Comparative Analysis of the Prognostic Significance of IDH,TERT, EGFR and MGMT Status in Patients with Adult Non-H3-Altered Grade 4 Gliomas: A Prospective Cohort Study.

INTRODUCTION: Gliomas continue to have a dismal prognosis. A myriad of genetic alterations has been described in this subset of tumors over the last decades. The integrative interpretation of the biomarker constellation for individual patients remains unclear. This study aims to evaluate the impact of some known genetic factors as prognostic biomarkers in grade 4 gliomas. METHODS: Adult non-H3-altered grade 4 gliomas who underwent maximal safe resection accompanied by adjuvant therapy were successively enrolled since January 2019 till January 2021. Patient data were documented preoperatively and during the follow-up visits. The genetic profiling of the tumors included Isocitrate Dehydrogenase (IDH)-1 and IDH-2 mutation, MGMT promoter methylation rate, EGFR gene amplification and telomerase reverse transcriptase gene promoter (TERTp) mutation. RESULTS: Mean Overall survival (OS) and Progression-free survival (PFS) were 14.45 ± 5.13 months (3-24 months) and 10.66 ± 4.87 months respectively. TERTp-mutant group had a significantly lower OS (10.9 vs. 15.9) and PFS (6.9 vs. 12.3) than TERTp wildtype group. In the TERT-mutant group, those with concomitant IDH wildtype tumor had higher OS and PFS, comparable to those with both TERTp and IDH wildtype tumors. In multivariate analysis, IDH mutation and TERTp wildtype status were predictive of longer OS and PFS. While IDH and absence of TERTp mutation were associated with KPS > 80 across the follow-ups, their predictive values were inferior to preoperative KPS scores. CONCLUSIONS: TERTp mutation and IDH-wildtype status were associated with worse OS and PFS and lower follow-up KPS score in surgically resected gliomas, while MGMT and EGFR status did not have considerable prognostic value in this study.

Prognostic Factors of the Primary Central Nervous System Lymphoma: Clinical Experience from a Tertiary Care Center in the Middle East.

Aim Primary central nervous system lymphoma (PCNSL) is a rare extra nodal non-Hodgkin's lymphoma. The optimal treatment for PCNSL is still unclear. In this study, we present our experience with management of PCNSL in a tertiary care center in Iran. Methods In this retrospective study, 58 patients with tissue diagnosis of PCNSL were studied. All patients were treated with chemotherapy including intravenous high-dose methotrexate, rituximab and temozolomide and radiotherapy by the same oncologist. Statistical analysis was performed using SPSS. Results The mean overall survival (OS) in this study was 37.4 ± 13.6 months and the mean progression free survival (PFS) was 35.1 ± 9.8 months. The mean time to progression was 15.2 ± 8.79 months among 8 patients who experienced progression in this series. Finding of a positive CSF cytology was not linked with disease progression, while HIV infection and multifocal involvement at initial presentation were strongly linked to a lower PFS. The single most important factor affecting the OS was the histopathologic type of the PCNSL; two of the three patients who died from their disease in this series had non-B cell PCNSL, whereas only one patient with DLBCL died because of brainstem involvement. Conclusion The results of this study show a lower rate of HIV-infection in patients with PCNSL as compared to the series from the western countries. Non-B cell histopathology and HIV-infection were found to be associated with the dismal prognosis.

Clinical Follow-Up of Patients with Neurocutaneous Melanosis in a Tertiary Center; Proposed Modification in Diagnostic Criteria.

INTRODUCTION: Neurocutaneous melanosis (NCM) is a rare congenital syndrome. Except for some retrospective studies, information on clinical follow-up and management of these patients are limited. This study aimed to review our experience on diagnostic protocol and clinical follow-up of patients with NCM in a referral children's hospital in Iran. METHODS: Between 2012 and 2019, eight patients with NCM were consecutively managed in our center. Brain magnetic resonance imaging and cutaneous biopsy were done in all patients at diagnosis. Follow-up surveillance and characteristics of the disease are described. RESULTS: The mean follow-up period was 25.75 ± 13.81 months, and 75% of patients were male. Most magnetic resonance imaging findings were hypersignal lesions in the temporal lobe (75%), cerebellum (62.5%), brainstem (50%), and thalamus (12.5%). Dandy-Walker syndrome was found in 4 patients (50%), and shunt-dependent hydrocephalus was found in 3 patients (37.5%). Cutaneous malignant melanoma and malignant involvement of the central nervous system were found in 2 (25%) and 3 cases (37.5%), respectively. The mortality rate was 37.5%. CONCLUSIONS: There are no specific guidelines for management of NCM due to the rarity of the disease. This study proposed modifications in diagnostic criteria, as well as recommendations for follow-up surveillance.

Adult cerebellopontine angle ependymoma presenting as an isolated cisternal mass: A case report.

INTRODUCTION: Ependymomas represent approximately 2%-8% of all primary intracranial brain tumors. The occurrence of extra-axial posterior fossa ependymomas in adults is rare. CASE AND OUTCOMES: We report a case of extra-axial cerebellopontine (CP) angle ependymoma in an adult patient, managed through gross total resection (GTR) and adjuvant radiotherapy. At her one-year postoperative visit, the patient remained clinically stable without any symptoms or focal neurological deficit and a follow up MRI showed no evidence of tumor recurrence. DISCUSSION: Only six cases of adult cerebellopontine angle ependymomas have been reported in the English literature, with the left side affected more commonly. Including this case, the mean age of the reported cases of adult extra-axial CP angle ependymoma is 44.14 years (range 22-66 years). Men accounted for five out of seven cases (71.4%). Maximal surgical resection is the mainstay of treatment in extra-axial CP angle ependymomas. Among seven reported cases, five received GTR and two had subtotal resection (STR). Patients were followed an average of 13.6 months (range 2-30 months) and only two patients with STR died during the follow-up period (6 weeks and 2 months after surgery). Six of the seven reported cases (including this one) received adjuvant radiotherapy. CONCLUSION: Although rare, extra-axial CP angle ependymomas should be considered as a differential diagnosis to other lesions of the CPA. Radical resection, whenever possible, is usually associated with a good outcome. Adjuvant radiotherapy remains an optional treatment with an unknown impact on overall and progression-free survival.

Ovarian and uterine alterations following forced swimming: An immunohistochemical study.

BACKGROUND: Physical exercise is known to be a stressor stimulus that leads to reproductive disruption. OBJECTIVE: The aim of this study was to evaluate the effect of forced swimming on the uterus and ovaries in mice. MATERIALS AND METHODS: Adult mice (N=24) were divided into the following three groups: A, control; B, swimming in water (10oC); and C, swimming in water (23oC). Swimmers swam for 5 min daily for 5 consecutive days/ wk during 2 wks. An enzyme linked immunosorbent assay was used to determine serum estradiol, follicle stimulating hormone (FSH) and testosterone levels. Immunohistochemistry was performed to study apoptotic cells or estrogen receptor (ER) expression in uterine epithelial cells and ovaries. ANOVA was used for statistical analysis. RESULTS: Swimming in both groups reduced the serum FSH and estradiol levels (p<0.01) without having a significant effect on the serum testosterone level or percentage of apoptosis in ovarian and uterine tissues (p<0.01) compared with controls. A significant reduction in the number of ERs in the uterus and ovaries, and secondary and graafian follicles were observed in groups B and C compared with controls (p<0.01); however the number of primordial and primary follicles were not significantly changed in the ovaries. CONCLUSION: Forced swimming of 2 wks duration reduces the serum levels of FSH and estradiol without having effects on apoptosis in the ovaries or uteri of mice. Over a long period of time, forced swimming may have an adverse effect on fertility.

Giant Right Coronary Artery Aneurysm Mimicking a Mediastinal Cyst With Compression Effects: A Case Report.

INTRODUCTION: Giant coronary artery aneurysm is an extremely rare form of coronary artery disease. The most common cause of coronary artery aneurysms is atherosclerosis. Although it is usually asymptomatic, it may have various clinical presentations, including angina, myocardial infarction or sudden death. CASE PRESENTATION: A 32-year-old woman presented with edema of the upper and lower limbs, palpitation, and chest pain, and was diagnosed with a giant right coronary artery aneurysm that had initially mimicked a mediastinal cyst. Although computed tomography (CT) suggested a mediastinal cyst, trans-thoracic echocardiography revealed an extra pericardial cyst. The definitive diagnosis of right coronary artery aneurysm was made based on CT angiography and coronary angiography findings. As treatment, aneurysmectomy was performed, and she was discharged on the sixth postoperative day with good general health condition. CONCLUSIONS: Coronary artery aneurysm should be a differential diagnosis in cases of mediastinal cyst and mass lesion.

Reconstruction of chest wall using a two-layer prolene mesh and bone cement sandwich.

Wide surgical resection is the most effective treatment for the vast majority of chest wall tumors. This study evaluated the clinical success of chest wall reconstruction using a Prolene mesh and bone cement prosthetic sandwich. The records of all patients undergoing chest wall resection and reconstruction were reviewed. Surgical indications, the location and size of the chest wall defect, diaphragm resection, pulmonary performance, postoperative complications, and survival of each patient were recorded. From 1998 to 2008, 43 patients (27 male, 16 female; mean age of 48 years) underwent surgery in our department to treat malignant chest wall tumors: chondrosarcoma (23), osteosarcoma (8), spindle cell sarcoma (6), Ewing's sarcoma (2), and others (4). Nine sternectomies and 34 antero-lateral and postero-lateral chest wall resections were performed. Postoperatively, nine patients experienced respiratory complications, and one patient died because of respiratory failure. The overall 4-year survival rate was 60 %. Chest wall reconstruction using a Prolene mesh and bone cement prosthetic sandwich is a safe and effective surgical procedure for major chest wall defects.

Cervical esophageal perforation: a 10-year clinical experience in north of iran.

Perforations of the cervical esophagus are infrequent severe conditions associated with a high rate of morbidity and mortality if misdiagnosed. The diagnosis and management of cervical esophageal perforation remains a challenging clinical problem. We aimed to present our experience of the etiology, presentation, management and outcome of cervical esophageal perforation in a 10 years period. In this cross-sectional study, we reviewed the records of all patients with a diagnosis of cervical esophageal perforation admitted at the teaching Razi Hospital of Rasht, north of Iran, between 2001 and 2011. 26 patients (15 male) were studied with mean age of 47.6 ± 13.78 years, a range from 10 to 68 years. Only 16 (61.5 %) of patients were referred within 24 h of injury. The etiology was iatrogenic in 15 cases (57.69 %), foreign body ingestion in 7 cases (26.9 %), and penetrating traumatic injury in 4 cases (15.4 %). The common clinical manifestations of perforation were neck pain in 22 cases (84.6 %), fever in 19 cases (73.1 %), and subcutaneous emphysema in 12 cases (46.2 %). Barium and gastrografin swallow were performed in 57.7 and 23.1 % of patients, respectively and flexible esophagoscopy was used in 23.06 %. Most of patients (65.4 %) were managed by primary repair. Overall, mortality rate was 7.7 %. Our study demonstrates that the most common cause of cervical esophageal perforation is iatrogenic injury. Clinical suspicion is most important problem. Furthermore, Diagnosis is mainly made by Barium and gastrografin swallow. For a successful outcome, primary repair is a preferred treatment for most perforation patients.

Classification and Management of Subcutaneous Emphysema: a 10-Year Experience.

Subcutaneous emphysema (SE) is a condition often causing minimal symptoms, but sometimes it can be severe and even life-threatening. This study is the first great survey about SE. The aim of this study is to classify and evaluate the etiology, signs, symptoms, and management of SE. This retrospective study was performed by reviewing patients who had been diagnosed as having SE in Rasht, between January 2001 and January 2011. We classified the severity of SE in five grades including the (1) base of the neck, (2) all of the neck area, (3) subpectoralis major area, (4) chest wall and all of the neck area, and (5) chest wall, neck, orbit, scalp, abdominal wall, upper limbs, and scrotum. We excluded all patients in grades 1 and 2, because the symptoms and signs were not significant. Statistical analysis was carried out with SPSS 18. We collected 35 cases of SE with the mean age of 53 ± 14.83 (71 % men). The most common cause of SE was pneumothorax with background of COPD and surgery in grade 5, trauma due to rib fracture in grade 4, and iatrogenicity in grade 3. We performed two bilateral 2-cm infraclavicular incisions. In our patients with infraclavicular incisions, expansion of the lung was better, and the patients' appearance improved. Infraclavicular incisions as a simple method for the management of SE can decrease the severity of SE with no cosmetic problem.

Posterior mediastinal cyst.

Hydatid disease is still an important health problem in the world. Mediastinal localization of hydatidosis is very rare. We report the case of a 19-year-old man who presented with right chest wall pain and cough. Chest X-Ray and computed tomography showed a mediastinal cystic mass. A right posterolateral thoracotomy was performed and the cystic mass was identified. Daughter cysts were seen within the cyst when the lesion was accidentally opened during the procedure. Cyst mass was radically resected. No complication or recurrence was seen in the follow-up period.

Clinical presentation and operative repair of Morgagni hernia.

OBJECTIVES: Morgagni hernia (MH) is an uncommon type of diaphragmatic hernia. This study aimed to summarize clinically relevant data with respect to MHs in adults. METHODS: We performed a retrospective chart review of patients who underwent surgical repair of foramen due to MH at our hospitals between 1996 and 2010. Data were collected on patient demographics, presenting symptoms, modes of diagnosis, surgical procedures, surgery outcomes, recurrence of hernia and follow-up of the patients. RESULTS: We included 36 patients with the mean age of 50.2 years. Of these 66.7% (n = 24) were female. Thirty-one patients had MH on the right side and 1 patient had bilateral MH. Most of the patients experienced abdominal symptoms. 72.2% of patients underwent laparotomy (n = 26, 72.2%), (n = 6, 16.7%) thoracotomy (n = 6, 16.7%), and a thoraco-abdominal approach (n = 4, 11.1%). Resection of the hernia sac and insertion of a mesh were not done in any patients. No recurrence occurred. CONCLUSIONS: We conclude that preoperative diagnosis and early diagnosis of MH by using laparotomy and thoracotomy is useful for safe and effective repair. Also we suggest that resection of the hernia sac and insertion of a mesh are not necessary.

One stage operation for five giant hydatid cysts of both lungs and liver in a 20-year-old female.

Echinococcosis remains a significant health hazard in endemic areas, including the Middle East, Mediterranean countries and Central Asia. Pulmonary disease appears more commonly in younger individuals but bilateral pulmonary involvement is relatively rare. Concomitant pulmonary and liver hydatid disease may occur in 4% to 25% of patients with hydatidosis. A previously healthy 20-year-old femal from north of Iran without any previous history of lung or liver disease presented with complaints of right upper quadrant and right side chest pain and a low grade fever for the past one month. In imaging studies, one cyst in the left lung, two cysts in the right lung and two cysts in the liver were found. Since the cysts were symptomatic, it was decided to treat these cysts surgically. The patient underwent bilateral antero-lateral thoracotomies and phrenotomy under general anesthesia and all cysts were evacuated. The patient was discharged in good condition.