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New Delhi, India

Chakrabarti I.,North Bengal Medical College | Majumdar K.,Academic Block | Giri A.,North Bengal Medical College
Journal of Pediatric Neurosciences

Medulloepitheliomas (WHO grade IV) are rare, malignant embryonal tumors of pediatric population, classified under the central nervous system (CNS) primitive neuroectodermal tumors (PNET). Histologically, these tumors are characterized by neoplastic neuroepithelium recapitulating the embryonic neural tube. We describe a rare case of infratentorial medulloepithelioma with divergent differentiation in a 1-year-old male child who presented with headache, vomiting, and seizures. Histopathologic examination of the excised tumor revealed the characteristic neuroepithelium, along with other areas showing primitive neuroectodermal (blastemal) cells in sheets, ependymoblastic rosettes, and nodular areas of neuronal differentiation. Possibly, this proliferating immature neuroepithelium is the cause of poor outcome in medulloepitheliomas. Due to the rarity of these tumors, it remains to be established whether infratentorial location or tumors with divergent differentiation are also predictors of adverse prognosis. Source

Tyagi I.,Academic Block | Majumdar K.,Academic Block | Mehta S.,Artemis Health | Batra V.V.,Academic Block
Brain Tumor Pathology

Glioosarcomas represent a rare entity of intrinsic CNS neoplasms consisting of glial and sarcomatous elements; they account for 2 % of glioblastomas. There have been few reports of metaplastic osseous transformation in gliosarcomas. Here we report a rare case of gliosarcoma with metaplastic osteoid in a 57-year-old male patient. Magnetic resonance imaging revealed a large solid cystic heterogeneous mass lesion in the left temporal lobe with peri-tumoral oedema and areas of calcification. Histology revealed a grade IV tumour with neoplastic glial and mesenchymal components with myxoid change and areas of necrosis. The sarcomatous areas contained regions of benign osteoid formation. The histogenesis of the osseous element in gliosarcomas is not well understood, and could be a result of a metaplastic change or divergent differentiation from a common progenitor cell. The osseous tissue may be in the form of newly formed osteoid, benign bone tissue formation with lacunae and osteoblastic rimming, or unequivocally malignant tissue resembling an osteosarcoma. © 2012 The Japan Society of Brain Tumor Pathology. Source

Majumdar K.,Academic Block | Sakhuja P.,Academic Block | Rastogi A.,Institute of Liver and Biliary science | Gondal R.,Academic Block | Agarwal A.,G B Pant Hospital
Journal of Cancer Research and Therapeutics

Inflammatory myofibroblastic tumor (IMT) has been described as a pseudosarcomatous proliferation of spindled myofibroblasts admixed with lymphoplasmacytic cells. The various terminologies like inflammatory pseudotumor, plasma cell granuloma, and inflammatory myofibrohistiocytic proliferation, used to describe this entity, highlight the controversial etiopathogenesis of this relatively indolent neoplasm. IMT has now been described in different anatomic locations. However, cases occurring in the gastrointestinal tract are rare with very few cases described in the appendix. We present a case of inflammatory myofibroblastic tumor appendix with mucosal dysplasia in a 41-year-old male, presenting with abdominal pain and lump in the right iliac fossa. Aspiration cytology yielded few atypical epithelial cells and spindle cells in a mucinous background, suggesting the possibility of pseudomyxoma peritonei. Awareness of IMT appendix with rare presence of mucosal dysplasia may help in preventing overzealous resection, especially in situations that on preoperative evaluation may suggest malignancy. Source

Patidar Y.,Academic Block | Gupta M.,Academic Block | Khwaja G.,Academic Block | Chowdhury D.,Academic Block | And 2 more authors.
Annals of Indian Academy of Neurology

Aims: To evaluate clinical profile and short-term outcome of psychogenic non-epileptic seizures (PNES) in Indian adult population. Setting and Design: A prospective observational study, conducted at tertiary teaching institute at New Delhi. Materials and Methods: Sixty-three patients with confirmed PNES were enrolled. The diagnosis was based on witnessing the event during video-electroencephalography (Video-EEG) monitoring. A detailed clinical evaluation was done including evaluation for coexistent anxiety or depressive disorders. Patients were divided into two groups on the basis of excessive or paucity of movements during PNES attacks. Patients were followed-up to 12 months for their PNES frequency. Statistical Analysis: Means and standard deviations were calculated for continuous variables. Chi-square and Students t-test were used to compare categorical and continuous variables respectively. Results: The mean age at onset of PNES was 25.44 years; with F:M ratio of 9.5:1. Coexistent epilepsy was present in 13 (20.63%) cases. Twenty-two patients (44%) with only PNES ( n = 50) had received antiepileptic drugs. Out of 63 patients of PNES 24 (38.1%) had predominant motor phenomenon, whereas 39 (61.9%) had limp attacks. The common features observed were pre-ictal headache, ictal eye closure, jaw clenching, resistant behavior, ictal weeping, ictal vocalization, and unresponsiveness during episodes. Comorbid anxiety and depressive disorders was seen in 62.3% and 90.16% patients, respectively. Short-term (6-12 months) outcome of 45 patients was good (seizure freedom in 46.66% and >50% improvement in 24.44% cases). Conclusion: PNES is common, but frequently misdiagnosed and treated as epileptic seizures. A high index of suspicion is required for an early diagnosis. Proper disclosure of diagnosis and management of the psychiatric comorbidities can improve their outcome. Limitation: Limited sample size and change in seizures frequency as the only parameter for the assessment of the outcome are the two major limitations of our study. Source

Majumdar K.,Academic Block | Tyagi I.,Academic Block | Saran R.K.,Academic Block | Sakhuja P.,Academic Block | Sharma A.,G B Pant Hospital
Brain Tumor Pathology

Medulloblastomas with myogenic differentiation, previously termed medullomyoblastomas, form rare variants of medulloblastomas. Occasional tumors showing combined myogenic differentiation and melanotic tubular structures have also been described. On studying the records of a tertiary-care super specialty hospital, of 80 cases of medulloblastoma in a 5-year period, 36 showed nodular islands of neuronal, three myogenic, two glial, and one melanotic differentiation. Of the three cases of medulloblastomas with myogenic differentiation, we came across an extremely rare variant of medulloblastoma showing, in addition to the predominant primitive neuroectodermal component, a single minute nodular focus of 0.8 cm diameter, with divergent differentiation into mainly myogenic, epithelial, cartilaginous, and osseous lineages. Two ependymoblastic rosettes were also identified at different foci. In conclusion, a medulloblastoma with a small nodular island of divergent differentiation into various lineages has been seldom documented in literature. Microscopic foci of multilineage differentiation may be explained by the teratoid potential of medulloblastoma, or pluripotent nature of the neural crest cells, and requires extensive sampling for detection. It remains to be confirmed through more reports whether medulloblastomas with differentiation respond to a treatment protocol similar to that of medulloblastomas. © 2012 The Japan Society of Brain Tumor Pathology. Source

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