%). The respective frequencies of all other genotypes were below 4%. The agreement with HR-HC2 was 78.8%. The distribution frequency data were also analyzed relatively to cytological and histological results. Our method enables the diagnosis of HPV infections with the additional advantage of genotyping. CONCLUSION: HPV infections in the area of France studied here involve numerous HPV types, but the high cumulative prevalences of types 16, 18, 6 and 11 (44.6% in total) would suggest a major impact of vaccination on these genotypes.

Is sclerosing angiomatoid nodular transformation (SANT) of the splenic red pulp identical to inflammatory pseudotumour? Report of 16 cases.

AIMS: To report 16 cases of sclerosing angiomatoid nodular transformation (SANT) of the splenic red pulp. METHODS AND RESULTS: Patients were selected in two phases. An initial group of seven patients was diagnosed with SANT based on the presence of angiomatoid nodules. Sheets of inflammatory fibrosis were found in three patients, resembling inflammatory pseudotumour (IPT); nine further cases of IPT were reviewed. Angiomatoid nodules were detected, leading to the diagnosis of SANT in all cases. The splenic mass (10-150 mm in diameter) was polycyclic, composed of multiple small nodules of loose connective tissue comprising myofibroblasts and a dense network of capillaries as well as some remnants of sinuses. Collagenous fibrosis surrounded them. Bands or large sheets of fibrosis, infiltrated by various inflammatory cells, particularly polytypic plasmacytes, resembling IPT, were present in 10 cases. CONCLUSIONS: SANT of the red pulp is a distinct benign pseudotumorous lesion of the spleen characterized by the presence of angiomatoid nodules. We observed such angiomatoid nodules in all our cases of splenic IPT, which were not follicular dendritic cell or myofibroblastic tumours. We therefore recommend careful examination for angiomatoid nodules in all suspected cases of splenic IPT.

[Gastric calcifying fibrous pseudotumor, a rare mesenchymal tumor of the stomach]. / La pseudotumeur fibreuse calcifiante gastrique, une tumeur rare de l'estomac.

We report a case of calcifying fibrous pseudotumor fortuitously discovered on abdominal CT-scan in a 50 year-old woman presenting with metrorragia. CT-scan showed a 2 cm hypodense nodular tumor of the gastric wall with granular calcifications. Upper endoscopy showed a nodular tumor along the lesser curvature of the gastric body, covered by normal gastric mucosa and biopsies were negative. A diagnosis of gastric stromal tumor was suspected and a partial gastrectomy was performed. On gross examination surgical specimen showed a firm, whitish nodular tumor measuring 2 cm in diameter. On microscopic examination the tumor was composed of whorls of dense hyalinized collagen bundles with a few fibroblasts. There were psammomatous calcifications and nodular aggregates of mononuclear inflammatory cells. Immunohistochemically, spindle cells stained for vimentin. They did not stain for smooth muscle actin, desmin, CD34, CD117, S100 protein, Ki-67 and ALK-1. Surgical resection of the tumor was complete. Patient has no evidence of disease with a follow-up of 12 months. Only seven cases of gastric calcifiying fibrous pseudotumors have been reported in the literature. These benign tumors are usually incidentally discovered. They have characteristic imaging and microscopic features and appear as a distinct clinicopathologic entity different from stromal tumors.

[Inflammatory fibroid polyp, a rare tumor of the appendix]. / Le polype fibroïde inflammatoire, une tumeur rare de l'appendice.

We report the rare occurrence of an inflammatory fibroid polyp of the appendix. The lesion was diagnosed in a 33-year-old woman presenting with abdominal pain, fever and localized tenderness in right iliac fossa on abdominal palpation. CT-scan showed an 8 cm appendiceal mass and a laparoscopic appendectomy was consequently performed. On microscopic examination, the tumor consisted of spindle cells dispersed in a loose fibromyxoid stroma containing numerous blood cells and inflammatory cells with abundant eosinophils. On immunohistochemistry, the spindle tumor cells were positive for vimentin, fascin and focally for CD34 and CD35. They were negative for smooth muscle actin, desmin, CD21, CD23, CD117 and S100 protein. Inflammatory fibroid polyp is a rare benign mesenchymal tumor of the gastrointestinal tract rarely reported in the appendix. This tumor shares some common pathologic features with the myofibroblatic inflammatory tumor but they are two different entities. The pathogenesis of this tumor remains unclear but fascin and CD35 immunoreactivity of the tumor cells suggests a probable dendritic cell origin.

[Bone epithelioid hemangioendotheliom]. / Hémangioendothéliome épithélioïde osseux.

INTRODUCTION: Epithelioid hemagioendothelioma (HHE) is a rare mesenchymal tumor of vascular origin and epithelial appearance, which develops like angiosarcoma to mimic endothelial cells. According to the literature, its prognosis is variable and remains unpredictable. CASE REPORT: We report a 72-year-old man who presented with an inflammatory pain in the left lower limb. Several osteolytic lesions involving the knee, the upper third of the tibia, the medial malleolus and the left calcaneus were identified. The diagnosis HHE was obtained by histological examination of a bone sample. The patient died after 5 months, despite taxol chemotherapy. CONCLUSION: No therapeutic behavior is standardized in this uncommon type of cancer.

A case of Erdheim-Chester disease with spinal cord compression and sphenoid sinus involvement.

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis. It is an inflammatory disorder associated with BRAF V600E mutation in 50% of cases. This multisystem disease is rarely associated with spinal involvement. Neurological involvement is an independent predictive factor of poor prognosis. The diagnosis is histopathological based on CD68-positive and CD1A-negative histiocytes. Treatment with interferon-alpha is an independent predictor of survival in Erdheim-Chester disease and vemurafenib has also been shown to be effective for BRAF V600E mutation. We report a clinical case of a 51-year-old patient with multiple and rare locations of Erdheim-Chester disease, particularly at the sphenoid sinus.

[Cytological history of cases of invasive cervical cancer diagnosed in France in 2006]. / Histoire cytologique des cancers du col utérin diagnostiqués en France en 2006.

OBJECTIVE: After a significant decrease of the incidence of invasive cervical cancer with the practice of cytologic screening, the numbers remain the same nowadays. To explain the reasons for that, we performed a survey of the cases of invasive cervical cancer observed in France in 2006. MATERIAL AND METHOD: A questionnaire about screening practice was sent to every member of the French Society of Colposcopy (SFCPCV) to fill out for every invasive cancer diagnosed in 2006. RESULTS: Five hundred and twenty-four cases were collected: 2/3 of them never had a pap smear or were under screened. Indeed 27% had a labelled normal smear in the last three years. DISCUSSION AND CONCLUSION: As many others, this study shows that the first risk factor remains the lack of screening. But, 27% are false negative pap smears and it is even 45% under 45 years. The first thing to do is an organized mass screening but the increasing number of false negatives raises the question of the quality control of the cytology, or else to turn to a new test.

[Biometric and histological study of human, porcine and sheep internal thoracic arteries (reflections on their use in revascularization of arteries of less than four millimetres diameter)]. / Etude biométrique et histologique comparative des artères thoraciques internes de l'homme, du porc et du mouton (application en chirurgie de revascularisation des artères de moins de quatre millimètres de diamètre).

In order to enhance the knowledge of the internal thoracic arteries of pig, sheep, and man, allowing to constitute a fundamental basis as for the current and later applications in the interventions in surgery of revascularization of the arteries of less than four millimetre of diameter, a biometric and histological comparative study of these arteries was carried out. Forty human corpses, 27 men and 13 women (mean age 75+/-6 years) were dissected, alike with three pigs respectively weighing 80 kg, 80 kg and 84 kg and four sheep weighing 70 kg each. The left and right internal thoracic arteries were harvested: exposure over the entire length and remote dissection before excision. At the same time their internal lengths and gauges were measured. The internal thoracic artery (ITA) of human has an average useful length of 18 cm and an average internal gauge close to 1.5 mm. ITA of the pig has an average length of 27 cm and an average internal gauge close to 2.8 mm. The ITA of the sheep has an average length of 18 cm with an average internal gauge close to 1 mm. The porcine Internal Thoracic Artery is an elastic artery like its human counterpart. Many elastic fibres and few smooth muscle cells are present in the media. On the other hand, the internal thoracic artery of the sheep has a mixed structure. Its media contains more smooth muscle cells than elastic fibres.

Impact of anaesthesia mode on evaluation of LEEP specimen dimensions.

OBJECTIVES: To study the influence of anaesthesia (local by cervical block vs. general or spinal anaesthesia) on height and volume of resection specimens in case of conization treatment for cervical intraepithelial neoplasia (CIN). METHODS: Prospective observational study of all patients who underwent a first treatment by loop electrosurgical excision procedure (LEEP) for CIN. Height of fresh resection specimens was first measured by the operator and then by the pathologist after formaldehyde fixation. Volume of fresh specimens was measured in a measuring cylinder by fluid displacement. RESULTS: One hundred patients were included and 35% of LEEP were performed under local anaesthesia. There was a significant difference in height of specimens depending on anaesthesia mode: after fixation, the average height was 11.2mm in the general or spinal anaesthesia group vs. 8.8mm in the local anaesthesia group (P=0.002). There was also a difference in terms of volume depending on anaesthesia mode: 1.6mL in local anaesthesia group vs. 2.3mm in general and spinal anaesthesia group (P=0.01). CONCLUSIONS: Anaesthesia mode has an impact on height and volume of LEEP specimens. In our experience, local anaesthesia could reduce LEEP specimen height.