Ewing sarcoma arising in colon and ovary--Two rare sites of presentation.

Ewing sarcoma is a rare aggressive malignant round cell tumor, primarily presenting in bone and soft tissues. This study presents two cases of this tumor in unusual locations, one in right colon which presented with intussusception and other in ovary which presented clinically as carcinoma ovary. Both the cases showed histomorphology of primitive round cell tumor with characteristic immunohistochemical profile and was confirmed on molecular analysis. We aim to highlight the importance of considering Ewing sarcoma in the differential diagnoses in these locations as they have dismal prognosis with no standard treatment modality.

Uterine collision tumor of endometrial stromal sarcoma and endometrioid adenocarcinoma: A rare case report and review of literature.

Collision tumors are characterized by occurrence of two or more histologically distinct tumor types at the same anatomic site. Collision tumors have been reported in various organs, such as esophagus, stomach, colon, kidney, lung, skin, thyroid, breast, ovary and uterus. Uterine collision tumors of epithelial and mesenchymal origin are rare and often underrecognized. We report a rare concurrent occurrence of endometrial stromal sarcoma and endometrioid adenocarcinoma in a 65-year-old female. It is important to differentiate collision tumors from carcinosarcoma due to impact on clinical management and prognosis. Extensive gross sampling and careful morphological examination aided by immunohistochemical studies is necessary for the diagnosis of this rare entity. This case report aims to increase the awareness of this rare pathological entity with discussion on the management issue based on review of literature. This is the first case in Indian literature to the best of our knowledge.

Role of whole-body magnetic resonance imaging in the evaluation of extrapulmonary tuberculosis in immunocompetent patients.

OBJECTIVE: The aim of this study was to evaluate the role of whole-body magnetic resonance imaging (WB-MRI) in imaging of extrapulmonary tuberculosis. METHODS: Eighteen patients with single-site extrapulmonary tuberculosis were evaluated with contrast-enhanced dedicated MRI of the clinically symptomatic site followed by WB-MRI using contrast-enhanced 3-dimensional (3D) modified DIXON technique (m-DIXON) and diffusion-weighted WB imaging with background body signal suppression (DWIBS) sequences. Studies were read by 2 experienced radiologists, and additional lesions seen on WB-MRI were separately charted. RESULTS: Of 18 patients, 14 were found to have asymptomatic involvement of other organs on WB-MRI. In 5 patients, the information was helpful in choosing an easily accessible site for biopsy/aspiration. Postcontrast 3D m-DIXON was better in picking up brain and lymph nodal lesions, whereas DWIBS was better in detecting vertebral lesions. CONCLUSIONS: Whole-body MRI may be used for assessing the asymptomatic involvement of other body organs in tuberculosis. The combination of postcontrast 3D m-DIXON and DWIBS is complementary and may provide a road map for biopsy of accessible lesions.

Etiologic types of end-stage chronic liver disease in adults: analysis of prevalence and their temporal changes from a study on native liver explants.

BACKGROUND: Whole native livers from orthotopic liver transplant (LT) recipients provide an ideal resource material for the proper identification and etiologic evaluation of end-stage liver diseases in these patients. This study determined the etiologic types of chronic liver disease (CLD) in adults of our geographic region receiving living donor LT and projected approximate estimates of their current prevalence and temporal changes in these in the general population. MATERIALS AND METHODS: The final etiologic categorization of CLD in 372 adult LT recipients was made only after correlating the morphologic findings on explanted whole native livers with all pre-LT data and diagnosis. RESULTS: The final etiologic categorizations of end-stage CLD in the majority (88.4%) of explanted livers in our series were as follows: hepatitis virus related--48.6% [hepatitis C virus (HCV)--31.1%, hepatitis B virus (HBV)--15.9%, HCV and HBV--1.6%]; alcohol related--23.1%; and NALD related--16.7%. Of 84 cases clinically considered as cryptogenic cirrhosis, 57 and nine were finally categorized as nonalcoholic fatty liver disease (NAFLD) cirrhosis and noncirrhotic portal fibrosis, respectively. Hepatocellular carcinoma (HCC) was found in 20.7% of all livers, 81.8% of these tumors developing in HBV-related and/or HCV-related CLD and 9.1% each in alcohol-related and NAFLD-related CLD. CONCLUSION: The etiology of end-stage CLD in adults of our region has changed over time. HCV, more than HBV, is now the major cause of both CLD and HCC; alcohol-related CLD has increased significantly and several cases of cirrhosis clinically considered as cryptogenic, some of them with HCC, evolve from NAFLD. A proportion of cryptogenic cirrhosis cases that require LT are constituted by the noncirrhotic disease noncirrhotic portal fibrosis.

End-stage nonalcoholic fatty liver disease: evaluation of pathomorphologic features and relationship to cryptogenic cirrhosis from study of explant livers in a living donor liver transplant program.

In a proportion of liver cirrhosis, the etiology continues to remain elusive. It is uncertain whether and to what extent cirrhosis evolving from nonalcoholic fatty liver disease contributes to this group of cryptogenic cirrhosis because the clinicopathologic features of nonalcoholic fatty liver disease cirrhosis are largely unknown. We explored these facets by examining the explant livers and clinical data in living donor liver transplant recipients. Among 103 adult liver transplant recipients with different types of chronic liver disease, 30 had a pre-liver transplant diagnosis of cryptogenic cirrhosis. A final categorization of the native liver disease was attempted in these cases on the basis of detail pathomorphological findings in adequately sampled explant liver correlated with careful review of pre-liver transplant clinical data. Of the 30 cryptogenic cirrhosis cases, 19 (63.3%) finally labeled as nonalcoholic fatty liver disease cirrhosis showed histologic features in several respects different from those reported for the early and established phases of nonalcoholic fatty liver disease. Steatosis was infrequent and focal or even absent, whereas variable grades of Mallory hyaline and inflammation were consistently present. Ductular proliferation and hydropic change of hepatocytes were also frequent. Only 9 (47%) of the 19 cases had nonalcoholic fatty liver disease associated risk factors like diabetes and obesity. It was concluded that appreciation of quantitative and qualitative differences in hepatic morphology between the cirrhotic and the early/established stage of nonalcoholic fatty liver disease will help in making a correct diagnosis of nonalcoholic fatty liver disease cirrhosis in the proper clinical setting. When appropriate criteria are used, nonalcoholic fatty liver disease appears to account for close to two thirds of cases currently labeled as cryptogenic cirrhosis.

Spectrum of histological features in non-alcoholic fatty liver disease.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) has become an Important entity globally, including in India and other Asian countries. Morphological evaluation of a liver biopsy is necessary for the diagnosis, staging and possibly management of this disease. However, the spectrum of changes in the liver, their evolution, Interrelationships and implications are incompletely understood. We aimed to study the spectrum of histological abnormalities in NAFLD. METHODS: The study material was drawn from a pool of 80 liver biopsies diagnosed in our laboratory as NAFLD, 67 retrieved retrospectively from our records and 13 obtained prospectively with complete clinical data. After comprehensive histological assessment, a detailed analysis was done of 32 of those categorized as definitive NAFLD on the basis of a dependable history of no alcohol Intake and seronegativity for hepatitis virus B and C Infections. RESULTS: Fatty change was preferentially seen in acinar zones 2 and 3, more so in the former. Steatotic cells varied in size; some large ones were non-spherical. Steatosis alone was present in more than a quarter of the cases and steatosis along with inflammation was present in half. The magnitude of steatosis correlated with inflammation, while both these seemed to correlate with hepatocyte Injury and fibrosis. CONCLUSION: A proportion of patients with NAFLD show only hepatic steatosis. An Increasing grade of steatosis is associated with greater Inflammation, hepatocyte injury and acinar fibrosis. Preferential involvement of acinar zone 2 by steatosis, the morphology of the steatotic cells, and nature and location of inflammation are important in the diagnosis of NAFLD and its differentiation from other causes of fatty liver.

Distinguishing between periampullary carcinoids and carcinomas--is this possible preoperatively?

It is difficult to distinguish between carcinoid tumors of the pancreatic head and periampullary region and carcinomas preoperatively. Between 1996 and 2002, 125 consecutive pancreaticoduodenectomies done by us for periampullary tumors (14 carcinoids, 111 carcinomas) were analyzed. Patients with carcinoid tumors had significantly younger mean age (48 vs. 54 years), longer history (32 vs. 8 weeks), lower serum total bilirubin levels (1.4 vs. 6.3 mg/dL) and on CT scan, had larger, well-localized tumors (5 cm vs. 2 cm). Their postoperative course was better with no mortality or major morbidity, whereas after resection for carcinoma 7 (6.3%) patients died and 30 (27%) had major postoperative complications. Thus, a tumor of this region in a young patient with indolent history, low bilirubin level and with CT scan depicting a large expansile lesion suggests a carcinoid. Such tumors may be safely resected with low postoperative morbidity and mortality and good long-term prognosis.

Non-specific interstitial pneumonia in an HIV positive patient.

A 56 year old male presented with features of interstitial lung disease and was detected HIV positive. Histopathology of the lung suggested non-specific interstitial pneumonia with no evidence of tumour or Pneumocystis carinii. The patient responded to steroids and was started on anti-retroviral treatment.

Immunohistochemical detection of serum autoantibodies--a new technique using routine formalin fixed paraffin sections.

Detection of autoantibodies in serum is important in the diagnosis of primary immune mediated diseases. Tests of these antibodies are conventionally done by indirect immunoflourescence (IIF) on frozen sections of fresh target tissues from the murine species. More recently enzyme-linked immunoassays and IIF on cultured human epithelial cells or on fresh frozen sections of murine tissues coated onto wells or glass slides are also being used. But these tests are more expensive and generally not easily available to laboratories in developing countries. Obtaining small animals for preparing frozen sections of fresh tissue is also getting to be increasingly difficult. A simple; new and inexpensive technique was developed to perform IIF on routine paraffin sections of human tissues following antigen unmasking. This, technique offers qualitatively good, consistent, species specific and dependable results with several advantages over the conventional IIF on animal tissue frozen sections, particularly in a sausage block made from different types of tissues. Immunoperoxidase stain for autoantibodies can also be easily performed with the advantages of permanent preservation and clearer evaluation in light microscopy. Most importantly the technique is easily affordable and practicable in all histopathology laboratories.

Connective tissue lipoatrophic panniculitis.

A 23 years old girl with a past history of sero-positive oligoarticular juvenile idiopathic arthritis developed nodules and depressed scars over face and limbs. Biopsy from the lesion revealed mixed panniculitis. She responded to steroids and hydroxychloroquine but has residual lipoatrophic scars needing reconstructive surgery.

CD99 expression in hepatocellular carcinoma: an immunohistochemical study in the fibrolamellar and common variant of the tumour.

Histological diagnosis of hepatocellular carcinoma (HCC) in biopsy and fine needle aspiration material can pose difficult problems. This is particularly so in the fibrolamellar variant of the tumour (FLHCC) which has biological features distinctly different from those of common HCC (CHCC). Expression of CD99, a membrane localised glycoprotein which is known to be a feature of diagnostic importance in several malignant neoplasms was looked for in immunohistochemical preparation of tissue material from 18 cases of HCC, 6 FLHCC and 12 CHCC accessioned in our department. Positive staining of variable degrees and intesity was observed in all 18 cases. Non-neoplastic hepatocytes, normal or others and all the 18 other cases of non-HCC cancers outside the liver or metastatic within the liver were negative. In some cases, mostly of FLHCC, a definitive diagnosis could be suggested on the basis of CD99 positivity. It is concluded that in cases of HCC, particularly in small specimens and in FLHCC, immunostaining for CD99 will be of significant help in diagnosis.

Choroid plexus papilloma of the fourth ventricle.

A 14 year old girl presented with a 6 months' history of headache with vomiting, ataxia and cerebellar signs. CT and MRI revealed obstructive hydrocephalus secondary to a large fourth ventricular mass which enhanced markedly on contrast administration. Histopathology of the resected mass revealed papillary structures lined by cuboidal cells confirming choroid plexus papilloma.