A web-based prognostic tool for extremity and trunk wall soft tissue sarcomas and its external validation.

BACKGROUND: We developed a web-based, prognostic tool for extremity and trunk wall soft tissue sarcoma to predict 10-year sarcoma-specific survival. External validation was performed. METHODS: Patients referred during 1987-2002 to Helsinki University Central Hospital are included. External validation was obtained from the Lund University Hospital register. Cox proportional hazards models were fitted with the Helsinki data. The previously described model (SIN) includes size, necrosis, and vascular invasion. The extended model (SAM) includes the SIN factors and in addition depth, location, grade, and size on a continuous scale. Models were statistically compared according to accuracy (area under the ROC curve=AUC) of 10-year sarcoma-specific survival prediction. RESULTS: The AUC of the SAM model in 10-year survival prediction in the Helsinki patient series was 0.81 as compared with 0.74 for the SIN model (P=0.0007). The corresponding AUCs in the external validation series were 0.77 for the SAM model and 0.73 for the SIN model (P=0.03). A web-based calculator for the SAM model is available at http://www.prognomics.org/sam. CONCLUSION: Addition of grade, depth, and location as well as tumour size on a continuous scale significantly improved the accuracy of the prognostic model when compared with a model that includes only size, necrosis, and vascular invasion.

[Purtscher-like retinopathy associated with adult onset still disease: Case report and review of the literature]. / Pseudo-rétinopathie de Purtscher dans le cadre d'une maladie de Still de l'adulte : à propos d'un cas et revue de la littérature.

INTRODUCTION: Putscher-like retinopathy is a retinal disease that is similar to the syndrome initially described in 1910 by Purtscher, but occurring in a non-traumatic context. CASE REPORT: We describe a case of acute, Putscher-like retinopathy in a 48-year-old woman experiencing adult onset Still's disease. The diagnosis was based on fundus examination and fluorescein angiography. Based on a review of the literature, we discuss the current available data on the pathophysiology of this syndrome and its prognostic significance. The treatment remains controversial. CONCLUSION: When visual functional signs appear during adult Still's disease, it is necessary to evoke Putscher-like retinopathy, and to ask for an ophthalmological expertise.

[Ocular syphilis, a re-emergent pathology: Series of 12 patients in one Hospital, 2017]. / La syphilis oculaire, une pathologie ré-émergente : série de 12 patients au CHU de Marseille en 2017.

INTRODUCTION: Syphilis is a sexually transmitted disease. All organs might be affected, but ocular syphilis only occurs in 0.6 percent of patients. We collected all cases of ocular syphilis requiring hospitalization at the University Hospital Center (UHC) in Marseille in 2017. PATIENTS AND METHODS: This was a retrospective monocentric study. The diagnosis of ocular syphilis was based on the combination of ocular inflammation with a positive syphilitic serology. For each patient, sex, age, HIV status, ocular and extraocular symptoms, initial visual acuity, syphilis serology, cerebrospinal fluid (CSF) analysis if done, treatment and clinical response were collected. RESULTS: Ten men and two women, aged 28 to 86 years, were hospitalized. Two patients were HIV-positive. Ophtalmological lesions were heterogeneous the posterior structures were most affected. Anterior uveitis was isolated in one patient. Five patients had extraocular signs with cutaneous and/or mucosal involvement. No patient had neurological symptoms. Diagnosis of neurosyphilis through CSF analysis was definite for one patient, probable for 5 patients and ruled out for 2 patients. Six patients received treatment with penicillin G and six with ceftriaxone. Visual acuity improved in all cases. DISCUSSION: Ophtalmic cases of syphilis have become more frequent over the past few years in France. The diagnosis should be suspected in cases of eye inflammation even in the absence of favourable clinical presentation or anamnesis. Search for HIV co-infection should be systematic. Our study shows that ceftriaxone remains an effective alternative to penicillin G.

Microvascular reconstruction after resection of soft tissue sarcoma of the leg.

BACKGROUND: Limb-sparing surgery and satisfactory functional outcome is the goal of extremity soft tissue sarcoma (STS) surgery. Tissue defects after tumour excision are often extensive, and microvascular reconstruction is frequently required. METHODS: Seventy-three patients with STS of the leg requiring microvascular reconstruction were treated between 1985 and 2006. Radiotherapy was delivered if the microscopic surgical margin was less than 2.5 cm. RESULTS: Mean follow-up was 65.9 months. Seventy-five free flaps were performed, with a success rate of 95 per cent. One patient died within a month of surgery. Five-year local recurrence-free survival was 82 per cent, metastasis-free survival 59 per cent, disease-free survival 56 per cent and disease-specific overall survival 70 per cent. Fifty-five (75 per cent) of the 73 patients were able to walk normally or had only minor walking impairment. CONCLUSION: Without microvascular reconstruction, amputation would have been necessary in most patients. Microvascular reconstruction is safe and reliable in lower extremity STS reconstruction.

Craniomaxillofacial osteosarcoma - The role of surgical margins.

BACKGROUND: Osteosarcomas (OS) in the craniomaxillofacial (CMF) region are typically diagnosed at later age than long-bone OS, but they are reported to have better 5-year survival. Curative treatment warrants wide surgical resection, which is often not possible in the CMF region. The purpose of this article is to present a nationwide series of CMF in Finland to discuss the role of surgery. PATIENTS AND METHODS: All 21 CMF OS patients managed in Finland from 1992 to 2009 were included. The mean age was 40 years (range 15-72). Data on patient and tumor characteristics, treatment modalities, and survival were recorded. All patients had a minimum follow-up of 5 years or until death. RESULTS: OS was evenly represented in the mandible and maxillary bones, which together constituted 76% of all sites. Surgery with curative intent was carried out in 20 patients. Clear margins were achieved in only five cases. Eight (40%) of these 20 patients died due to OS, and their average survival time was 1.3 years. Seven (35%) out of the 20 patients received radiotherapy due to close/intralesional surgical margins, and four of them did not develop recurrences during the follow-up. CONCLUSIONS: The results suggest that postoperative radiotherapy may alter the prognosis in CMF OS, particularly in cases with close or intralesional margins. This may increase the survival rates achieved by prompt action in performing radical surgery.

Impact of the smallest surgical margin on local control in soft tissue sarcoma.

BACKGROUND: The aim was to review a single-institution experience of a prospective treatment protocol for soft tissue sarcoma of the extremity and trunk wall, with particular focus on the smallest surgical margin leading to local control. METHODS: The study included 270 patients who had surgery for soft tissue sarcoma at Helsinki University Central Hospital between 1987 and 1997. Resection margins were measured prospectively from tumour specimens. Radiotherapy was administered if the smallest margin measured less than 2.5 cm, irrespective of tumour grade. RESULTS: With a median follow-up of 6.6 years, the 5-year local control rate was 76.4 per cent. On multivariable analysis, the smallest surgical margin around the sarcoma (after radiotherapy) was prognostic for local control. A margin of at least 2.5 cm was associated with a local recurrence-free rate of 89.2 per cent at 5 years. Tumour size, depth or grade and patient's age had no independent prognostic effect on local control. CONCLUSION: Surgical margin had independent prognostic value for local control. A surgical margin of 2-3 cm provided reasonable local control of soft tissue sarcoma, even without radiotherapy. Radiotherapy is recommended for smaller margins, irrespective of tumour grade.

Intraocular pressure measurements and corneal biomechanical properties using a dynamic Scheimpflug analyzer, after several keratoplasty techniques, versus normal eyes.

PURPOSE: To evaluate the biomechanical properties of the cornea and their impact on intraocular pressure (IOP) measurement after lamellar keratoplasty, compared to healthy eyes, using a non-contact tonometer with a Scheimpflug camera. METHODS: This study, from 2014 to 2015, included 22 primary DSAEK, 5 DALK, 6 DSAEK after PK, and 50 control eyes. Using a non-contact tonometer with a high-speed Scheimpflug camera (CORVIS ST, Oculus Optikgeräte GmbH, Wetzlar, Germany), several biomechanical parameters were recorded, including radius at highest concavity (Rhc) and defomation amplitude (DA). Central corneal thickness (CCT) and uncorrected IOP, were also recorded. For the control eyes only, a corrected IOP was calculated, based on age, central corneal thickness, and biomechanical parameters. RESULTS: Rhc was significantly lower after DALK (Rhc=5.54±0.71, P=0.007) and DSAEK (Rhc=6.26±0.77, P=0.042) compared to control eyes (Rhc=6.82±0.76). DA was higher after DALK and DSAEK, but not significantly (respectively 1.24±0.09 P=0.41 and 1.22±0.15, P=0.923) compared to normal eyes (1.18±0.15). Uncorrected IOP was not significantly different between post-keratoplasty and control eyes. In control eyes, the corrected IOP (15.23±1.88) was lower than the uncorrected IOP (16.10±2.34); a statistically significant positive correlation between Rhc and CCT (R2=0.6020, P<0001), and a significant negative correlation between DA and CCT (R2=-0.641, P<0.0001) were found. CONCLUSION: Our study showed that, after lamellar keratoplasty, corneal biomechanics are altered. Corneas with higher ocular rigidity will show a lower DA and a higher Rhc.

[Effect of donor graft characteristics on clinical outcomes in Descemet stripping automated endothelial keratoplasty (DSAEK)]. / Impact des caractéristiques du greffon cornéen sur les résultats cliniques après Descemet stripping automated endothelial keratoplasty (DSAEK).

INTRODUCTION: The purpose of our study was to evaluate the progression of lamellar corneal grafts after endothelial keratoplasty by Descemet stripping automated endothelial keratoplasty (DSAEK) and the effect of their characteristics on the clinical and functional results. MATERIALS AND METHODS: This was a prospective study in which 74 patients who had undergone endothelial keratoplasty by DSAEK were included. The corneal grafts were obtained from a French tissue bank and were delivered in a preservation medium containing a deturgescent agent (Corneajet® or Stemalpha 3®). Pachymetry of the corneal graft was measured ex vivo by ultrasonic pachymetry prior to dissection, then after the 1st or 2nd cut as necessary. Corrected visual acuity in logMAR, total graft pachymetry, measured by anterior segment OCT (Spectralis® HRA+SDOCT, Heidelberg engineering, Inc., Heidelberg, Germany) and corneal density (Pentacam®, Oculus, Inc., Wetzlar, Germany) were obtained for each patient preoperatively, at D5, M1, M3, M6, M9 and M12. RESULTS: Overall, 74 corneal grafts, from donors of mean age 69.7±13.3 years (37 to 92 years) were transplanted into 67 patients aged 70.3±12.1 years. Predissection pachymetry was statistically significantly thicker in the case of short deturgescence time (r=-0.383, P=0.001). For the same deturgescence time, predissection pachymetry was statistically significantly thicker in the Stemalpha 3® medium (691±89µm for Corneajet® vs. 760±100µm for Stemalpha 3®, P=0.01), with the same final pachymetry (168±31µm vs. 166±25µm, P=0.833). Graft pachymetry and total corneal pachymetry decreased significantly between the preoperative measurement and M12 (168±29µm vs. 92±57µm, P=0.0008 et 665±124µm vs. 566±73µm, P=0.027 respectively). Preoperative endothelial cell density (2938±418 cells/mm2) did not correlate with postoperative visual acuity. There was a positive correlation between visual acuity at D5 and donor age (r=0.3, P=0.04). Postoperative visual acuity correlated positively with preoperative visual acuity (P<0.05). Corneal densitometry was statically higher than normal postoperative densitometry. Corneal density at M1 correlated positively with time since obtaining the donor tissue (r=0.373, P=0.043). There was no significant difference between the sub-groups of over or under 130µm. Seven patients experienced graft failure with no statistically significant graft risk factor. DISCUSSION: The preservation medium affects predissection pachymetry with no consequence on clinical results. In our study, preoperative visual acuity and donor age were correlated with visual acuity after DSAEK. CONCLUSION: Although donor age was associated with better visual acuity at D5, the other donor characteristics had no effect on clinical results.

[Eplerenone treatment in chronic central serous chorioretinopathy]. / Traitement par éplérénone dans la choriorétinite séreuse centrale chronique.

PURPOSE: To evaluate the efficacy of eplerenone, a mineralocorticoid receptor antagonist, in the treatment of chronic central serous chorioretinopathy (CSCR). MATERIALS AND METHODS: We conducted a retrospective study of 27 patients treated with eplerenone for chronic CSCR of at least 3 months duration. For each patient, visual acuity and macular OCT (central retinal thickness, height of foveal subretinal fluid (SRF), central choroidal thickness) were evaluated before treatment and at 1 month and 3 months. In the case of complete disappearance of SRF at 1 month, treatment was discontinued and follow-up was performed at 3 months. RESULTS: Central retinal thickness was 371.6µm (266-573µm) before treatment. A clear decrease in retinal central thickness and height of SRF was observed at 1 month in 74% of patients (20 of 27 patients, central retinal thickness: 322.6µm at 1 month, P=0.01), with improvement of visual acuity in all of these patients. Follow-up at 3 months also found a decrease in SRF and central retinal thickness (294.3µm, P=0.002). Six patients had complete resolution of SRF at 1 month, without recurrence at 3 months. Six other patients had complete resolution of SRF at 3 months. No side effects requiring treatment discontinuation were observed. CONCLUSION: In our study, eplerenone was associated with regression of central retinal thickness and height of SRF. Eplerenone appears to be a safe and effective treatment for chronic CSCR, with a probable mechanism of action on the pathophysiology of this disease.

[Visual acuity, pachymetry and corneal density after 5% sodium chloride treatment in corneal edema after surgery]. / Acuité visuelle, pachymétrie et densité cornéenne après traitement par solution hyperosmolaire au chlorure de sodium 5 % dans l'œdème cornéen postopératoire.

PURPOSE: To evaluate the effectiveness of a hyperosmolar medication in corneal edema. METHODS: This was a prospective randomized study, performed in patients with post-operative corneal edema. Two groups were created: group 1 treated with hypertonic eye drops of 5% sodium chloride with 0.15% sodium hyaluronate in addition to the usual post-operative treatment for 1 month; group 2 without treatment for edema. Visual acuity, pachymetry and corneal density were measured at 1 day, 1 week, 1 month, 3 months and 6 months following surgery. RESULTS: Forty-four patients were included in group 1 and 42 in group 2. Visual acuity was significantly better in group 1 than in group 2 at 7 days (0.84 logMAR vs 1.55 logMAR, P=0.019 CI 95% [-1.298;-0.128]) and was no longer different at one month, three and six months. Pachymetry decreased significantly in 7 days in group 1 (decrease of 17%, P=0.04, CI 95% [1.987; 258.305]), compared to group 2 (P=0.8, CI 95% [-132.9; 161.8]), but the difference between the 2 groups was not statistically significant (P=0.11, CI 95% [-16; 19.1]). Corneal density was also not significantly different between the two groups. CONCLUSION: Five percent sodium chloride hypertonic eye drops show a statistically significant reduction in post-operative corneal edema as shown by the improvement in visual acuity at one week and the downward trend in pachymetry.

Surgically treated patients with axial and peripheral Ewing's sarcoma family of tumours: A population based study in Finland during 1990-2009.

BACKGROUND: The surgical treatment of Ewing's sarcoma family tumours (ESFTs) is challenging especially with axial tumours. The aim of the study was to analyse surgical treatment and outcome in a nationwide, population-based material consisting of surgically treated axial and peripheral ESFTs of bone and soft tissue. METHODS: The data were collected from the Finnish National Cancer Registry and the medical records of patients diagnosed during 1990-2009. Fifty-seven patients with surgically treated ESFTs were included, 22 with an axial and 35 with a peripheral primary tumours. The surgical treatment, its complications, survival and prognostic factors were analysed. RESULTS: Fifty-four patients underwent surgery with a curative intent and three underwent de-bulking operations. Bone reconstruction was performed in six patients with an axial and 15 with a peripheral tumour. Positive resection margins were associated with a worse five-year local relapse-free survival (33% vs. 84% for those with resection margins free of tumour cells, p = 0.003). The five-year sarcoma-specific survival was affected only by an axial location of the primary (61% vs. 89% for those with a peripheral tumour, p = 0.031). The late complications were mainly associated with bone reconstruction and more frequent among patients with a peripheral compared to an axial tumour (p = 0.031). CONCLUSIONS: In the treatment of ESFTs, achieving adequate resection margins is crucial to avoid local relapses. Surgical complications are common particularly with bone reconstruction.