Aggressive embryonal rhabdomyosarcoma presenting as schwanoma : a case report

Rhabdomyosarcomas (RMS) are thought to arise from pluripotent muscle cell precursors intended to form the bodyís future striated muscle. Due to the wide distribution of striated muscle across the body these tumors may appear anywhere and cause symptoms accordingly. Presenting symptoms are highly variable and based on the tumor size and location making clinical diagnosis difficult. The diagnosis of RMS is based on histological examination; however they express varying cytologic pictures representing the different stages of rhabdomyoblasts and their development into skeletal muscle presenting pictures from highly to poorly differentiated neoplasms. Therefore their histological diagnosis also requires the use of specialized stains and complex staining procedures which may not be available at peripheral centers. Here we would like to present a highly aggressive embryonal rhabdomyosarcoma of the maxilla, presenting as a schwanoma on initial histopathology

Neuronavigation assisted endoscopic sinus surgery

In the past two decades, endoscopic sinus surgeries (ESS) have been widely advocated as a safe and effective treatment for disorders of paranasal sinuses that are refractory to medical therapy. ESS caters surgeons with two-dimensional visualization of the anatomical structures; however in scenarios where there is a close anatomical relation between the disease and delicate intracranial or intraorbital structures drastic complications can occur. Hence, endoscopic procedures had to be converted into open surgical procedures if the extent of the disease could not be visualized or cleared thoroughly. Recently however; neuronavigation systems have been combined with ESS to yield better results and facilitate this procedure. The implementation of these systems with ESS has aided surgeons in difficult approaches. We recently decided to use the neuronavigation system of our Neurosurgical department to help aid eradicate various nasal and sinus pathologies in a series of patients

Long-tunneled versus short-tunneled external ventricular drainage: Prospective experience from a developing country

Background: External ventricular drains (EVD) are commonly utilized for temporary diversion of cerebrospinal fluid (CSF). Many neurosurgeons prefer long-tunneled EVDs in their routine practice. However, it is still unclear whether this extended tunneling helps in reducing CSF infection. Keeping this in mind, we decided to compare infection rates in long-tunneled versus short-tunneled EVDs in the setting of a developing country.Materials and methods: A prospective study of 60 patients was conducted. Consenting patients who underwent short-tunneled (Group A) or long-tunneled (Group B) EVDs between January 2008 and June 2009 were followed during the course of their inpatient care. All operational protocol was standardized during the trial. Serial samples of CSF were analyzed to detect infection.Results: Mean age of patients was 33.6 years with 32 males (53.3%). Mean duration of long-tunneled EVD was 13.4 ± 7.2 days, whereas that of short-tunneled EVD was 5.3 ± 2.7 days (P \u3c 0.001). Three patients with long-tunneled EVD (10.0%), whereas one patient with short-tunneled EVD (3.3%) developed drain-related infections; however, this was non-significant (P = 0.301). However, patients with short-tunneled EVD got infected earlier on day 3when compared with the long-tunneled EVDs, which got infected after a mean duration of 7.3 days. The overall risk of infection for long-tunneled EVDs was 7.46 per 1,000 ventricular drainage days which was comparable to the risk of 6.33 per 1,000 ventricular drainage days seen for short-tunneled EVDs.Conclusion: Long-tunneled EVDs appear to only delay potential infections without having any effect on the actual risk of infection. Long-tunneled EVD in a resource-limited setting is technically challenging and may not yield additional benefits to the patient. However, larger and prospective studies are needed to establish the rate of infections and other complications

Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan

OBJECTIVE: To determine the major endocrine complications present in patients of Thalassemia major presenting to a tertiary care center in Karachi, Pakistan. METHODS: Records of all thallasemia major patients at a Haematology Department in a tertiary care hospital of Karachi were retrospectively reviewed from May to August 2009 with specific focus on endocrine data and investigations. The patients\u27 data was recorded in a questionnaire and analyzed using SPSS v.17, frequencies and percentages along with corresponding 95% confidence interval were computed. RESULTS: Our data revealed that a significantly small percentage of those under care received regular endocrine follow-up. Male hypo-gonadal abnormalities had the highest probability; 100% of the tested patients had decreased levels of testosterone, while 95.2% had raised serum creatinine levels. Parathyroid dysfunction was noted in 40% of the patients. Of those screened 29.4% had blood glucose levels in the diabetic range and 11.8% of the tested patients had reduced levels of FT4. CONCLUSION: A high frequency at endocrine dysfunctions seen in thalasaemia patients included: male hypogonadism, parathyroid dysfunction, deranged blood glucose and FT4 levies

Causes of smoking in Pakistan: an analysis of social factors

OBJECTIVE: To determine the factors contributing to the initiation and propagation of smoking in visitors to a major tertiary health center in Karachi, Pakistan. METHODS: Seven major contributing factors to the initiation and propagation of smoking were presented to consenting study participants (n=170) in a questionnaire. Participants were then requested to use their experience and opinion to rate each of the given factors on a scale of 1 to 5 regarding its importance as a causative factor in the initiation and propagation of smoking. Results were analyzed using SPSSv16.0. RESULTS: Preliminary analysis revealed occupational stress relief as the most important factor contributing to smoking with a mean score of 3.25 +/- 1.32. Peer pressure ranked second (Score 3.20 +/- 1.42). Domestic stress relief ranked third with a score of 3.19 +/- 1.32. Smokers gave lower rating than non-smokers to most factors. Younger participants gave higher ratings to peer pressure, and most participants were found to have begun smoking at a young age. CONCLUSIONS: Even though the addictive power of nicotine or stress may appear as a factor in middle aged smokers, the root of their habit lies in the initiation due to peer pressure

Successful management of aggressive fibromatosis of the neck using wide surgical excision: a case report

<p>Abstract</p> <p>Introduction</p> <p>Aggressive fibromatosis is a benign tumor, thought to arise from deep musculoaponeurotic structures, rarely found in the head or neck. However, when it does occur in the head and neck region, it tends to be more aggressive and associated with significant morbidity, which may be attributed to the vital vascular, neurological or anatomical structures in close proximity.</p> <p>Case presentation</p> <p>We report the case of a 39-year-old Pakistani man who presented with a two-month history of a lump on the right side of his neck. The mass was excised and histopathological analysis revealed a case of aggressive fibromatosis.</p> <p>Conclusion</p> <p>Due to the rarity of the condition no guidelines are available on the indications and extent of each modality. Due to its aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. On the basis of this case, we suggest that aggressive surgery is a viable management option and may be successfully used as a single modality treatment.</p

A comparative analysis of toluidine blue with frozen section in oral squamous cell carcinoma

Background:Surgical excision of the primary tumor with safe margins remains the mainstay of treatment for oral cavity squamous cell carcinoma (OSCC). The standard of care for assessment of intraoperative margins is frozen section histopathology. Unfortunately the facility is not available at most centers in limited resource countries. Toluidine blue, a metachromatic dye, has been well described in clinical identification of malignant and premalignant lesion in the oral cavity. Considering this we decided to explore intraoperative use of toluidine blue staining, in comparison with frozen sections, for the assessment of tumor-free margins. Methods: After obtaining clearance from the in-house ethical review committee, a prospective study was conducted at Aga Khan University Hospital, Karachi, from August 15, 2009 to March 14, 2010. A sample of 56 consenting Patients with biopsy-proven OSCC were included in the study, giving us 280 tumor margins. Margins were analyzed using toluidine blue staining and frozen section histopathology. A receiver operator curve (ROC) was then applied to compare assessment of margin status by toluidine blue and frozen section. Results: Of the 280 examined margins 11 stained positive with toluidine blue, three were positive on frozen section biopsy, and three were positive on final histopathology. Toluidine blue staining had sensitivity and specificity of 100% and 97%, respectively. The diagnostic accuracy of toluidine blue was found to be 97.1% with a positive predictive value (PPV) of 27.2% and a negative predictive value (NPV) of 100%. Conclusions: Toluidine blue can be used as an effective screening modality for the assessment of intraoperative margins in resource limited environments and reducing the number of frozen section biopsies performed. Further by providing real-time clinical information within minutes it can reduce indirect costs such as operating room time. It may also be used as an ad hoc for frozen section biopsies where frozen section facilities are available