Low-grade malignant peripheral nerve sheath tumour presenting as retroperitoneal spindle cell neoplasm.

Retroperitoneal spindle cell neoplasms are diagnostically challenging. Malignant peripheral nerve sheath tumours (MPNSTs) can sometimes present as sporadic primary retroperitoneal tumours. MPNSTs are usually high-grade and highly aggressive tumours and are associated with a poor prognosis. Low-grade MPNSTs are very rarely described. This current case report describes a case of sporadic primary low-grade MPNST presenting as retroperitoneal spindle cell neoplasm. The diagnosis, imaging and immunohistopathological findings, as well as its successful surgical management, are presented.

Effect of ABCA1-R219K Polymorphism in Serum Lipid Parameters in Patients under Statin Therapy Visiting Tertiary Cardiac Center of Nepal.

Introduction ATP-binding cassette transporter A1 (ABCA1) encoded by ABCA1 gene is one of the important protein involved in lipid metabolism. The effect of statin therapy on dyslipidemia varies among individuals and it may be due to different genetic polymorphism. The R219K polymorphism of ABCA1 gene is found to have a significant role in the response of statin. Objective This study was designed to evaluate the effect of R219K polymorphism in lipid-lowering action of statin in patients with dyslipidemia. Material and Methods This study was conducted in 88 patients. Blood samples were taken from patients before and at the end of 3 months of statin use and were analyzed for lipid profile. Whole blood was analyzed for R219K Polymorphism using polymerase chain reaction-restriction fragment length polymorphism. Results R219K polymorphism was associated with significant percentage reduction of serum triglyceride/high-density lipoprotein (TG/HDL) ratio and total cholesterol/high-density lipoprotein (TC/HDL) ratio in atorvastatin users. However, there was no significant association of polymorphism with change in serum TC, HDL-C, LDL-C, TG, and very low-density lipoprotein (VLDL). Among KK genotype individuals, value of TG, VLDL, TG/HDL, and TC/HDL were significantly lower than in RR genotypes. Also, TG/HDL and TC/HDL were significantly lower in RK genotype than in RR. Treatment of dyslipidemia with statin was found to be comparatively better in patients having the genotypes KK and RK. Conclusion Our study demonstrated association of R219K polymorphism with the significant reduction of TG/HDL and TC/HDL and particularly the KK genotype was associated with significant improvement of lipid parameters following atorvastatin treatment.

Comparison of Volunteer Non-Remunerated Donors and Replacement Donors.

BACKGROUND: There are mainly two types of blood donor in Nepal i.e., Voluntary Non-Remunerated Donor and Replacement Donor. The main aim of this study is to compare between Voluntary Non-Remunerated Donor and Replacement Donor. METHODS: This is retrospective and cross-sectional study conducted in the blood transfusion Service, Tribhuvan University Teaching Hospital. The study was conducted on both Voluntary Non-Remunerated Donor and Replacement Donor who came to blood transfusion Service and voluntary blood donation program. All the blood donors donating in blood transfusion Service were considered as the study population. RESULTS: Out of total 25951 donors, there were 15565(60.02%) Voluntary Non-Remunerated Donor and 10386(39.97%) Replacement Donor. On the whole, there were 21938(84.59%) male donors and 3995(15.40%) female donors. The male Replacement Donor was 14273(93.12%) followed by a male Voluntary Non-Remunerated Donor 7582(73.71%). The female Voluntary Non-Remunerated Donor was 2703(67.65 %) followed by female Replacement Donor 1292(32.34 %). CONCLUSIONS: This study concludes that the Voluntary Non-Remunerated Donor is quite low, in comparison to Replacement Donor. 100 % Voluntary Non-Remunerated Donor could not be achieved due to lack of awareness about health and voluntary type of blood donation.

Foreign body esophagus: Six years of silence.

Foreign body esophagus remains one of the common medical emergencies which may lead to significant morbidity and mortality. Sharp objects, batteries, and elderly with foreign body esophagus should be treated with emergent removal owing to the complications that might ensue. Endoscopic removal is the preferred choice of treatment but for large foreign body, sharp foreign body, and so on, rigid esophagoscopic removal might be more preferable. Foreign body esophagus though an obvious situation might at times be missed. It is important to make an early definitive diagnosis. We report a unique case of missed foreign body (denture) esophagus despite the obvious signs and symptoms. Definitive diagnosis was made only after 6 years due to the lack of definitive diagnostic procedures and expertise. The foreign body was impacted in the mucosal wall of the esophagus requiring Gastric resection and anastomosis (with McKeown procedure). With this we have tried to highlight the pitfalls in the diagnosis and management of foreign body esophagus. We report a case of a 55-year-old female who presented to the Emergency Room with history of progressive dysphagia and odynophagia for 6 years which was aggravated for the past 6 months. A radiological diagnosis was made. It was followed by a failed attempt of endoscopic removal which warranted the surgical removal of the foreign body.

Buccal oncocytoma: Report of a case and literature review.

INTRODUCTION: More common in major salivary glands, oncocytomas are very rare tumors. They commonly occur in the parotid gland and are painless slow growing predominantly benign tumors. The term "oncocytoma" was introduced by Jaffe to designate those tumors of the salivary glands that consist predominantly of oncocytic cells lining the salivary ducts (1) Similalry, Meza- Chavez had proposed the name "oxyphilic granular cell adenoma. (2) Oncocytomas are extremely rare, benign and slow growing in nature. Here we present a rare case of buccal oncocytoma which is to our knowledge the 19th case of intraoral minor salivary gland tumor and the 7th reported case of buccal oncocytoma. CASE PRESENTATION: Here we present an exceedingly rare case of buccal oncocytoma in a 14 years boy who presented to the department of ENT with right buccal swelling for 6 months. He was posed the diagnosis of buccal cyst after cytological examination supported by CT scan. He then underwent an excisional biopsy where the final diagnosis was made as Buccal Oncocytoma. CONCLUSIONS: Though very rare in the picture, conditions like salivary gland oncocytomas still are reported on and off in the literature. The treatment of which is complete surgical excision.

Vestibular Schwanomma: An Experience in a Developing World.

BACKGROUND: Tumors related to the acoustic nerves represent 90% of cerebellopontine angle diseases and have been in the picture for at least 200 years. Famous as acoustic neuromas and vestibular neuromas, these are usually benign, slow-growing tumors of Schwann cells of the myelin sheath. Surgery is the treatment of choice though some authors have suggested "wait and watch" policy. The aims of our study were to study the clinical presentation and management of the tumors, and to evaluate the perioperative outcomes of the surgery. METHODS: A retrospective review of the datasheet of 33 patients diagnosed with vestibular schwanomma who had undergone surgery from January 2014 to January 2017 was performed in National Academy of Medical Sciences, Bir Hospital, Kathmandu, Nepal. Analysis of the demographic data and perioperative outcomes was performed. RESULTS: Hearing loss was the main presenting symptom in 72% cases followed by tinnitus, dizziness, facial numbness and sudden sensorineural hearing loss. Mean tumur size was 39.7 ± 3 mm. The mean age of the patients was 46 ± 3 years with a female preponderance (1.2:1). In particular, the retrosigmoid route was preferred in all the cases since it was the most employed approach at our center and 63% of the tumors presented to us were grade 5. The surgical techniques allowed safe preservation of the facial function which was 93%. The hearing loss did not improve after the surgery in 94% while it worsened in 6% of cases. We did not find any significant relation between outcome and size, age, gender or laterality of the tumor (P > 0.05). There was no perioperative mortality. CONCLUSIONS: The benign and slow-growing nature of vestibular schwanomma usually poses problems for the early diagnosis and treatment especially in a poor resource setting like ours. Likewise, there are very few studies so far done in the country regarding the incidence and management of the disease. Thus, this study might be helpful in providing insight into the occurrence of the disease in the present scenario and the need for much more studies in the future.