Does Hepatocyte Paraffin 1 expression stand a role in determining the site origin of an adenocarcinoma from unknown gastrointestinal primary.

Background: Hepatocyte Paraffin 1 (Hep Par 1) was being extensively used to recognize the hepatocellular carcinomas, until recognition of its expression in tumors without hepatocellular differentiation. Aims and Objectives: The aim of this study was to analyze if Hep Par 1 stain can serve as a specific marker of the small intestinal (SI) adenocarcinomas, versus other gastrointestinal tract (GIT) primary tumors. Materials and Methods: In this retrospective cross‑sectional study, normal GIT mucosa (n ‑ 60), corresponding adenocarcinomas (n ‑ 60) and nodal metastatic foci (n ‑ 60) from the same patients, including 10 cases each from the esophagus, stomach, SI periampullary region, colon, rectum, and gall bladder were included. H‑score was calculated by multiplying the stain distribution and intensity scores. The H‑scores were compared with other clinical and histological parameters. Results: While normal SI mucosa showed diffuse strong Hep Par 1 staining, normal esophageal and gastric epitheliums were negative and normal colon, rectal, and biliary epithelium showed weak focal positivity. Adenocarcinomas from all these sites, however, showed Hep Par 1 expression, irrespective of the tumor type, site or origin, and tumor stage. The corresponding metastatic sites also showed variable Hep Par 1 positivity, without any site specificity. Conclusion: Hep Par 1 stain cannot help to determine the exact site of origin of primary GIT tumors. Its expression in adenocarcinomas across the GIT and their metastatic foci proves that it cannot be regarded as a marker of SI differentiation, especially in malignancy.

Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.

Neurofibromatosis type‑1 (NF‑1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50‑year‑old female NF‑1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Thorough radiological and peroperative work‑up revealed multiple ulcerated submucosal and serosal nodules in the proximal small intestine. Histological work‑up revealed diagnosis of a duodenal submucosal somatostatinoma with multifocal serosal gastrointestinal stromal tumors. This case is being reported to highlight the rare coincidence of multiple GIT tumors in an NF‑1 patient.

Demographic profile, host, disease & viral predictive factors of response in patients with chronic hepatitis C virus infection at a tertiary care hospital in north India.

Background & objectives: Standard of care for chronic hepatitis C (CHC) in India is peginterferon and ribavirin (RBV). The response to treatment in real life stetting is unclear. The objectives of this study were to evaluate the demographic profile and assess the virological response and predictors of response in CHC patients. Methods: Consecutive patients with CHC were included in this study. Detailed clinical history, risk factors, and predictive factors of response were noted. Patients were treated with peginterferon α2b (1.5 μg/kg/wk) and RBV (12 mg/kg/day) for 6 to 18 months based on response. Results: A total of 211 patients were included in the analysis, mean age 40.6±12.3 yr, 144 (68%) were males and 71 (34%) had compensated cirrhosis. Commonest risk factor for acquiring CHC was previous transfusion and surgery (51%). Genotype 3 (72%) was most common followed by genotype 1 (23%). Overall sustained virologic response (SVR) was 64 per cent [95% CI 57.1%-70.4%]. The SVR was 66.5 per cent [95% CI 58.34-73.89%] for genotype 3 and 61.2 per cent [95% CI 46.23 to 74.80%] for genotype 1. Non-cirrhotics had better SVR rates compared to cirrhotics (76 vs 41%, P<0.001). On multivariate analysis, BMI ≥23 kg/m2, HOMA-IR ≥2, compliance (≤80%), and fibrosis >2 were predictors of low SVR. Interpretation & conclusions: Genotype 3 was the commonest HCV genotype. The commonest source of infection was previous transfusion and surgery. SVR rates for genotypes 3 were better than genotype 1 patients. Predictors of non-response were high BMI, insulin resistance, significant fibrosis and inadequate compliance.

Coeliac disease in patients with short stature: A tertiary care centre experience.

Background. We aimed to determine the prevalence of coeliac disease among children with short stature at a tertiary care centre and to define the predictors for coeliac disease, if any, in them. Methods. In this retrospective study, we reviewed the case records of children and adolescents with growth retardation attending the Paediatric Endocrinology Clinic from January 2008 to June 2011. All patients underwent the multi-tier stratified diagnostic protocol for complete evaluation of short stature. Coeliac disease was screened using IgA-anti-tissue transglutaminase antibody. The diagnosis of coeliac disease was made on the basis of the modified European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) criteria. Results. Of 432 patients (238 boys) who presented with short stature, 72 (16.7%) had physiological, while 360 (83.3%) had pathological causes. Endocrine causes were growth hormone deficiency (86 patients, 19.9%), hypopituitarism (31, 7.2%), hypothyroidism (22, 5.1%) and others (7, 1.6%). The systemic causes were: coeliac disease (47, 10.9%), haematological diseases (14, 3.2%), renal diseases (11, 2.5%) and others (24, 5.6%). Chronic diarrhoea (OR 15.7, 95% CI 7.8–31.5) and anaemia (OR 4.9, 95% CI 1.9–12.7]) were significant predictors for coeliac disease in patients with short stature. There was a definite response to gluten-free diet in them and the mean (SD) growth velocity measured over at least 6 months of gluten-free diet was 8.1 (3.0) cm/year. Conclusion. Nearly 11% of patients presenting with short stature have coeliac disease. In these patients chronic diarrhoea and anaemia were significant predictors of coeliac disease.

Interpretation of ileal biopsies.

The ileum is one of the most common sites of intestine to undergo endoscopic biopsy. However, even with the experienced histopathologists, a defi nite diagnosis can be achieved only in 18% cases. Lack of knowledge about proper tissue handling, tissue orientation, overlapping histological fi ndings, and lack of a standard algorithm based approach results in this low diagnostic yield. In this review article, we have tried to discuss these aspects and give a clear picture how to approach the ileal lesions. It would help the surgical pathologists in effectively interpreting the lesions and to identify the common pitfalls.

Pelvic mature cystic teratoma with neuroendocrine carcinoma: Report of a rare association and review of literature.

Extra-gonadal malignant transformation of teratoma is rare and there are only a few reports available citing malignancy arising in the non-germ cell components. We hereby report a case of a 35-year-old female, who presented with lower backache with a radiologically identifi able mass lesion in the pre-sacral region. Clinical and radiological fi ndings suggested the possibility of a cystic teratoma. Histopathological examination and relevant immunohistochemical tests detected a mature cystic teratoma with features of a grade 2 neuroendocrine tumor in it. Like the index case, most of the previously reported cases of teratoma with malignant transformation of the somatic components were found in extra-gonadal site. This case is being reported to emphasize that any extra-gonadal mass in reproductive age group, even if it appears radiologically and per-operatively benign, must be subjected to histopathological examination to rule out possibility of malignant transformation of the germ cell or non-germ cell components.

Subcutaneous panniculitis-like T-cell lymphoma: A clinicopathologic study of 5 cases.

Background: Subcutaneous panniculitis-like T-cell lymphoma is as yet a poorly characterized subtype of cutaneous T-cell lymphomas. It is difficult to diagnose and lacks a standard treatment protocol. Materials and Methods: We report our experience with five such cases in patients with age ranging from 13 to 45 years, wherein we reviewed their clinical presentations, histopathological findings, treatment and clinical outcome. Results: Three out of the five cases are alive of which two are in complete remission. Our series stresses on the need to be aware of this uncommon entity in a dermatology clinic. Facial and upper extremity skin nodules with involvement of the lower dermis on light microscopical examination and suggestive immunohistochemical findings were frequently observed in our patients in contrast to previously described cases.

Current spectrum of malabsorption syndrome in adults in India.

Aim Tropical sprue was considered to be the most important cause of malabsorption in adults in India. However, several reports indicate that celiac disease is now recognized more frequently. Methods We analyzed the clinical presentation, endoscopic and histological features of 94 consecutive patients (age >12 years) with chronic diarrhea and malabsorption syndrome. The spectrum of disease in these patients and features differentiating celiac disease and tropical sprue are reported here. Results Celiac disease (n=61, 65%) was the most common cause of malabsorption followed by tropical sprue (21, 22%). Other conditions including cyclosporiasis (3), Crohn’s disease (2), common variable immunodeficiency (2), lymphangiectasia (1), William’s syndrome (1), and idiopathic malabsorption (3) accounted for the remainder. A greater number (21, 34%) of patients with celiac disease than those with tropical sprue (4, 19%) presented with atypical manifestations. Patients with celiac disease were younger (p=0.001), more often had anemia, (p=0.001), scalloping of folds (p=0.001), moderate (p=0.02) or severe (p=0.001) villous atrophy, higher grade of intraepithelial lymphocytic infiltration (p=0.001), crypt hyperplasia (p=0.001), cuboidal (p=0.001) and pseudostratified (p=0.009) surface epithelial cells, and diffuse (p=0.001) epithelial damage. In comparison, patients with tropical sprue were older and more often had normal duodenal folds, normal villi, tall columnar epithelial cells and focal epithelial damage. Conclusions Celiac disease was the most frequent cause of malabsorption syndrome in this series of patients. There are significant clinical and histological differences between celiac disease and tropical sprue.

Mediastinal liposarcoma of mixed type in childhood: A report of a case with unusual histologic features.

We hereby report the occurrence of mediastinal liposarcoma in a 11-year-old female child. Dyspnea and wheezing of long-standing duration were the presenting complaints and a preoperative biopsy failed to yield the diagnosis. Histologic examination revealed heterogeneous areas with well-differentiated liposarcoma-like areas, areas resembling myxoid liposarcoma, and areas of dedifferentiation. Osteosarcomatous foci and whorled areas reminiscent of meningioma were identified in the dedifferentiated areas. Liposarcomas in the mediastinum are extremely rare tumors of childhood and the present case showed unusual histologic features. Complete surgical excision with clear surgical margins remains the primary modality of treatment, although chemotherapy and radiotherapy have been tried.

Primary intestinal lymphangiectasia as a component of autoimmune polyglandular syndrome type I: a report of 2 cases.

Chronic diarrhea and steatorrhea occur frequently in patients with autoimmune polyglandular syndrome (APS) type I. Intestinal lymphangiectasia has been reported earlier as a cause of steatorrhea in a young girl with APS Type I. We describe 2 patients with APS Type I who were found to have intestinal lymphangiectasia, one of whom had symptomatic protein-losing enteropathy.

Spectrum of microscopic colitis in a tertiary care centre in India.

INTRODUCTION: The incidence of microscopic colitis has recently increased. Although collagenous colitis and lymphocytic colitis are the two main subtypes of microscopic colitis, many patients may not fit into either category and are thus included under the header nonspecific colitis. Of late, the spectrum of microscopic colitis has widened to include minimal change colitis, microscopic colitis not otherwise specified and microscopic colitis with giant cells. There is a lack of information concerning the spectrum of microscopic colitis in Asia. METHOD: In a retrospective analysis, case records of 29 patients diagnosed with microscopic colitis between 1999-2005 were analysed. Drug use parasitic infection and common bacterial infections were excluded. Colonoscopic/ sigmoidoscopic examination was done and multiple colonic mucosal biopsies were stained serially with haematoxylin and eosin for detailed histological examination and Masson trichrome for sub-epithelial collagen band. Based on histological criteria, patients were categorised into five subtypes: collagenous colitis (presence of collagenous thickening of surface epithelium basement membrane > 10 microm), lymphocytic colitis (intra-epithelial lymphocytes more than 20 per 100 colonocytes), minimal change colitis (crypt architectural abnormality in the form of cryptitis and crypt dilatation in the absence of increase in intraepithelial lymphocytes and subepithelial collagenous band), microscopic colitis not otherwise specified (increased inflammatory cell infiltrates in the lamina propria in the absence of other abnormalities) and microscopic colitis with giant cells. RESULTS: Mean age of patients was 38.59 years (range 12-62). Of 29 patients with microscopic colitis, 7 (24.1%), 4 (13.8%), 7 (24.1%) and 11 (37.9%) were classified as collagenous colitis, lymphocytic colitis, minimal change colitis and microscopic colitis not otherwise specified, respectively. None of these patients had giant cells. There was no significant correlation between disease type and clinical manifestations. CONCLUSION: Microscopic colitis has a wide histological spectrum. Cases reported as non-specific colitis, may be categorised into definite subtypes of microscopic colitis.

Chronic diarrhea and malabsorption caused by Leishmania donovani.

Chronic diarrhea and malabsorption are uncommon in immunocompetent patients with visceral leishmaniasis. We report two immunocompetent patients with visceral leishmaniasis where the predominant presentation was chronic diarrhea. One of them had clinically overt malabsorption and duodenal mucosa was loaded with Leishmania donovani bodies. The other patient had diffuse colonic aphthous and discrete ulcerations and Leishmania donovani bodies were seen in the crush smears of the colonic mucosa. With amphotericin B, there was reversal of malabsorption and healing of colonic ulcers.

Association of Dubin-Johnson syndrome and portal vein thrombosis.

Dubin-Johnson syndrome is neither complicated by liver cell necrosis nor associated with portal hypertension. We report a 22-year-old man who had recurrent episodes of jaundice (conjugated hyperbilirubinemia) because of Dubin-Johnson syndrome and portal hypertension secondary to portal vein thrombosis. The relationship between Dubin-Johnson syndrome and portal vein thrombosis in this case is most likely a chance occurrence.