Body Stalk Syndrome: A Curiosity

Limb body wall complex (LBWC) /Body stalk syndrome anomaly refers to a rare complicated polymalformative fetal malformation syndrome of uncertain etiology firstly described by Van Allen et al in 1987. There are very few cases reported in literature and thus we report a rare case of LBWC. Twenty seven years female presented to labour room with 32 weeks of gestation with no prenatal care and delivered a low birth weight still born fetus weighing 1100gms. On fetal autopsy large abdominal wall defect was noted with difficulty in identifying abdomino-pelvic organs and ambiguous genitalia. Placenta weighed 250gms with attached short umbilical cord measuring 7cms, arising from periphery. A cyst noted attached to placental membrane measuring 9x5cms which on dissection retrieved partially maldeveloped organs. Post mortem radiological findings included Absence of right femur with short tibia and right fibula, Complex vestibral malformation, Craniosynostosis and Overcrowding of ribs

Dandy Walker Syndrome with Unusual Associated Findings in a Fetal Autopsy Study

Dandy Walker Syndrome (DWS) is a congenital brain malformation characterized by hypoplasia or absence of cerebellar vermis, cystic dilatation of fourth ventricle and hydrocephalus. It is frequently associated with other congenital anomalies. Associated central nervous system anomalies such as agenesis of corpus callosum and vermis are associated with poor prognosis. Association of DWS with congenital absence of spleen is life threatening condition and has been reported very rarely. Autopsy findings of DWS with association of congenital absence of corpus callosum, vermis and spleen are reported in a stillborn fetus of 28 weeks gestation and review of relevant literature was don

Effect of 1,25-dihydroxy vitamin D3 (1,25(OH)2D3 ) on hexavalent chromium (Cr (VI)) induced alteration of glucose homeostasis in Wistar rats

Background: 1,25-dihydroxy vitamin D3(1,25(OH)2D3) is a fat-soluble known antioxidant vitamin to protect cardiovascular health. Hexavalent chromium (Cr (VI)) as a heavy metal has adverse effects on vascular system. Aim and Objectives: To evaluate the possible protective effects of 1,25(OH)2D3 supplementation on Cr (VI) induced altered glucose regulation. Materials and Methods: Twenty-four adult Wistar male rats were divided into four groups (n=6 in each group) as following: Group-1 (control); Group-2 received K2Cr2O7 5.0 mg/kg body weight intraperitoneally for 10 dosages on every alternate day for 20 days; Group-3 received 1,25(OH)2D3 12.5μg/kg/d, orally daily till 20 days; and Group-4 received both K2Cr2O7 and 1,25(OH)2D3 with dosages as above. At the end of 10th dosage after overnight fasting i.e. (on day 21) blood samples were collected from tail vein of all the rats. Oral Glucose Tolerance Test (OGTT), serum glucose and insulin concentrations were estimated. Insulinogenic index was also calculated. Liver glycogen concentrations were assessed after sacrificing the animals. Results: OGTT showed an increase of fasting blood glucose levels in Cr (VI) treated Group-2 rats till at the end of 2.0 hrs. The Cr (VI) treated and simultaneously supplemented with 1,25(OH)2D3 Group-4 rats showed lesser elevation of blood glucose level till 2.0 hrs. A decrease in plasma insulin level and increase in insulinogenic index were also found in Cr (VI) treated Group-2 rats but in case of vitamin D supplemented Group-4 rats, both plasma insulin levels and insulinogenic index were found to be improved remarkably. Liver glycogen concentrations in K2Cr2O7 treated rats were also found to be reduced significantly but 1,25(OH)2D3 supplemented Group-4 rats showed improvement in liver glycogen concentrations. Conclusion: 1,25(OH)2D3 is found to be beneficial against hexavalent chromium induced alteration of glucose homeostasis

Metastatic Renal Cell Carcinoma Masquerading As Benign Cystic Lesion on Cytology

Clear Cell-Renal Cell Carcinoma (CC-RCC), the most common subtype, accounting for 75% of all RCCs, can metastasize to any part of the body. Nodal metastasis is commonly noted in the pulmonary, tracheal and retroperitoneal lymph nodes. However, metastasis to cervical/supraclavicular lymph nodes is extremely rare. Moreover, these metastatic nodal deposits are usually solid, but may become cystic, which warrants a differential diagnosis of metastatic lesion in an elderly patient. So, here we report a case of metastatic RCC presenting as left supraclavicular lymphadenopathy without any clinical manifestations of the primary which was misdiagnosed as a benign cystic lesion on cytology

Phaeohyphomycosis Masquerading As Epidermal Cyst – A Diagnostic Dilemma in Immunocompetent Hosts and Review of Literature

Phaeohyphomycosis is caused by a rare dematiaceous, pigmented, mycelia forming fungi. It is an opportunistic fungal infection in immunocompromised hosts. It can present with varied clinical manifestations, as subcutaneous infection to widespread fulminant disseminated disease. Here we present three cases of subcutaneous phaeohyphomycosis in immunocompetent hosts at unusual site – popliteal fossa, great toe and sole of the foot. Awareness of unusual clinical presentation of Phaeohyphomycosis can lead to early diagnosis which may help in prevention of wide spread dissemination of disease and fatal outcome

Intestinal Duplication Cyst Mimicking as Mesenteric Cyst with Asso- ciated Ileal Atresia Type III A

Intestinal duplication cysts (IDC) are uncom-mon congenital malformations that couldpresent diagnostic and therapeutic challenge.They may be often mistaken as mesentericcysts, omental cyst, cystic lymphangioma etc.However, IDC are differentiated from otherintra-abdominal cystic lesions by presence ofgastrointestinal mucosal lining and smoothmuscles in their wall. We report a case of IDCmimicking as mesenteric cyst associated withatresia of ileum in a neonate presented withacute surgical emergency

Xanthogranulomatous Appendicitis: A Rare Case Report

Xanthogranulomatous inflammation is a form of chronic inflammation where the exact eitiology is not known. It is commonly reported in organs like kidney and gall bladder Very few cases of xanthogranulomatous inflammation of appendix have been reported in the literature. We report a case of 30 year female presenting with pain abdomen and fever for which provisional diagnosis of appendicitis was made. Open appendicectomy was performed which on intraoperative examination showed that appendix was adherent to surrounding structures forming a mass. Resection of the mass was done and sent for histopathological examination. Gross examination of the received specimen revealed multiple irregular tissue bits and one tubular tissue bit. Multiple sections studied from resected tissue showed features of xanthogranulomatous inflammation in appendix and periappendicular tissue

Effectiveness of the International Consensus Group criteria for manual peripheral smear review

Context: The International Consensus Group for Hematology Review (ICGHR) are essentially review criteria designed to reduce the number of manual smear reviews following analysis in automated hematology analyzers (AHAs). Although AHAs are an indispensable part of the present-day clinical laboratory, manual smear reviews still play an integral role in identifying morphological abnormalities and to confirm the results of the analyzers. Aims: The aim of this study is to evaluate the efficacy of the ICGHR criteria and our laboratory criteria using the Sysmex XN-1000 for manual peripheral smear review (MSR). Study Design: A prospective cross-sectional comparative study between the two sets of criteria for MSR was performed. Material and Methods: A total of 860 whole blood samples sent over a period of 19 months for complete blood count testing to our laboratory were collected using systematic random sampling. Truth tables were prepared for each set of criteria. Tests of proportion were used to compare performance specifications between both sets of criteria. Results: Using ICGHR criteria, sensitivity was 81.58%, specificity was 84.61%, 83.38% positive predictive value, and 82.92% negative predictive value. The microscopic smear review rate was 47.56% and efficiency was 83.14%. Using our laboratory criteria, sensitivity was 98.80%, specificity was 41.40%, positive predictive value of 61.46%, and negative predictive value of 97.34%. The microscopic smear review rate was 78.14% and efficiency 69.30%. Conclusions: There was a significant reduction in the microscopic smear review rates using the ICGHR criteria compared to our laboratory criteria. The ICGHR criteria can thus be adapted to daily laboratory practice provided they are first optimized and locally validated before use

A Rare Cutaneous Adnexal Tumour with a Rare Presentation

Proliferating Trichilemmal Tumour (PTT) is a rapidly growing large cutaneous adnexal neoplasm. Although biologically considered as benign, it may be locally aggressive. Malignant transformation of these lesions, known as Malignant Proliferating Trichilemmal Tumour (MPTT) has rarely been reported. So far in the literature, only 39 well-documented cases of MPTT have been reported. MPTT has been stated to be a neoplasm of the older age group according to review of the literature. We present a case of MPTT in a young male. A 25 year old male presented with a scalp swelling of 2 years duration with a recent rapid enlargement. The swelling was excised and histopathological examination of the excised specimen revealed features of MPTT. The differential diagnosis of MPTTis squamous cell carcinoma as both share common features. Accurate diagnosis of MPTT is essential since it has a tendency to metastasize and recur more frequently than squamous cell carcinoma

Expression of proliferative marker Ki-67 in epithelial dysplasia and squamous cell carcinoma of oral cavity

Background: The proliferative index helps to evaluate the growth rate and aggressiveness of the malignant tumor. Ki-67 is one of the proliferating markers that help to predict the patient's survival in Oral Epithelial Dysplasia (OED) and Oral Squamous Cell Carcinoma (OSCC). Hence, the present study evaluated the expression of Ki-67 in OSCC and OED. Aim and Objectives: To evaluate the expression of Ki-67 in OED and OSCC and to correlate the Ki-67 expression with various grades of OED and OSCC. Material and Methods: A prospective observational study was done on clinically suspected oral epithelial dysplasia and malignancy cases received in the histopathology section. For each case, two sections of 4 μm were prepared. One was stained by hematoxylin and eosin stain, and another section was used for immunohistochemical staining of Ki-67. Results: Out of 64 cases evaluated for Ki-67 expression, 19 cases were OED, and 45 were OSCC. In OED, 15 cases were mild, and three were severe. In severe OED, Ki-67 expression was seen in all three lining epithelium layers: basal, parabasal, and spinous. In mild OED, Ki-67 expression was noted only in the basal layer. Out of 45 cases of OSCC, 31 cases were moderately differentiated, and one was poorly differentiated OSCC. Ki-67 expression was highest in poorly differentiated OSCC and lowest in well-differentiated OSCC. Conclusion: Ki-67 expression was high in poorly differentiated carcinoma compared to well-differentiated squamous cell carcinoma, and in severe OED Ki-67 was noted in all layers of lining epithelium. These findings suggest that Ki-67 expression correlates with the grading of OED and OSCC. Thus, it can be considered a proliferating marker that predicts the malignant transformation in OED and the aggressiveness of tumor