ABSTRACT
Context: The diagnosis of giant cell tumor of bone (GCTB) is difficult in small biopsies with unusual age of presentation, location, and extensive secondary changes. Most of the GCTBs harbor H3F3A G34W mutations with a subset of cases showing alternate G34V, G34R, and G34L mutations. Objectives: To analyze the expression of anti-histone H3.3G34W antibody in different cellular components of GCTB across different locations and presentations (including the unusual ones) and validate the utility of this antibody in the diagnosis of GCTB and differentiate it from the other osteoclast-like giant-cell-rich lesions. Design: Immunohistochemistry was performed using anti-histone H3.3G34W antibody in the diagnosed cases of GCTB (136 cases of GCTB from 133 patients, including two malignant GCTBs) and other giant cell-containing lesions (62 cases). The presence of unequivocal crisp nuclear staining was considered positive. Results: Immunohistochemistry revealed unequivocal nuclear positivity in the mononuclear cells in 87.3% of the cases of GCTB. Of these, most showed diffuse expression with moderate to strong intensity staining. The positive staining was restricted to the nuclei of mononuclear cells with the nuclei of osteoclastic giant cells being distinctly negative. In addition to conventional GCTBs, two cases each of multicentric and malignant GCTB showed positive staining. The other giant-cell containing lesions were distinctly negative. The present study showed a sensitivity of 87.3% with specificity and positive predictive value of 100%. Conclusion: The anti-histone G34W antibody is a highly sensitive and specific marker for the diagnosis of GCTB and differentiating it from its mimics. The positive staining is restricted to the mononuclear cell component of GCTB with sparing the osteoclastic giant cells further reiterating the fact that the mononuclear stromal cells are the true neoplastic component of GCTB.
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Background: Juvenile idiopathic inflammatory myopathies (JIIM) are rare and heterogeneous. Subtype identification is important for treatment. Materials and Methods: Patients below 18 years diagnosed as idiopathic inflammatory myopathy (IIM) according to the Bohan and Peter criteria between January 2010 and May 2015 were evaluated with muscle biopsy in the four domains: muscle fiber, inflammation, connective tissue, and vascular, with basic panel of histochemical stains as per recommendations of the European Neuromuscular center (ENMC) workshop 2015. Immunohistochemistry with CD 31 was done to assess capillary density. Results: JIIM constituted 15.25% of IIM with juvenile dermatomyositis (JDM) being the most common subgroup (24/27) followed by juvenile overlap myositis (JOM) (3/27) in association with systemic lupus erythematosus (2) and systemic sclerosis (1). Muscle biopsy in JDM was characterized by perifascicular atrophy, necrosis, degeneration, and regeneration in all and the other features included perivascular inflammation (21), lymphoid aggregates (2), mitochondrial abnormalities (9), sarcoplasmic vacuoles (6), capillary dropout (5), capillary dilatation (6), and perimysial fibrosis (14). JOM was characterized by auto-antibodies and perivascular inflammation. Conclusion: JIIMs were rare and JDM was the most common subtype. Muscle biopsy evaluation as per ENMC criteria characterized the subgroups.
ABSTRACT
Pericardial mesotheliomas are rare tumors which often present with features of constrictive pericarditis. We present clinical, imaging, histological, and immunohistochemical findings of three cases presenting with chronic constrictive pericarditis. Two of these cases were initially treated as tuberculous pericarditis. Histologically, all the three cases were of an epithelioid type and showed positivity for more than one mesothelial markers. Two patients had a fatal outcome, and one was lost to follow-up.
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BACKGROUND AND OBJECTIVE: Spontaneous intracerebral haemorrhage (ICH) is more common in Asia than in western countries, and has a high mortality rate. A simple prognostic score for predicting grave prognosis of ICH is lacking. Our objective was to develop a simple and reliable score for most physicians. MATERIAL AND METHOD: ICH patients from seven Asian countries were enrolled between May 2000 and April 2002 for a prospective study. Clinical features such as headache and vomiting, vascular risk factors, Glasgow coma scale (GCS), body temperature (BT), blood pressure on arrival, location and size of haematoma, intraventricular haemorrhage (IVH), hydrocephalus, need for surgical treatment, medical treatment, length of hospital stay and other complications were analyzed to determine the outcome using a modified Rankin scale (MRS). Grave prognosis (defined as MRS of 5-6) was judged on the discharge date. RESULTS: 995 patients, mean age 59.5 +/- 14.3 years were analyzed, after exclusion of incomplete data in 87 patients. 402 patients (40.4%) were in the grave prognosis group (MRS 5-6). Univariable analysis and then multivariable analysis showed only four statistically significant predictors for grave outcome of ICH. They were fever (BT > or = 37.8 degrees c), low GCS, large haematoma and IVH. The grave prognosis on spontaneous intracerebral haemorrhage (GP on STAGE) score was derived from these four factors using a multiple logistic model. CONCLUSION: A simple and pragmatic prognostic score for ICH outcome has been developed with high sensitivity (82%) and specificity (82%). Furthermore, it can be administered by most general practitioners. Validation in other populations is now required.