Adenocarcinome au sein d'un adenome pleomorphe de la glande lacrymale. A propos d'un cas. [Adenocarcinoma in a pleomorphic adenoma of the lacrimal gland: a case study]

PURPOSE: To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland. METHODS: The clinical presentation, workup, surgical approach, and pathological findings were reviewed. RESULTS: A 56-year-old man presented with a 3-month history of a lacrimal fossa mass. This patient presented a painless mass in the upper outer eyelid with significant displacement of the globe. The tumor was localized to the lacrimal gland. Radiological investigations showed a round, well-defined lesion in the fossa of the lacrimal gland. We performed a lateral orbitotomy; en bloc resection was accomplished. The final specimen showed an adenocarcinoma in a pleomorphic adenoma. No recurrences were detected during follow-up. CONCLUSION: Although adenocarcinoma has rarely been reported in association with a pleomorphic adenoma of the lacrimal gland, this combination can exist. If the malignant tumor is limited in the pleomorphic adenoma, the prognosis appears to be better than in cases of local extension. Adjuvant therapy, radiation or others, are not useful.

Les adénocarcinomes kystiques de la glande lacrymale [Cystic adenocarcinomas of the lacrymal gland]

Adenoid cystic carcinoma of the lacrimal gland. BACKGROUND: Adenoid cystic carcinoma is the most frequent epithelial tumour of the lacrimal gland. It represents 1.6 % of the orbital tumours. The aim of this study is to present a series of such patients who were followed up in our clinic and to discuss their clinical management in order to achieve a better life prognosis. MATERIALS AND METHODS: The cases we followed up in our department were presented, the treatment modalities were discussed, and conclusions were drawn. RESULTS: Six patients with this pathology were examined, 3 men and 3 women, aged 31 to 69 years. A large excision was performed in 5 patients (exenteration alone or extended exenteration with complementary radiotherapy) and in 1 patient with excision of the tumour followed by radiotherapy. One patient died due to carcinoma six years after the first surgery. The median follow-up period of our cases is 25 months. CONCLUSION: Adenoid cystic carcinoma of the lacrimal gland is a malignant tumour with a reserved prognosis. Various treatments were proposed to improve the mortality rate of the disease. At present radical surgery with or without complementary therapy seems to give the best chances of healing, at least with short or mid-term follow-up.

Adénome pléomorphe simulant une dacryoadénite [Pleomorphic adenoma mimicking dacryoadenitis].

A 56-year-old female patient displayed an increased volume of her left lacrimal gland with pain and inflammation. On CT scan, a hyperdense lacrimal mass was found in the left lacrimal fossa. Observation was the first course, for no steroid therapy could be applied because of evolving digestive ulcers. After 9 months of observation, the gland was surgically removed en bloc through a wide anterolateral approach. Pathological analysis of the gland showed a pleomorphic adenoma. This case illustrates the advantage of a wide surgical approach in treating lacrimal gland tumors, in order to avoid or limit the risk of recurrence.

Primary Sjögren's syndrome (SS) and malignant lymphoma. A retrospective cohort study of 55 patients with SS.

The aim of this study was to assess the prevalence of malignant lymphomas in patients with long-standing primary Sjögren's syndrome (pSS). We retrospectively studied a cohort of 55 patients with pSS over a mean follow-up period of 12 years. Five patients (9%) developed malignant lymphoma. The interval between the diagnoses of SS and lymphoma ranged from four to 12 years (mean = 6.5 years). The lymphoma arose in the lymph nodes in two cases, the parotid gland in one case, the lacrimal gland in one case, and the lung in one case. All five cases were B-cell low-grade lymphomas. Among our SS patients, those with extraglandular manifestations and/or a mixed cryoglobulin were at increased risk for lymphoma development. Secondary lymphoma carried a poor prognosis in our study. Three of the six SS patients who died during the follow-up period had lymphoma.

Different locations of dermoid cysts in the orbital region.

BACKGROUND: Dermoid cysts are frequent unilateral congenital benign tumors that can be found at different locations throughout the body. In the orbital region, dermoid cysts occur predominantly in the supero-temporal quadrant. However, different orbital locations are possible, making this entity an important differential diagnosis for orbital tumors. PATIENTS AND METHODS: We retrospectively reviewed the charts of 23 consecutive patients operated in our institution between 2005 and 2014 for orbital tumors that were diagnosed as congenital dermoid or epidermoid cysts. RESULTS: There were 21 dermoid and 2 epidermoid cysts. The median age at surgery time was 7 years (range 1-41). There were 15 females and 8 males. Eleven cysts were located supero-temporally (47.8%), seven supero-medially (30.4%), two temporally (8.7%), one at the frontal bone (4.4%) and two in the fossa of the lacrimal gland (8.7%). The mean cyst diameter was 13.1 mm±5.0 (SD). None had signs of malignant disease. No recurrence was observed after complete excision. CONCLUSION: Dermoid cysts of orbital region are mostly located in the supero-temporal quadrant. However, other orbital locations are possible and dermoid cysts must thus be considered in the differential diagnosis of any mass in the orbital region.

Gastric outlet obstruction by Brunner's gland hyperplasia in an 8-year-old child.

Several cases of Brunner's gland hyperplasia causing hemorrhage, obstruction, or intussusception have been published in the adult literature. Similar cases in the pediatric population are very rare and have only been described twice, always associated with chronic renal failure. We report the third and youngest case of gastric outlet obstruction because of Brunner's gland hyperplasia focusing on histopathologic condition and treatment based on a review of the literature.

Suprapineal recess: an alternate site for third ventriculostomy? Case report.

This 30-year-old woman presented with clinical symptoms and signs of intracranial hypertension and Parinaud syndrome secondary to ventriculoperitoneal shunt dysfunction. Magnetic resonance (MR) imaging revealed gross triventricular hydrocephalus with a large suprapineal recess due to aqueductal stenosis. Using an endoscopic approach, a ventriculostomy was performed within the floor of the dilated suprapineal recess. Following this procedure the patient experienced alleviation of all her neurological symptoms and signs. Postoperative MR imaging and cerebrospinal fluid flow studies demonstrated a functioning ventriculostomy. The anatomy of the suprapineal recess and its suitability for endoscopic ventriculostomy are discussed.

Clinicopathological features of eyelid skin tumors. A retrospective study of 5504 cases and review of literature.

Eyelid tumors are the most common neoplasm in daily ophthalmology practice and encompass a wide variety of benign and malignant tumors. In this retrospective study, we report the clinical and histological features of 5504 eyelid skin tumors diagnosed at the Laboratory of Ophthalmopathology of the Hôpital Ophtalmique Jules Gonin, Lausanne, Switzerland, between January 1989 and December 2007. Benign tumors largely predominated over malignant ones, representing 84% of cases in this series, and the 5 most frequent subtypes were squamous cell papilloma (26%), seborrheic keratosis (21%), melanocytic nevus (20%), hidrocystoma (8%), and xanthoma/xanthelasma (6%). Basal cell carcinoma was the most frequent malignant tumor (86%), followed by squamous cell carcinoma (7%) and sebaceous carcinoma (3%). For several tumor subtypes, there was a poor correlation between clinical and histological diagnosis, stressing the numerous pitfalls in the diagnosis of eyelid tumors. We further discuss our results with reference to previously published series.

The use of mannan antigen and anti-mannan antibodies in the diagnosis of invasive candidiasis: recommendations from the Third European Conference on Infections in Leukemia.

INTRODUCTION: Timely diagnosis of invasive candidiasis (IC) remains difficult as the clinical presentation is not specific and blood cultures lack sensitivity and need a long incubation time. Thus, non-culture-based methods for diagnosing IC have been developed. Mannan antigen (Mn) and anti-mannan antibodies (A-Mn) are present in patients with IC. On behalf of the Third European Conference on Infections in Leukemia, the performance of these tests was analysed and reviewed. METHODS: The literature was searched for studies using the commercially available sandwich enzyme-linked immunosorbent assays (Platelia™, Bio-Rad Laboratories, Marnes-la-Coquette, France) for detecting Mn and A-Mn in serum. The target condition of this review was IC defined according to 2008 European Organization for Research and Treatment of Cancer/Mycoses Study Group criteria. Sensitivity, specificity and diagnostic odds ratios (DOR) were calculated for Mn, A-Mn and combined Mn/A-Mn testing. RESULTS: Overall, 14 studies that comprised 453 patients and 767 controls were reviewed. The patient populations included in the studies were mainly haematological and cancer cases in seven studies and mainly intensive care unit and surgery cases in the other seven studies. All studies but one were retrospective in design. Mn sensitivity was 58% (95% confidence interval [CI], 53-62); specificity, 93% (95% CI, 91-94) and DOR, 18 (95% CI 12-28). A-Mn sensitivity was 59% (95% CI, 54-65); specificity, 83% (95% CI, 79-97) and DOR, 12 (95% CI 7-21). Combined Mn/A-Mn sensitivity was 83% (95% CI, 79-87); specificity, 86% (95% CI, 82-90) and DOR, 58 (95% CI 27-122). Significant heterogeneity of the studies was detected. The sensitivity of both Mn and A-Mn varied for different Candida species, and it was the highest for C. albicans, followed by C. glabrata and C. tropicalis. In 73% of 45 patients with candidemia, at least one of the serological tests was positive before the culture results, with mean time advantage being 6 days for Mn and 7 days for A-Mn. In 21 patients with hepatosplenic IC, 18 (86%) had Mn or A-Mn positive test results at a median of 16 days before radiological detection of liver or spleen lesions. CONCLUSIONS: Mn and A-Mn are useful for diagnosis of IC. The performance of combined Mn/A-Mn testing is superior to either Mn or A-Mn testing.

Ataxia telangiectasia mutated (ATM) inhibition transforms human mammary gland epithelial cells.

Carriers of mutations in the cell cycle checkpoint protein kinase ataxia telangiectasia mutated (ATM), which represent 1-2% of the general population, have an increased risk of breast cancer. However, experimental evidence that ATM deficiency contributes to human breast carcinogenesis is lacking. We report here that in MCF-10A and MCF-12A cells, which are well established normal human mammary gland epithelial cell models, partial or almost complete stable ATM silencing or pharmacological inhibition resulted in cellular transformation, genomic instability, and formation of dysplastic lesions in NOD/SCID mice. These effects did not require the activity of exogenous DNA-damaging agents and were preceded by an unsuspected and striking increase in cell proliferation also observed in primary human mammary gland epithelial cells. Increased proliferation correlated with a dramatic, transient, and proteasome-dependent reduction of p21(WAF1/CIP1) and p27(KIP1) protein levels, whereas little or no effect was observed on p21(WAF1/CIP1) or p27(KIP1) mRNAs. p21(WAF1/CIP1) silencing also increased MCF-10A cell proliferation, thus identifying p21(WAF1/CIP1) down-regulation as a mediator of the proliferative effect of ATM inhibition. Our findings provide the first experimental evidence that ATM is a human breast tumor suppressor. In addition, they mirror the sensitivity of ATM tumor suppressor function and unveil a new mechanism by which ATM might prevent human breast tumorigenesis, namely a direct inhibitory effect on the basal proliferation of normal mammary epithelial cells.

β-Glucan antigenemia assay for the diagnosis of invasive fungal infections in patients with hematological malignancies: a systematic review and meta-analysis of cohort studies from the Third European Conference on Infections in Leukemia (ECIL-3).

BACKGROUND: Invasive fungal infections (IFIs) are life-threatening complications in patients with hemato-oncological malignancies, and early diagnosis is crucial for outcome. The compound 1,3-β-D-glucan (BG), a cell wall component of most fungal species, can be detected in blood during IFI. Four commercial BG antigenemia assays are available (Fungitell, Fungitec-G, Wako, and Maruha). This meta-analysis from the Third European Conference on Infections in Leukemia (ECIL-3) assessed the performance of BG assays for the diagnosis of IFI in hemato-oncological patients. METHODS: Studies reporting the performance of BG antigenemia assays for the diagnosis of IFI (European Organization for Research and Treatment of Cancer and Mycoses Study Group criteria) in hemato-oncological patients were identified. The analysis was focused on high-quality cohort studies with exclusion of case-control studies. Meta-analysis was performed by conventional meta-analytical pooling and bivariate analysis. RESULTS: Six cohort studies were included (1771 adult patients with 414 IFIs of which 215 were proven or probable). Similar performance was observed among the different BG assays. For the cutoff recommended by the manufacturer, the diagnostic performance of the BG assay in proven or probable IFI was better with 2 consecutive positive test results (diagnostic odds ratio for 2 consecutive vs one single positive results, 111.8 [95% confidence interval {CI}, 38.6-324.1] vs 16.3 [95% CI, 6.5-40.8], respectively; heterogeneity index for 2 consecutive vs one single positive results, 0% vs 72.6%, respectively). For 2 consecutive tests, sensitivity and specificity were 49.6% (95% CI, 34.0%-65.3%) and 98.9% (95% CI, 97.4%-99.5%), respectively. Estimated positive and negative predictive values for an IFI prevalence of 10% were 83.5% and 94.6%, respectively. CONCLUSIONS: Different BG assays have similar accuracy for the diagnosis of IFI in hemato-oncological patients. Two consecutive positive antigenemia assays have very high specificity, positive predictive value, and negative predictive value. Because sensitivity is low, the test needs to be combined with clinical, radiological, and microbiological findings.

Interet de la double voie d'abord dans les blepharoplasties superieures associees aux malpositions palpebrales. [Advantages of a double approach to upper blepharoplasty associated with eyelid malpositions]

During upper blepharoplasty, myocutaneous excess and fat pads are treated using an anterior approach. Eyelid malpositions such as involutional ptosis or lid retraction could be associated and should be treated with associated procedures. Aponeurotic surgery on the levator muscle can make use of the same anterior approach, with the major difficulty of dosage. In cases of ptosis with a positive epinephrine test or minor muscular retraction, the Muller muscle-conjunctival surgery via a posterior approach seems to be more reproducible. Double-approach techniques are described.