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Background Solitary Fibrous Tumor (SFT) is a distinct soft tissue neoplasm associated with NAB2-STAT6 gene fusion. It can involve a number of anatomic sites and exhibits a wide spectrum of histological features. Main body Apart from diversity in morphological features seen even in conventional SFT, two histologic variants (fat-forming and giant cell-rich) are also recognized. In addition, a malignant form and dedifferentiation are well recognized. Owing to diverse histological features and involvement of diverse anatomic locations, SFT can mimic other soft tissue neoplasms of different lineages including schwannoma, spindle cell lipoma, dermatofibrosarcoma protuberans, liposarcoma, gastrointestinal stromal tumor (GIST), malignant peripheral nerve sheath tumor (MPNST), and synovial sarcoma. SFT is classified as an intermediate (rarely metastasizing) tumor according to World Health Organization Classification of Tumors of Soft tissue and Bone, 5th edition. The management and prognosis of SFT differs from its malignant mimics and correct diagnosis is therefore important. Although SFT expresses a distinct immunohistochemical (IHC) profile, the classic histomorphological and IHC profile is not seen in all cases and diagnosis can be challenging. NAB2-STAT6 gene fusion has recently emerged as a sensitive and specific molecular marker and its IHC surrogate marker signal transducer and activator of transcription 6 (STAT6) has also shown significant sensitivity and specificity. However, few recent studies have reported STAT6 expression in other soft tissue neoplasms. Conclusion This review will focus on describing the diversity of histological features of SFT, differential diagnoses and discussing the features helpful in distinguishing SFT from its histological mimics.

Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control. Results In our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up. Conclusion Owing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma.

Objectives: Congenital heart defects (CHDs) affect more than 40,000 children annually in Pakistan. Approximately 80% of patients require at least one surgical intervention to achieve a complete or palliative cardiac repair. The Glenn shunt, a palliative procedure is established between superior vena cava (SVC) and the right pulmonary artery to provide an anastomosis offering minimal risk to patients with univentricular heart disease. The aim of this study was to assess the clinical outcomes of the Glenn shunt procedure in patients with complex congenital heart diseases in a developing country like Pakistan. Materials and Methods: A retrospective chart review was conducted on patients who underwent a bidirectional Glenn shunt procedure from July 2006 to June 2017. Data were collected on a structured questionnaire and analyses performed on SPSS version 22. Frequencies and percentages were computed for categorical variables while mean and standard deviation for continuous variables where appropriate. Results: A total of 79 patients underwent the Glenn shunt procedures. The median age was 1.9 years and 54.5% were male. Tricuspid atresia was the primary diagnosis in 30.4% of the patients. Common morbidities included arrhythmias (6.3%), pleural effusion (8.9%), wound infection (3.8%), pneumonia (2.5%), and seizures (3.8%); reopening was required in 2.5% of the patients and 8.8% were readmitted within 30 days of index operation. There were three (3.8%) deaths in total. Conclusions: Bidirectional Glenn shunt procedure can be performed safely in patients with ideal characteristics as the first stage palliation and has favorable results with acceptable rate of complications.

Background. Diagnosis of dedifferentiated liposarcoma (DDL) can sometimes be challenging due to a wide variety of histological features. “Meningothelial-like” whorl is an uncommon histological feature of DDL, which is also observed in neural tumors and follicular dendritic cell sarcoma. This feature is frequently associated with metaplastic bone formation. We conducted this study to describe the clinicopathological features of DDL with meningothelial-like whorls that would aid in establishing accurate diagnosis. Material and Methods. Microscopic glass slides of 5 cases of DDL with meningothelial-like whorls, diagnosed between January 2010 and December 2019, were reviewed. Results. Paratesticular region was the most common site. Whorls occupied 10% to 75% of tumor area and ranged in size from 2 cm. In 1 case, these whorls coalesced to form large areas of dedifferentiation. The cells forming whorls were spindle to epithelioid shaped and lacked significant nuclear pleomorphism and increased mitoses. Metaplastic bone formation was observed in 4 cases and cartilage formation in 3 cases. p16 and α-smooth muscle actin (α-SMA) immunohistochemical stains were positive in 2 cases, when performed. MDM2 gene amplification was observed in all cases by fluorescence in situ hybridization technique. These tumors showed aggressive behavior, similar to that of DDL without meningothelial-like whorls. Two patients died, 1 developed recurrence, 1 presented as recurrent tumor, and 1 developed metastasis. Conclusion. Meningothelial-like whorls in DDL most likely represent an early stage of dedifferentiation. Presence of well-differentiated liposarcoma areas, metaplastic bone formation, positive expressions for p16 and α-SMA immunohistochemical stains, and MDM2 gene amplification are useful diagnostic clues. These tumors have the potential to behave aggressively.

To study the frequency of the thigh, hip and groin soft tissue sarcomas and retrospectively analyse the management, treatment results, and outcomes of these uncommon malignant tumours, in a tertiary care hospital of the city of Karachi.Data of soft tissue tumours registered from 2017-2018 was retrieved during January 2019 to March 2019 from Aga Khan University Hospital, Karachi bone and soft tissue tumour registry. A retrospective review was performed and all soft tissue tumour cases treated with surgical intervention (with adjuvant /neoadjuvant therapy) or palliative intention were included.Total 119 cases of soft tissue tumours (STS) were identified out of which 85 were malignant cases (sarcomas) while 30 were benign. On presentation 84 (70.6%) were primary cases. On topographical distribution, there were 25 patients who had hip, groin and thigh sarcoma. Of these, 15 were males and 10 were females. As treatment, neo-adjuvant radiation was done in 4 (16%) patients and adjuvant chemo/radio therapy was given to 13 (52%) patients. Wide margin excision was performed in 19 (76%) patients and 4 (16%) had amputation. Reconstruction was offered to 3 (12%) patients. In post-surgical complications, 1 (4%) patient had wound infection. On final surgical histopathology, majority of the sarcomas were liposarcomas, myxofibrosarcoma, synovial sarcoma and Leiomyosarcoma. Post-surgery recurrence occurred in 7 (28%) patients. Overall survival was 76%.In treatment of soft tissue sarcoma, limb salvage is an achievable option and survival results are also good.

A 21-year-old man presented with chest pain, diaphoresis and dyspnoea. Electrocardiogram (ECG) showed inferior ST-elevation myocardial infarction. Troponin I was positive. Patient underwent left heart catheterisation, which revealed normal epicardial coronary arteries except for right coronary artery which could not be engaged. CT coronary angiogram was done, which revealed large right sinus of Valsalva aneurysm giving rise to a pinched out right coronary artery. Patient underwent composite graft replacement of aortic valve, aortic root and ascending aorta along with a saphenous vein graft to right coronary artery. He was discharged in stable condition on fifth postoperative day. Biopsy of the aneurysmal tissue was suggestive of vasculitic aetiology. There was no evidence of systemic vasculitis and Magnetic Resonance Angiography (MRA) screen was negative for Takayasu’s arteritis. Our patient was, hence, diagnosed with clinically isolated aortitis leading to ST elevation myocardial infarction due to an unruptured sinus of Valsalva aneurysm.

Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

Solid papillary carcinoma (SPC) is an uncommon breast tumor whose prognosis depends on invasive component. We studied clinicopathological features of SPC by reviewing 65 cases. Invasive component was seen in 75.4% cases. Almost all tumors with grade III nuclei had invasive component. Mean patients' age of invasive tumors was significantly higher than that of non-invasive tumors (P = .036). All patients were alive and disease free except for a single patient who developed distant metastasis and died of disease. SPC have excellent clinical course. Careful search for invasive component is mandatory, especially in tumors with older patient's age and higher nuclear grade.

Objective The discrepancy between admission and discharge diagnosis can lead to possible adverse patient outcomes. There are gaps in integrated studies, and less is understood about its characteristics and effects. Therefore, this study was conducted to determine the frequency, characteristics, and outcomes of diagnostic discrepancies at admission and discharge. Design and data sources This retrospective study reviewed the admitting and discharge diagnoses of adult patients admitted at Aga Khan University Hospital (AKUH), Internal Medicine Department between October 2018 and February 2019. The frequency and outcomes of discrepancies in patient diagnoses were noted among Emergency Department (ED) physician versus admitting physician, admitting physician versus discharge physician, and ED physician versus discharge physician for the full match, partial match, and mismatch diagnoses. The studied outcomes included interdepartmental transfer, Intensive Care Unit (ICU) transfer, in-hospital mortality, readmission within 30 days, and the length of stay. For simplicity, we only analyzed the factors for the discrepancy among ED physicians and discharge physicians. Results Out of 537 admissions, there were 25.3–27.2% admissions with full match diagnoses while 18.6–19.4% and 45.3–47.9% had mismatch and partial match diagnoses respectively. The discrepancy resulted in an increased number of interdepartmental transfers (5–5.8%), ICU transfers (5.6–8.7%), in-hospital mortality (8–11%), and readmissions within 30 days in ED (14.4%-16.7%). A statistically significant difference was observed for the ward’s length of stay with the most prolonged stay in partially matched diagnoses (6.3 ± 5.4 days). Among all the factors that were evaluated for the diagnostic discrepancy, older age, multi-morbidities, level of trainee clerking the patient, review by ED faculty, incomplete history, and delay in investigations at ED were associated with significant discrepant diagnoses. Conclusions Diagnostic discrepancies are a relevant and significant healthcare problem. Fixed patient or physician characteristics do not readily predict diagnostic discrepancies. To reduce the diagnostic discrepancy, emphasis should be given to good history taking and thorough physical examination. Patients with older age and multi-morbidity should receive significant consideration.

Atypical/anaplastic meningiomas are prone to aggressive behaviour which affects treatment planning and prognostication. Our aim was to assess the role of Apparent Diffusion Coefficient (ADC) values of MRI brain in differentiating typical from atypical/anaplastic meningioma. We reviewed 84 typical and 37 atypical/anaplastic meningiomas and compared mean ADC values and ADC ratios of their preoperative MRI brain. At 3 Tesla, mean ADC value for typical meningioma was1.03±0.10x10-3 and 0.63±0.05x10-3 for atypical/anaplastic meningioma. At 1.5 Tesla, mean ADC value for typical meningioma was 1.05±0.11x10-3 and atypical/ anaplastic meningioma was 0.70 ± 0.04x10-3. The mean ADC ratios were 1.08 ± 0.17 and 0.85 ± 0.15 for typical and atypical/anaplastic meningomas respectively. Mean ADC ratios and the mean ADC values of typical and atypical/anaplastic meningiomas were significantly different (p0.001). ADC values and ADC ratios have important role in differentiating typical from atypical/anaplastic meningioma and it must be part of the routine preoperative MRI reporting.

Enterobius vermicularis (EV) is a pinworm which commonly resides in the lumen of the intestinal tract and lays eggs on the perianal skin. However, rarely the worm can infest various other sites in the body and cases with infestation of such ectopic sites have been reported in literature. Rare cases of mesenteric lymph node involvement have also been reported. We report a case in a young male who presented with signs and symptoms of acute appendicitis. During surgery, enlarged mesenteric lymph nodes were identified. Histological examination revealed adult worm in the appendiceal lumen. Histological examination of mesenteric lymph node revealed degenerated worm surrounded by caseating chronic granulomatous inflammation. We conclude that EV infestation should be considered in the differential diagnosis of enlarged mesenteric lymph node with chronic granulomatous inflammation, especially in young patients and when accompanying bowel tissue also reveal the helminth.

Objective Lipoblastoma is a rare neoplasm that occurs mostly in infants and children. Although benign, it has a tendency for local recurrence. Results Clinical and pathological features of 23 cases of lipoblastoma described. Patients’ age ranged from 8 months to 18 years with mean and median age 4.1 and 2.5 years, respectively. Male:female ratio was 2.8:1. Most common sites were lower extremities (9 cases), followed by abdominal cavity and retroperitoneum (4 cases), and scrotum/groin (3 cases). Grossly, 22 tumors were well circumscribed and multi nodular. All cases showed lobules composed of adipocytes and lipoblasts with intervening fibrous septa and fine vascular network. Myxoid change, capsule formation and septation were seen in all cases. Zonation was seen in 2 cases. Follow-up was available in 14 out of 23 patients. Of these, 13 were alive and free of disease with no evidence of any recurrent lesion. One patient with a mediastinal infiltrating lipoblastoma experienced 4 recurrences. Lipoblastoma is a benign adipocytic neoplasm of infants and young children. Correlation of clinical and histological features helps in reaching a correct diagnosis. Owing to a high recurrence rate following incomplete resection, a complete resection is essential. Prognosis is excellent after complete resection. Electronic supplementary material The online version of this article (10.1186/s13104-018-3153-8) contains supplementary material, which is available to authorized users.

Anorectal malignant melanomas (AMMs) are aggressive neoplasms, which account for less than 1% of all anorectal tumors. Anorectal malignant melanomas are notorious for their diversity of histologic features and mimic a number of other tumors. Aberrant expression of immunohistochemical stains such as cytokeratins and CD117 (c-kit) further increases the risk of misdiagnosis. Aim of our study was to describe the common as well as unusual architectural and cytologic features that create difficulty in diagnosis. We also discussed the role of immunohistochemical stains in diagnosis of AMMs. We retrieved and reviewed 61 cases of anal melanoma diagnosed in our institution between January 2005 and May 2014. Epithelioid cell type was observed in 57 (93.4%) cases, spindle cells in 35 (57.4%) cases, pleomorphic in 12 (19.7%) cases, and lymphoma-like in 2 (3.3%) cases. Cytoplasmic clearing was observed in 16.4% and nuclear pseudoinclusions in 9.8% cases. Twenty-one point three percent cases were completely amelanotic, and 36.1% showed focal melanin pigment. Average mitotic count was 2 mitoses/high-power fields. Nesting pattern was seen in 24.6%, pseudoalveolar pattern in 11.5%, and peritheliomatous/pseudopapillary pattern in 5% cases. Positive expression of vimentin, S-100, HMB-45, and Melan A was seen in 100%, 100%, 94.4%, and 93.3% cases, respectively. Cytokeratins were positive in 9% and CD117 (c-kit) in 20% of cases in which they were performed. In conclusion, AMMs should be considered in the differential of any malignant tumor of anorectal region without obvious glandular and squamoid differentiation. The knowledge of amelanotic nature, unusual histologic features, and aberrant immunohistochemical expression is helpful in avoiding misdiagnosis.

Hairy polyps (HPs), dermoids or teratoid tumors are rare tumors of naso-oropharyngeal region which commonly present at or shortly after birth. The etiology and classification of these tumors is still debatable and categorized by different authors differently. HPs have female predominance and usually present with respiratory and feeding problems. Microscopically, the polyp is covered by skin with underlying mesenchymal core. The aim of this study is to describe the clinicopathological features of Hairy polyps on a cohort of cases. We reviewed the surgical pathology database of our institution for last 10 years and retrieved four cases of hairy polyps. The age of patients ranged from 1 month to 18 years (mean = 12 years), with a female to male ratio of 1:3. Two of our cases presented at birth and two cases in late teens. Two of the HPs were located in nasopharynx, one on soft and hard palate and one on lower lip. One case was associated with bifurcation of tongue. Size of the polyps ranged from 2.3 to 4.5 cm (mean = 3 cm). Histologically, all HPs were lined by skin and the underlying core consisted of adnexal structures, adipocytes, skeletal muscle, smooth muscle and seromucinous glands. Lymphoid aggregates, cartilage and bone were seen in one case each. Our series highlights the diverse nature of this entity in terms of age of presentation and location. HP at lower lip and associated bifurcation of tongue has not been previously reported. We observed a male predominance in contrast to the published literature. However, number of cases is too few to read a definite conclusion on this point. The etiology is still controversial and includes congenital malformation and activation of pluripotent stem cells.

Multiple myeloma is a clonal B-cell malignancy, characterised by proliferation of plasma cells and secretion of paraproteins. These plasma cells accumulate predominantly in the bone marrow; rarely, they invade other areas, especially the thorax. Myeloma presenting with a pleural effusion is rare and reported in only 6% of patients with myeloma. Such patients generally present late and have a poor prognosis. Here, we describe a patient presenting with a lung mass, renal failure and a massive unilateral pleural effusion due to multiple myeloma who was treated successfully.

Background Procedural skills training forms an essential, yet difficult to assess, component of an Internal Medicine Residency Program. We report the development of process of documentation and assessment of procedural skills training. Method An explanatory sequential mixed methods design was adopted where both quantitative and qualitative information was collected sequentially. A survey was conducted within the Department of Internal Medicine at The Aga Khan University Hospital, Karachi, Pakistan to determine the optimum number of procedures needed to be performed by residents at each year of residency. Respondents included both faculty and the residents in the Department. Thereafter, all responses were compiled and later scrutinized by a focus group comprising of a mix of faculty from various subspecialties and resident representatives. Results A total of 64 responses were obtained. A significant difference was found in eight procedural skills’ status between residents and faculty, though none of these were significant after accounting for multiple consecutive testing. However, the results were reviewed and a consensus for the procedures needed was developed through a focus group. A finalized procedural list was generated to determine: (a) the minimum number of times each procedure needed to be performed by the resident before deemed competent; (b) the level of competency for each procedure for respective year of residency. Conclusion We conclude that the opinion of both the residents and the faculty as key stakeholders is vital to determine the number of procedures to be performed during an Internal Medicine Residency. Documentation of procedural competency development during the training would make the system more objective and hence reproducible. A log book was designed consisting of minimum number of procedures to be performed before attaining competency. Electronic supplementary material The online version of this article (doi:10.1186/s12909-015-0457-4) contains supplementary material, which is available to authorized users.

Background: The 2016 central nervous system (CNS) World Health Organisation (WHO) Update has merged the entities of meningeal solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into a single entity based on the presence of the nerve growth factor 1A (NGFI-A) binding protein 2 (NAB2)- signal transducer and activator of transcription 6 (STAT6) gene fusion in these tumors. Immunohistochemical (IHC) staining with STAT6 results in a strong nuclear positivity confirming the diagnosis. Meningeal SFT/HPCs are currently histologically graded according to a three-tiered system. Grade I (SFT phenotype) is benign, whereas grades II and III (HPC phenotype) are malignant and require radiotherapy in addition to gross total resection. Objectives: The objectives were to review the cases diagnosed as meningeal SFT or HPC between 2010 and 2017 and classify them into SFT (grade I) or HPC (grades II and III) phenotypes; to confirm the diagnosis by performing STAT6 immunohistochemistry; and to observe and record the histological features in detail and correlate the tumor grades with their behavior. The published literature on the subject was also reviewed. Materials and Methods: A total of 21 cases diagnosed between 2010 and 2017 as meningeal SFT or HPC were included in the study. All cases were reviewed by the authors and were categorized and graded according to histologic phenotype and mitotic count. STAT6 immunohistochemistry was performed in all the cases. The epidemiological data and histologic findings in each case were recorded in detail. The follow-up of patients was obtained. Results: Fifteen patients were males and six were females. The mean age was 43.5 years. The mean tumor size was 6.8 cm. The tumor specimens in 20 out of 21 cases corresponded to the HPC phenotype, of which 6 were in grade II while 14 were in grade III. Thus, over 95% cases had malignant lesions. The tumor in all the 21 cases recruited for the study showed immunohistochemical positivity for SAT6, while CD34 was positive in all the 18 tumor in which it was performed. The follow-up was available in 14 of the patients. Recurrence occurred in six patients who had either a grade II or a grade III tumor and three patients died (including one patient with a grade III tumor. This patient died a month after initial resection although there was no evidence of recurrence). Radiotherapy was given to only 4 out of 14 patients in whom follow-up was available. Conclusion: These rare tumors need to be accurately diagnosed and optimally treated (gross total resection and radiotherapy) to improve the prognosis.

Background: Spermatocytic seminoma is a rare testicular germ cell tumor of old men. Accounting for 1-4% of all seminomas, spermatocytic seminomas have distinct pathogenesis, histological features, immunohistochemical profile and comparatively benign clinical behavior which distinguishes them from other germ cell tumors, especially classic seminoma. Aims: The purposes of our study were to assess the patient demographics, pathological features and to evaluate the utility of CD 117 immunostain along with other immunohistochemical stains in distinguishing Spermatocytic seminomas from classic seminomas. Material and methods: All spermatocytic seminomas patients diagnosed during 1992 to 2013 at Section of Histopathology, Department of Pathology and Microbiology, Aga Khan University hospital were included. Patient characteristics, histological details and follow-up data of few patients were available. CD 117 expression was determined by immunohistochemistry. Results: Total 16 cases of Spermatocytic seminomas were reviewed. Median age was 60 years and average tumor size was 10.4 cms. Microscopically, all of the 16 cases showed presence of edema and absence of lymphocytic infiltrate and intratubular germ cell neoplasia. Cytoplasmic glycogen was negative in all 13 cases, PLAP immunostain was negative in all 12 cases, while CD 117 was positive in all 8 cases, where applied. Conclusion: CD 117 is of limited utility in differentiating the spermatocytic seminoma from classic seminoma as it is expressed in significant number of spermatocytic seminomas. However, different histological features, PAS special stain and PLAP immunostain are significantly helpful in distinguishing these two entities.

Solitary bone cysts (SBCs) are benign, intraosseus, cystic lesions, which generally involve metaphysis of long bones during the period of skeletal immaturity. Histologic features are nonspecific, but identification of amorphous cementum-like material provides a significant diagnostic clue. This material is unique to SBC with reported frequency of 10% to 70% and has been described as an immature form of bone. We retrieved and reviewed 41 cases of SBC reported in the last 10 years. The ages of patients ranged from 4 to 64 years (mean, 16 years), with a male-to-female ratio of 3:1. Humerus and femur were the most common sites. Cementum-like matrix was observed in 26 cases (63.4%). This material was seen in different phases of deposition and progression, ultimately transforming into mature bone as seen in 4 cases. Cyst wall lining was observed in 70.7% of cases along with several other nonspecific histologic features including reactive bone formation, hemosiderin macrophages, hemorrhage, multinucleated giant cells, foamy macrophages, fibrin, cholesterol clefts, and granulation tissue. Cementum-like material in the wall of SBCs is a specific and fairly consistent finding of diagnostic significance in cases where cyst wall lining is deficient. We also histologically demonstrate, for the first time, transformation of cementum-like material into reactive and mature bone, which further validates the immature osteoid nature and finding of other authors.

Background: Papillary craniopharyngioma (PCP) are uncommon variants of craniopharyngiomas (CP), which are benign epithelial neoplasms of the sellar and suprasellar region. Histologically, PCPs are typically composed of well-differentiated stratified squamous epithelium; however, focal variations are not uncommon. A distinction from other lesions of the region, despite being difficult to achieve due to the overlapping radiological and clinical features, is important for adequate treatment to be administered. Objective: Our aim was to study the clinical and histological features of PCP with emphasis on features that are helpful in its distinction from other lesions that are similar in appearance. Materials and Methods: We reviewed 13 cases of PCP diagnosed in our institution between January 2010 and December 2015. Results: The mean age at presentation was 30.76 years. Two of the patients belonged to the pediatric age group. Male-to-female ratio was 2.25:1. Suprasellar location (either alone or in combination with sellar region) was the most common tumor site. Microscopically, all of the cases showed stratified squamous epithelium with frequent pseudopapillae formation. Focal adamantinomatous epithelium and columnar epithelium with variable cilia and goblet cells were seen in 4 (30.7%) cases. Brain invasion was observed in 3 (23%) cases. Four patients died of their disease; 2 of the 7 patients with an available follow up, developed recurrences; and, 5 experienced severe postoperative morbidity. Conclusion: Majority of the PCPs exhibited typical features with minor variations. Knowledge of the variations in histologic features helps in reaching the correct diagnosis. These tumors can behave aggressively with a high recurrence rate and decreased overall survival.

Pulmonic valve endocarditis is an extremely rare infection that shares epidemiologic, clinical, radiologic, microbiologic, and prognostic features with tricuspid valve endocarditis. We report a case of pulmonic valve infection on a structurally normal heart and review the English-language literature on this subject.

BACKGROUND:Cholera is an important infectious cause of secretory diarrhea. The primary symptom of infection is the sudden onset of watery diarrhea with subsequent volume depletion causing renal insufficiency. The objective of this research is to study the level of dehydration at presentation and subsequent fluid management in patients with cholera. METHODS:This study was conducted on 191 patients of Cholera admitted at a tertiary care hospital in Karachi, Pakistan during the period of 5 years. Medical charts were evaluated retrospectively for initial hydration status, baseline lab investigations on admission and discharge and fluid therapy given to all the patients while their stay in the hospital and the data was analyzed on SPSS 15.0. RESULTS:Out of the 191 patients, 83(43%) were males and 108 (57%) were females with mean age of 42.3 years (SD+/-18.34). The average duration of symptoms was 3.75 days (SD+/-2.04). Of 191 patients, 175 (92.1%) presented with dehydration, 80 (42.3%) were given Ringer's Lactate (R/L) + Normal Saline (N/S), 45 (24%) patients were given R/L + N/S + Oral Rehydration Therapy (ORS), 27 (14.3%) of the patients were kept on R/L only and remaining were given various combinations of R/L, N/S, ORS and Dextrose Saline (D/S). On admission mean Blood Urea Nitrogen (BUN) was 24.54 (SD+/-16.6), mean creatinine was 2.47 (SD+/-2.35) and mean BUN/Creatinine ratio was 11.63 (SD+/-5.7). CONCLUSION:Aggressive fluid rehydration remains the cornerstone of management of cholera. Instead of presenting with a classical BUN/Creatinine ratio of >20:1, patients with pre-renal failure in cholera may present with a BUN/Creatinine ratio of

Aim To acquire systematic data on the causes of hospital mortality in Pakistan, a developing country with scant mortality records. Study design Retrospective review of death certificates and hospital charts of patients dying on general and specialty medical services at our hospital during one calendar year. Results Of a total 10 590 admissions, 657 (6.2%) died in hospital. The deceased included 357 (54.4%) males and 299 (45.6%) females, with a collective median age of 63 years and mean length of stay 6.71 days (median 4 days, range 1–56 days). Primary cause of death was categorised as infectious (21.2%), pulmonary (17.2%), cancer related (15.7%), cardiovascular (12.6%), gastrointestinal and hepatic (10.8%), neurological (11.4%) and miscellaneous (11.1%). Within each category, the most common diagnoses were septicaemia (76.9% of infectious cases), pneumonia (55.7% of pulmonary cases), myocardial infarction (40.9% of cardiovascular), intracranial haemorrhage (37.3% of neurological), and cirrhosis (45.0% of gastrointestinal). There were multiple causes among malignant disorders with no single cause dominating. Patients with cardiovascular and pulmonary deaths tended to be older than the median age (p = 0.001), while patients with gastrointestinal and cancer related deaths tended to be younger than the median age (p = 0.001). Length of stay did not differ significantly among the various subgroups. About a quarter (26.4%) deaths occurred within 24 h of admission. Conclusions Infections, including septicaemia and pneumonia, are the leading causes of hospital mortality in our setting, followed by malignancy and cardiovascular causes. The overall mortality rate is comparable to published mortality data from other hospital settings.

SummaryBackgroundA large number of patients treated at our hospital for endocarditis have negative cultures. Taking into consideration the fact that many of these patients receive antibiotics prior to referral, we decided to study culture-negative endocarditis in Pakistan.MethodsThe medical records of all patients admitted to the Aga Khan University Hospital, Pakistan, for the period from 1988 to 2001, with an underlying diagnosis of infective endocarditis (IE) and negative cultures, were reviewed.ResultsOf the 159 patients diagnosed with IE by revised Duke criteria, 86 (54.1%) had persistent negative cultures. More than half of these patients (52%) had received antibiotics before being referred to our center. Patients with culture-negative endocarditis were less likely to be classified as definite endocarditis by revised Duke criteria (p

Objectives: Infective endocarditis is common and data regarding its pattern and outcome from developing countries is sparse. We therefore examined the spectrum, demographics and clinical features of infective endocarditis and sought to determine the factors affecting its clinical outcome.Patients and methods: Over a five-year period at our university hospital in Karachi, Pakistan, we identified 66 patients with infective endocarditis and compared their clinicoepidemiologic features and outcomes to subjects in the West.Results: The male:female ratio was 2:1; overall median age was 24 years (35.5 years for men and 13.5 years for women) (p

Background Ward rounds form an integral part of Internal Medicine teaching. This study aimed to determine the trainees' opinions regarding various aspects of their ward rounds, including how well they cover their learning needs, how they would like the rounds to be conducted, and differences of opinion between medical students and postgraduates. Methods A cross-sectional study was conducted on a total of 134 trainees in Internal Medicine, comprising medical students, interns, residents and fellows, who were asked to fill in a structured, self-designed questionnaire. Most of the responses required a rating on a scale of 1-5 (1 being highly unsatisfactory and 5 being highly satisfactory). Results Teaching of clinical skills and bedside teaching received the lowest overall mean score (Mean ± SD 2.48 ± 1.02 and 2.49 ± 1.12 respectively). They were rated much lower by postgraduates as compared to students (p < 0.001). All respondents felt that management of patients was the aspect best covered by the current ward rounds (Mean ± SD 3.71 ± 0.72). For their desired ward rounds, management of patients received the highest score (Mean ± SD 4.64 ± 0.55), followed by bedside examinations (Mean ± SD 4.60 ± 0.61) and clinical skills teaching (Mean ± SD 4.50 ± 0.68). The postgraduates desired a lot more focus on communication skills, counselling and medical ethics as compared to students, whose primary focus was teaching of bedside examination and management. A majority of the respondents (87%) preferred bedside rounds over conference room rounds. Even though the duration of rounds was found to be adequate, a majority of the trainees (68%) felt there was a lack of individual attention during ward rounds. Conclusions This study highlights important areas where ward rounds need improvement in order to maximize their benefit to the learners. There is a need to modify the current state of ward rounds in order to address the needs and expectations of trainees.

This is a case report of a patient who presented to the Aga Khan University Hospital with generalized abdominal lymphadenopathy and high-grade fever. Due to ambiguous clinical findings, which were suggestive of either abdominal tuberculosis, or a lymphoma, the patient was started on empirical anti-tuberculous treatment due to the endemicity of tuberculosis in this region. The blood culture reports, however, were reported to grow colonies of Salmonella paratyphi A; thus the diagnosis of the patient was changed to enteric fever, and the patient improved on the subsequently started therapy of ceftriaxone 2 000 mg bid. To the best of our knowledge, this is the first reported case of a patient suffering from enteric fever whose primary clinical findings were abdominal lymphadenopathy and fever.