Hodgkin Lymphoma in Children Under 5 Years: Do They Behave Differently?

The median age of presentation for Hodgkin lymphoma (HL) is lower in developing countries with a higher proportion under 5 years of age possibly attributable to the high prevalence of Epstein-Barr virus-driven disease. It is unclear whether the clinical presentation and outcomes of this cohort are different with concern regarding late effects being most pronounced in this age group. We report the outcome of children under 5 years of age enrolled in the InPOG-HL-15-01, the first multicentric collaborative study for newly diagnosed children and adolescents with HL from India. Thirty-five (9%) of the study population was younger than 5 years with a striking male preponderance of 34:1. They were less likely to have bulky disease, mediastinal or splenic involvement. The outcomes appear to be at least as favorable as in the older patient group. Efforts need to be made to evolve treatment strategies that spare this very young cohort from potential late effects.

Enter the Matrix: Factorization Uncovers Knowledge from Omics.

Omics data contain signals from the molecular, physical, and kinetic inter- and intracellular interactions that control biological systems. Matrix factorization (MF) techniques can reveal low-dimensional structure from high-dimensional data that reflect these interactions. These techniques can uncover new biological knowledge from diverse high-throughput omics data in applications ranging from pathway discovery to timecourse analysis. We review exemplary applications of MF for systems-level analyses. We discuss appropriate applications of these methods, their limitations, and focus on the analysis of results to facilitate optimal biological interpretation. The inference of biologically relevant features with MF enables discovery from high-throughput data beyond the limits of current biological knowledge - answering questions from high-dimensional data that we have not yet thought to ask.

Utility of a limited panel of calretinin and Ber-EP4 immunocytochemistry on cytospin preparation of serous effusions: A cost-effective measure in resource-limited settings.

BACKGROUND: Differentiation between reactive, but morphologically atypical, mesothelial cells and adenocarcinoma in effusions can be problematic. Elaborate immunohistochemical panels have been devised. Techniques like DNA analysis, flow/image cytometry, and K-ras mutation analysis are research oriented and difficult to perform in routine, especially in resource-poor centers. We evaluated the efficacy of a limited two-antibody panel comprising calretinin and Ber-EP4 on cytospin and cell block preparations, in 100 effusion samples. MATERIALS AND METHODS: Fifty cases of reactive mesothelial hyperplasia and 50 cases of adenocarcinoma diagnosed by cytomorphology in ascitic/pleural fluid specimens over a 2-year period were assessed. The diagnoses were confirmed by clinical/histopathologic correlation. Cytospin smears were made in all. Cell blocks were prepared, wherever adequate fluid was available. Immunocytochemistry (ICC) for calretinin and Ber-EP4 was performed. RESULTS: Forty-five of the reactive effusion cases (90%) were calretinin reactive and Ber-EP4 negative. Among the adenocarcinoma cases, 49 (98%) were calretinin negative but Ber-EP4 positive. Thus, both calretinin and Ber-EP4 had a high sensitivity (90% and 98%, respectively), as well as a high specificity (100% and 86%, respectively). In the 21 reactive mesothelial cases, whose cell blocks were made, results were comparable to those on cytospin. However, of the 19 adenocarcinoma cases in which cell blocks were prepared, all were Ber-EP4 immunopositive except for three, which were positive on cytospin, implying false-negative results on cell blocks. CONCLUSIONS: A limited panel of two monoclonal antibodies, calretinin and Ber-EP4, may be useful in cytology, as a "primary antibody panel", for accurate diagnosis and patient management. Additionally, ICC can be performed easily on cytospin preparations, which gave results comparable to cell blocks in our study.

Validation of Accuracy of Non-Invasive Imaging Methods (Magnetic Resonance Imaging (MRI) Fat Fraction Calculation and Computed Tomography (CT) Liver Attenuation Index) for Hepatic Graft Fat Quantification in Living Liver Transplant Donors.

BACKGROUND Diagnosis of significant hepatic graft steatosis remains vital for success of any transplant program as it has an impact on donor morbidity and recipient survival. Even histopathological quantification faces limitations. The present study compared the diagnostic accuracy of CT-LAI and MRI fat fraction imaging with histopathological analysis for donor graft parenchymal fat quantification. MATERIAL AND METHODS CT-LAI and MR-FF values and histopathological fat quantification results of 273 patients were identified from electronic records of the author's institutes from September 2015 to April 2020. Data analysis was done using SPSS version 21.0. RESULTS Most participants were young with nearly equal sex distribution and significant number of overweight and obese patients. Moderate agreement and significant positive correlation were found between MR fat fraction (%) and biopsy-macrosteatosis (%). Diagnostic accuracy and negative predictive value of MRI for fat fraction calculation was high (95.24% and 98.07% for fat fraction of 10% threshold, respectively), and it further improved for fat fraction threshold of 15%. CONCLUSIONS MRI-based fat quantification calculation displayed near-perfect negative predictive values and very high diagnostic accuracy, suggesting that it can obviate the need for biopsy in patients with graft fat percentage <10% on MRI.

Suprasellar ganglioglioma presenting with diabetes insipidus in a young boy: a rare clinical presentation.

OBJECTIVE: Gangliogliomas are rare tumors composed of an admixture of glial and neuronal components. These usually occur in young patients, who present with therapy-resistant seizures. Clinical presentation of ganglioglioma with diabetes insipidus is extremely rare with only one case reported earlier in the available literature. Due to this rarity, ganglioglioma is not considered in the differential diagnosis in a patient with diabetes insipidus. CASE: A 7-year boy presented with polyuria, polydipsia, and progressive visual loss for 18 months. Investigations revealed diabetes insipidus. Radiographic studies of the brain showed a solid and cystic mass in the suprasellar region effacing the third ventricle. Intraoperatively, diffuse thickening of bilateral optic nerves and optic chiasma was noted and a diagnosis of optic glioma was considered. A biopsy of the mass was taken, which on histopathological examination showed features of ganglioglioma. The patient was referred for further radiotherapy but was lost to follow-up. CONCLUSION: Diabetes insipidus as a presenting symptom of ganglioglioma is extremely rare. This benign tumor should be kept in mind in patients with central diabetes insipidus and a suprasellar mass lesion. This report describes the second such case in the literature.

Membranous glomerulonephritis in a patient with ankylosing spondylitis: a rare association.

Ankylosing spondylitis is a chronic inflammatory disease of the vertebral joints and soft tissues. Renal involvement, apart from amyloidosis, is rare in this disorder. Of the various glomerulonephritides reported in association with ankylosing spondylitis, IgA nephropathy is the most common. Membranous glomerulonephritis occurs very rarely in patients with ankylosing spondylitis, and only four such cases have been reported in the available English literature. Due to the rarity of this association, membranous glomerulonephritis may not initially be considered in patients with ankylosing spondylitis and proteinuria. We report the case of a 29-year-old man with ankylosing spondylitis who presented with pedal edema and was detected to have nephrotic syndrome. A percutaneous renal biopsy showed features of membranous glomerulonephritis with capillary wall granular deposits of IgG and C3 on immunofluorescence and subepithelial immune complex deposits on electron microscopy. No other secondary cause of membranous glomerulopathy was found on extensive investigations. Membranous glomerulonephritis is extremely rare in association with ankylosing spondylitis, the present case being the fifth such report. The exact relationship of these two entities (etiological or coincidental) still needs to be elucidated. The occurrence of this rare association needs to be recognized and differentiated from other more common causes of renal involvement in ankylosing spondylitis.

An efficient code for environmental sound classification.

An efficient code for classifying environmental sounds is described that exploits a recent significant advance in signal processing known as compressed sensing (CS) [cf.Donoho, D. (2006). IEEE Trans. Inf. Theory 52, 1289-1306]. CS involves a novel approach to sampling in which the salient information in signals is recovered from the projection onto a small set of random basis functions. The advantage of the random basis over traditional Fourier or wavelet representations is that it allows accurate classification at low target-to-interference ratios based on few samples and little or no prior information about signals.

Stroma-predominant Wilms tumor with teratoid features: report of a rare case and review of the literature.

We describe the case of a 4-year-old boy with a unilateral right-sided Wilms tumor with stromal predominance and teratoid features, which was localized to the kidney and hence, was completely resected. The pediatric surgeon and pathologist, both should be aware of this rare appearance of nephroblastoma so as to avoid the highly toxic chemotherapy and radiotherapy in such patients.

Detection-theoretic analysis of the observer-based psychophysical procedure.

The observer-based procedure, used in research applications to measure the hearing sensitivity of young infants, is analyzed within the framework of a two-stage (infant-judge) detection model involving a minimum number of assumptions. The model is taken to develop convergence theorems useful for estimating the number of judges and/or trials required to achieve a desired level of accuracy using the procedure. The model is also used to consider ways in which bias in estimates, known to be associated with the procedure, might be evaluated and reduced. Finally, a method is proposed by which the results of the analysis and different procedural variations designed to improve estimates can be evaluated empirically.

Cutaneous angiosarcoma in a patient with xeroderma pigmentosum.

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disorder characterized by photosensitivity, cutaneous pigmentary changes, premature skin ageing and development of various cutaneous and internal malignancies at an early age as a result of a defect in nucleotide excision repair following ultraviolet light exposure. Cutaneous angiosarcomas are aggressive neoplasms that are rarely associated with XP. In this communication, we report the case of a 40-year-old male patient with XP who developed an angiosarcoma of the face and discuss the implications of this association in view of recent developments in this field.

Hepatic tuberculosis mimicking Klatskin tumor: a diagnostic dilemma.

Tubercular involvement of liver is rare and usually occurs in association with pulmonary or miliary tuberculosis, as diffuse involvement without recognizable pulmonary tuberculosis or rarely in a localized form, which presents as a tuberculoma or tubercular abscess. We report the case of a 22-year-old boy presenting with features of obstructive jaundice and a clinico-radiological picture highly suggestive of a perihilar cholangiocarcinoma (Klatskin tumor), but found to have tubercular involvement of porta hepatis. We review the literature on this unusual presentation, highlight the considerable diagnostic challenge such cases can pose, and also emphasize the need to consider tuberculosis in differential diagnosis of lesions involving the porta hepatis, particularly in areas endemic for the disease.

Oncocytic carcinoid tumor of the lung: a case report of diagnostic pitfall in filter membrane preparation of bronchial washings.

BACKGROUND: Oncocytic carcinoid tumor of the lung is a rare variant of pulmonary carcinoid. This report describes the morphologic appearance of this rare tumor on filter membrane preparation along with potential pitfalls. CASE: A 49-year-old woman presented with cough and expectoration. On chest radiograph a mass lesion was seen in the upper zone of the right lung. Bronchial washings were sent for evaluation. On filter membrane (Millipore) preparation of bronchial washing the possibility of a non-small cell carcinoma, possibly squamous, was suggested. Right upper lobectomy was subsequently performed and a histologic diagnosis of oncocytic carcinoid given. The cytomorphologic features of this tumor on the Millipore preparation were reviewed. CONCLUSION: Differential diagnosis of oncocytic carcinoid should be kept in mind while assessing cytologic material when tumor cells show abundant granular cytoplasm and prominent nucleoli. Oncocytic carcinoid also must be differentiated from oncocytoma and granular cell tumor. Immunocytochemistry and electron microscopy are useful in confirming the diagnosis.

Acral and palmo-plantar hyperpigmentation in a patient with disseminated hepatocellular carcinoma.

Capecitabine (5-fluorouracil prodrug) is being evaluated for the management of hepatocellular carcinoma, and is associated with a peculiar skin reaction called hand and foot syndrome (HFS). We describe one patient with HCC and drug-induced HFS.

Giant fibroadenoma with massive infarction masquerading as malignancy: a case report.

Fibroadenoma is a common benign breast lesion that usually affects women in their second and third decade of life and usually present as small mobile painless lump. However, it is important to recognize that a small percentage have been shown to progress to giant fibroadenomas. Giant fibroadenomas can undergo infarction leading to significant morbidity and difficulty to distinguish from the more aggressive phyllodes tumors or carcinoma. We report an interesting case of giant fibroadenoma (17 × 11 × 7 cm) with massive infarction during lactation, further complicated by lactational mastitis with close resemblance to cystosarcoma phyllodes. Detailed clinical evaluation and proper investigation in the form of USG breast and PET-CT scan helped us to delineate the nature of pathology. Simple excision of lesion with curvilinear incision was done with excellent cosmetic outcome. Histopathologic features were consistent with fibroadenoma (giant) with large areas of infarction. Fibroadenoma breast has varied clinical presentations. The course of disease may be complicated by pregnancy, lactation and inflammatory processes. Massive infarction may make the picture more dubious and masquerade with malignant transformation altering our treatment decision. The presence of necrosis on core biopsy or intra-operative finding should be cautiously interpreted and is not itself a sign of malignancy. Detailed clinical evaluation and comprehensive workup should be done before embarking on radical treatment.

A case of Philadelphia chromosome positive acute lymphoblastic leukemia with partial trisomy of chromosome 1q involving chromosome 13 as the acceptor-A novel cytogenetic finding.

The Philadelphia (Ph) chromosome is infrequently found in acute lymphoblastic leukemia and is associated with poor prognosis. We present a case of Ph chromosome positive B cell-acute lymphoblastic leukemia with the partial trisomy of chromosome 1q involving chromosome 13 as the acceptor which has never been reported in the English literature. Jumping translocation (JT) of chromosome 1 is rare and is associated with disease progression and poor prognosis. Herein, we report the first case of Ph chromosome positive B cell-acute lymphoblastic leukemia with coexisting jumping translocation of chromosome 1 leading to trisomy of chromosome 1q. Dismal prognosis associated with synchronous presence of a Ph chromosome and JT leading to a partial trisomy of chromosome 1q may carry significant prognostic and therapeutic implications. This may be an incidental finding and further studies with large patient cohorts and clinical outcomes are needed to definitively determine the predictive value of this cytogenetic finding.

Accelerated Mini-batch Randomized Block Coordinate Descent Method.

We consider regularized empirical risk minimization problems. In particular, we minimize the sum of a smooth empirical risk function and a nonsmooth regularization function. When the regularization function is block separable, we can solve the minimization problems in a randomized block coordinate descent (RBCD) manner. Existing RBCD methods usually decrease the objective value by exploiting the partial gradient of a randomly selected block of coordinates in each iteration. Thus they need all data to be accessible so that the partial gradient of the block gradient can be exactly obtained. However, such a "batch" setting may be computationally expensive in practice. In this paper, we propose a mini-batch randomized block coordinate descent (MRBCD) method, which estimates the partial gradient of the selected block based on a mini-batch of randomly sampled data in each iteration. We further accelerate the MRBCD method by exploiting the semi-stochastic optimization scheme, which effectively reduces the variance of the partial gradient estimators. Theoretically, we show that for strongly convex functions, the MRBCD method attains lower overall iteration complexity than existing RBCD methods. As an application, we further trim the MRBCD method to solve the regularized sparse learning problems. Our numerical experiments shows that the MRBCD method naturally exploits the sparsity structure and achieves better computational performance than existing methods.