A Rare Case of Primary Malignant Melanoma of Cervical Spine Having Extramedullary Intradural Origin: A Single Case Report and Literature Review.

Introduction: Primary spinal malignant melanoma (PSMM) of extramedullary intradural origin is a rare malignant condition with limited current literature regarding its clinical course, magnetic resonance imaging (MRI) findings, treatment strategies, and outcomes. Case Discussion: This is a case report of a patient with PSMM who was treated with surgery followed by radiotherapy for his residual disease in Shaukat Khanum Memorial Trust, Pakistan. The clinical and radiological findings of this case were retrospectively analyzed using the Hospital Information System. Practical implementations: PSMM of extramedullary intradural origin is a rare malignant tumor that shows characteristic findings on MRI. Surgical resection is the preferred treatment, and radiotherapy is useful for residual disease.

The Use of Trimodality Treatment in Patients With Locally Advanced Oesophageal Squamous Cell Carcinoma: An Experience From a Large Cancer Centre in Pakistan.

Introduction Esophageal cancer is one of the most common cancers worldwide. Neoadjuvant chemoradiotherapy followed by surgery is the standard treatment for locally advanced squamous cell carcinoma (SCC). Pathological complete response (pCR) after surgery is associated with better outcomes in terms of overall survival and disease-free survival. We aim to determine the effectiveness of neoadjuvant chemoradiotherapy in patients with locally advanced SCC at our institute, the largest purpose-built cancer center in Pakistan. We also aim to identify various factors influencing pCR, such as chemotherapy regimen, total radiation dose, clinical stage at presentation, and gender. Materials and methods This is a retrospective review of all patients with esophageal SCC presented between January 2019 and 2021 to the institute for treatment. Patients received neoadjuvant chemoradiotherapy (nCRT) as per the CROSS trial protocol, followed by surgery. We assessed the pCR rate. Statistical analyses were performed using IBM SPSS Statistics for Windows, Version 22 (Released 2013; IBM Corp., Armonk, New York). pCR was studied alongside associated factors such as age, gender, stage of disease, chemotherapy regimen, and total dose of radiotherapy. A p-value of <0.05 was considered statistically significant. The chi-square test was used to compare categorical variables. Univariate and multivariate logistic regression was employed to evaluate factors affecting pCR. Results A total of 218 patients were included in the study. pCR was achieved in 64.2% of the patients. The female gender was associated with better outcomes, as 70.4% (n=81) of female patients achieved a complete pathological response, compared to 57.3% (n=59) of males, with a p-value of 0.03. On univariate analysis, the complete pathological response was 69.6% (n=94) in the age group of 45 years and below, whereas it was 55.4% (n=46) in the age group above 45 years, with a p-value of 0.024. Though statistically insignificant, outcomes were slightly better for those with node-negative disease, as 67.2% (n=41) achieved complete pathological response compared to those with node-positive disease at 63.1% (n=99). Univariate logistic regression analysis identified gender (p=0.044, OR=1.77, 95% CI: 1.016-3.108) and age group (p=0.034, OR=1.844, 95% CI: 1.046-3.252) as significantly associated with pCR. Female patients were 77% more likely to achieve pCR compared to male patients (OR=1.77, 95% CI: 1.016-3.108). Younger patients (≤45 years) were 84.4% more likely to achieve pCR compared to the older age group (OR=1.844, 95% CI: 1.046-3.252). However, these did not maintain significance in multivariate logistic regression analysis. Conclusion Our study indicated a high rate of pCR with nCRT in patients with esophageal SCC compared to other studies. The achievement of pCR was higher among females and younger patients, which was statistically significant on univariate logistic regression analysis. Our study also concluded that a higher dose of RT (50Gy/25#) is not superior to a lower dose (45Gy/25#) in terms of pCR achievement but was statistically insignificant. Similarly, CARBO/PAC was not superior to CIS/CAP in terms of pCR achievement and was also statistically insignificant.

Role Of Palliative Radiotherapy In Improving Performance Status And Quality Of Life In Patients With Metastatic Spinal Cord Compression.

Background: Palliative radiotherapy in patients with metastatic spinal cord compression is a well known treatment modality but little is known whether it improves quality of life and performance status. Our study focusses on analyzing the impact of palliative radiotherapy on these two parameters in patients with metastatic spinal cord compression. Methods: We performed a prospective observational study from August 2020 to April 2021 to assess consecutive patients presenting to emergency department with suspected MSCC. We enrolled 24 patients in the study, and they were evaluated for their performance status and quality of life using ECOG and FACIT PAL 14 scores respectively. Palliative radiotherapy was administered, and the patients were followed up four to six weeks later. Their ECOG and FACIT-PAL 14 scores before and after receiving palliative radiotherapy were analyzed. Results: The mean age of the patients was 48 (IQR 35-62), with 14 (58%) being male. 1 patient died soon after admission. Median dose fractionation was 2000 cGy. Median (IQR) of ECOG performance status score on admission and follow up was 2.5 (1.0-3.7) and 1.5 (1.0-3.7), p=0.719, respectively. Median (IQR) FACIT-PAL 14 score on admission and follow up were 35.5 (34.0-37.6) and 36.5 (30.2-44.7), p=0.277, respectively. Our results indicate that there was no statistically significant difference in the median ECOG performance status and FACIT PAL 14 scores before and after the administration of palliative radiotherapy. Conclusion: Our study indicates that palliative radiotherapy in patients with metastatic spinal cord compression had little benefit in objectively improving quality of life and performance status using the well-known and widely used scores. This lack of response could be due to delayed presentation of the patients. Earlier involvement of palliative care team could have improved both these parameters. Further research with larger population of patients over a longer period is needed to further assess these outcomes.

Central Pancreatectomy for Solid Pseudopapillary Tumour of Pancreas in A 13-Year Girl.

Solid Pseudopapillary Tumour (SPT) is a very rare tumour of the pancreas. A 13-year girl presented to us with the complaint of upper abdominal pain and non-bilious vomiting for 15 days. Preoperative diagnosis of SPT involving the body of the pancreas was made by CT scan and ultrasound- guided Trucut biopsy. A sparingly rare procedure of central pancreatectomy with distal pancreatico-jejunostomy was performed. This procedure offers excellent results in benign and low-grade malignant pancreatic neck and body tumours. In addition, it preserves functional elements of the pancreas and also eliminates the infective and haematological effects of splenectomy. Key Words: Solid pseudopapillary tumour, Central pancreatectomy, Pancreatic tumours.

Small cell neuroendocrine carcinoma of nose and paranasal sinuses: The Shaukat Khanum Memorial Cancer Hospital experience and review of literature.

Small cell neuroendocrine carcinoma (SCNEC) is a rare disease of nose and paranasal sinuses. In contrast to other regions SCNEC of this region has been reported to be recurrent and locally aggressive. No definite treatment has been established till date because of rarity of this disease. The purpose of this descriptive study is to present the series of 8 cases with SNEC of nose and paranasal sinuses. Retrospective review of 8 patients presenting with Small cell neuroendocrine carcinoma of nose and paranasal sinuses, from January, 2005 to December, 2014 treated at Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore was performed to determine the clinical characteristics and outcome of this disease. The subjects were 7 males and 1 female with a mean age of 45±15 years (range 24 to 59 years). Tumours were present in nasal cavity (n=4), ethmoid sinus (n=3) and maxillary sinus (n=1). Almost 50% of patients presented with AJCC stage IV-A. All patients had immunohistochemistry proven diagnosis. All patients (08) received radiotherapy with mean doses 58±Gy (Range 54-66Gy). Surgery was performed in 2 while chemotherapy was administered in 4 patients. Recurrence occurred in 3 patients, one each with loco-regional, distant and both. At a median follow up of 38 months, 5 patients were alive with no evidence of disease. SCNEC is a rare but aggressive neoplasm. Current standard of care varies but multi-modality approach should be the cornerstone in management of SCNEC. Early diagnosis and intervention improve the final outcome.

Retrospective study of rare cutaneous malignant adnexal tumors of the head and neck in a tertiary care cancer hospital: a case series.

BACKGROUND: Adnexal tumors of the skin are a large and diverse group of benign and malignant neoplasms, which exhibit morphological differentiation toward one of the different types of adnexal epithelium present in normal skin and they pose a diagnostic challenge. The purpose of this study is to share our experience with these rare but aggressive tumors at a tertiary care cancer hospital in a developing country. A retrospective review of 11 patients diagnosed with rare adnexal tumors and their variants from January 2005 to December 2014, treated either surgically or non-surgically, was performed to describe the clinicopathological characteristics and outcome of the disease. CASE PRESENTATION: A total of 11 patients were diagnosed with adnexal carcinoma and its variants: a 34-year-old Sindhi man, a 59-year-old Punjabi woman, a 32-year-old woman from Khyber Pakhtunkhwa, a 43-year-old Punjabi woman, a 64-year-old Punjabi man, a 51-year-old man from Khyber Pakhtunkhwa, a 51-year-old Punjabi woman, a 74-year-old Punjabi woman, a 75-year-old Punjabi man, a 61-year-old man from Khyber Pakhtunkhwa, and a 53-year-old man from Khyber Pakhtunkhwa. The male to female ratio was 1.2:1. The histological variations were sebaceous differentiation (n = 4), microcystic adnexal carcinoma (n = 4), trichilemmal carcinoma (n = 1), pilomatrix carcinoma (n = 1), and hidradenocarcinoma (n = 1). The mean age at presentation was 54 years (range 32 to 75). The primary subsite of involvement was the scalp in nine patients followed by eyelids in two patients. Surgery was the primary treatment modality in almost all patients; postoperative radiotherapy (PORT) was offered to eight patients. The median dose of radiation was 45 Gy to the primary site. Indications for radiotherapy included close margins (n = 2), positive margins (n = 1), high grade histology (n = 4), and multifocal disease (n = 1). On follow-up, two patients presented with local, one regional and two patients developed distant metastasis. CONCLUSIONS: Adnexal carcinomas are rare tumors with diverse histological patterns and a tendency for locoregional and distant metastasis. Surgery should be the mainstay of treatment reserving radiotherapy for adjuvant, palliative, and re-treatment scenarios.

Pulmonary renal syndrome in a patient with vasculitis: Case report and review of literature.

Granulomatosis with polyangiitis (GPA) previously known as Wegner's granulomatosis, is a small vessel vasculitis that preferentially involves capillaries, arterioles and venules, presenting as multisystemic disease classically with alveolar haemorrhage and renal insufficiency. We report a case of GPA diagnosed on history, clinical findings and supported by imaging and very high levels of C-ANCA. Renal biopsy confirmed the typical histopathological findings. We discuss herein the management of the case and review of literature.

Surgical treatment in lumbar spondylolisthesis: experience with 45 patients.

BACKGROUND: Spondylolithesis is forward slipping of upper vertebra in relation to its lower one, which at times requires surgery. The objective of present study is to document the outcome of surgical treatment in spondylolisthesis of lumbosacral region. METHODS: We reviewed outcome of surgery in 45 patients with spondylolisthesis. Improvement in pain intensity, neurological status and union achieved after surgery was studied. All patients requiring surgical treatment were included in the study. The patients were operated by single spine surgeon. A Performa was made for each patient and records were kept in a custom built Microsoft access database. RESULTS: Majority of our patient were in 4th and 51th decade with some male domination. Pain was main indication for surgery which was excruciating in 6. severe in 33, and moderate in 6 cases. The neurological status was normal in 34 cases while 11 patients had some deficit. L5-S1 was affected in 26, L4-L5 in 13 and multi or high level was found in rest of cases. Slip grade was measured with Meyerding grades, 18 had grade II, 15 had I, 9 had III and 3 had IV spondylolisthesis. Posterior lumbar inter body fusion (PLIF) was done in 24 patients, posterolateral, transforaminal lumbar inter body and anterior inter body fusion in others. Translaminar screw fixation, transpedicular transdiscal transcorporial and Delta fixation in some cases. Pedicle screw fixation was done in most cases, AO fixator internae and 4.5 mm screw in others. Average follow up was 2 years and 5 months, max 5 years and minimum 6 months. Pain relief was achieved in 82%, neurological improvement 60% and union in 91% cases. There was no deterioration of neurological status, two implant failure and one wound infection. CONCLUSION: Surgical procedure for Spondylolisthesis must be individualised. Young patients with spondylolysis can be treated with osteosynthesis and sparing of motion segment. PLIF provides satisfactory results in majority of low to moderate cases with some reduction. Transpedicular transdiscal transcorprial and delta fixation is good procedure for severe slips in adult.

Helicobacter pylori infection in patients with calcular cholecystitis: a hospital based study.

BACKGROUND: Helicobacter pylori, a gram negative bacillus has been recognised as a public health problem and approximately half of the world population has H. pylori infection causes chronic gastritis, peptic ulcer disease and gastric malignancies. Objective of this study was to determine the frequency of H. pylori infection in patients of chronic calcular cholecystitis. METHODS: This cross-sectional descriptive study was conducted at Liaquat University Hospital, Hyderabad, Pakistan from April 2010 to September 2010. All patients with history of gallstone presented with acute abdominal pain, dyspepsia, bloating and epigastric discomfort and diagnosed as calcular cholecystitis were further evaluated for the detection of H. pylori by serology and histopathology. Frequency and percentage of H. pylori infection in patients with calcular cholecystitis was calculated. RESULT: Total 100 patients of cholelithiasis underwent laparoscopic cholecystectomy were recruited. The pain in upper right part of the abdomen was observed in all 100 patients, fever in 75%, nausea and vomiting in 68%, loss of appetite in 45%, feeling of tiredness or weakness in 22%, headache in 38%, chills in 52%, backache in 58%, pain under the right shoulder in 45%, heartburn in 67%, belching in 54%, indigestion in 80%, dyspepsia in 90%, bloating in 88%, and epigastric discomfort in 85% patients. Eighty-two percent patients had family history of gallstones. The mean age of overall study population was 48.72 +/- 8.78 years and mean age of H. pylori infected calcular cholecystitis patients was 47.98 +/- 5.43 years in male and 48.76 +/- 6.68 years in females. The H. pylori infection was identified in 55% patients with calcular cholecystitis, of which 32.7% were males and 67.3% were females (p=0.03, statistically significant). Majority of females (60%) had > or = 40 U/ml antibody titre (p=0.917, non-significant). CONCLUSION: A possible relationship was identified between Helicobacter pylori and calcular cholecystitis.

Surgical management of tuberculous small bowel obstruction.

BACKGROUND: Acute intestinal obstruction due to tuberculosis is a common surgical problem in our community. Emergency surgery is usually required and surgical procedure depends upon the location and extent of the disease. The aim of this study was to determine the commonly involved region of intestine and different surgical procedures tailored. METHODS: Thirty patients operated upon for acute intestinal obstruction in emergency with operative and histopathological findings suggestive of tuberculosis were included in the study. Demographic profile, operative findings, details of surgical procedure, complications and post-op hospital stay were recorded. The patients were followed for 6 months. RESULTS: Intestinal tuberculosis is more common in young female, with male to female ratio of 1:1.5. Stricture of the small bowel was found in 50% of the cases. The next common finding was ileocaecal tuberculosis found in 40% of patients. Strictruplasty was performed in 11 (36.33%). The right hemicolectomy, limited ileocaecal resection and segmental bowel resection with end to end anastomosis were performed in four patients each. Other procedures were release of adhesions and bands in 4 patients, ileotransverse bypass in 1 patient and loop ileiostomy in 2 patients. Major complication in 10 patient and mortality rate was 10%. CONCLUSION: Because of non-specific clinical features, ignorance and malpractice intestinal tuberculosis presents late. Ileocecal tuberculosis is becoming less common as compared to small bowel strictures. Less radical surgery gives better results. Post operative complications and mortality are related to the perforation of the intestine at the time of surgery.

Total thyroidectomy as primary elective procedure in multinodular thyroid disease.

BACKGROUND: Multinodular goitre is one of the commonest thyroid diseases encountered in the practice of surgery. The most common surgery being performed for multinodular goitre is subtotal thyroidectomy. Total thyroidectomy is designed to remove all of the thyroid tissue. The objective of this study was to evaluate total thyroidectomy as a primary elective procedure for treatment of multinodular thyroid disease. This descriptive study was carried out at Combined Military Hospital Rawalpindi from June 2003 to September 2006. METHODS: 88 patients of multinodular thyroid disease were included. Patients having evidence of recurrent laryngeal nerve damage, recurrent goitre, evidence of altered parathyroid functions or evidence of malignancy were excluded. All patients underwent total thyroidectomy by the same team of surgeons and the patients were closely followed up for postoperative complications especially in terms of recurrent laryngeal nerve damage and hypocalcaemic tetany. RESULTS: No major postoperative complication was noted. Only 1 patient (1.14%) developed unilateral recurrent laryngeal nerve damage and 2 patients (2.27%) developed transient hypocalcaemia that recovered quickly. CONCLUSION: Total thyroidectomy as a primary elective procedure in multinodular thyroid disease is a safe option and it removes the disease process completely, lowers local recurrence rates and avoids the substantial risks of reoperative surgery.