Central liponeurocytoma: case report and review of literature.

The lipomatous differentiation occurring within neurocytoma has more frequently been reported in neurocytoma of cerebellar origin as case reports and short series. This case report describes the clinical, radiological, histopathological and ultrastructural findings of supratentorial central neurocytoma with lipomatous differentiation in a 45-year-old female. Magnetic resonance imaging (MRI) revealed an intraventricular lesion in the right trigone which was isointense on T1 and hyperintense on T2 weighted images. There were small cystic areas and it enhanced brilliantly and homogenously on contrast. The rare lipomatous differentiation occurring in neurocytoma in the supratentorial location lends it a distinct morphological profile and thus recommends its designation as "central liponeurocytoma".

Vaccination with a novel recombinant Leishmania antigen plus MPL provides partial protection against L. donovani challenge in experimental model of visceral leishmaniasis.

The acquisition of immunity following subclinical or resolved infection with the intracellular parasite Leishmania donovani suggests that vaccination could prevent visceral leishmaniasis. The characteristics and in vitro stimulating capability of the recombinant proteins expressed by previously identified clones on the basis of their capacity to stimulate an indigenously established Leishmania-specific cell line leading to high level of IFN-gamma suggested these to be potential candidates for immunoprophylaxis against leishmaniasis. In this study, we investigated the protective efficacy of purified recombinant proteins from two of the identified cDNA clones along with the adjuvant MPL, in a hamster model of experimental leishmaniasis. We demonstrate here that the immunization of animals with one of the recombinant proteins (rF14) having 97% similarity to C1 clone of L. chagasi ribosomal protein gene P0 (rLiP0) along with MPL provided partial protection against the virulent challenge of L. donovani. The absence of antigen-specific lymphoproliferative responses in these immunized animals may be responsible for the lack of complete and long-lasting protection.

Cutaneous Rosai-Dorfman disease: presenting as massive bilateral eyelid swelling.

Rosai-Dorfman disease is a rare, benign histiocytic proliferative disorder, which commonly affects the lymph nodes. Cutaneous Rosai-Dorfman disease is a rare form of Rosai-Dorfman disease limited to the skin. We report a case of cutaneous Rosai-Dorfman presenting with bilateral eyelid swelling in a child.

Expression of interferon-gamma- inducible protein-10 and its receptor CXCR3 in chronic pancreatitis.

AIM: The role of CXC chemokine, interferon-gamma-inducible protein-10 and its receptor CXCR3 in pathophysiology of chronic pancreatitis (CP) is not very clear. Hence, this study was carried out to analyze the expression of CXCL10 and CXCR3 in CP tissues. METHODS: Pancreatic tissues from 25 histopathologically graded CP cases (11 alcohol associated CP, 5 confirmed idiopathic and 9 of undefined nature) and 10 normal cases were studied. Tissues were subjected to real-time PCR, immunohistochemistry, and Western blot analysis for CXCL10 and CXCR3 expression. RESULTS: Real-time (RT)-PCR revealed increased expression of CXCL10 (13-fold) and CXCR3 (7-fold) in CP tissue. The immunohistochemistry and Western blot analysis of the same showed significant increased protein expression and correlated well with the histopathological grades. The CXCL10 was localized mainly in the cytoplasm of pancreatic acinar cells and expression increased from grade I to grade II and declined in grade III while no expression was recorded in normal. The CXCR3 was expressed strongly at the acinar cell membrane in CP as compared to normal. Further, comparative analysis by semiquantitative RT-PCR analysis was performed for other CXC/CC chemokines (CXCL9, CXCL11, CCL3, CCL4, CCL5) and receptor (CCR5) which revealed their upregulation in the diseased state. CONCLUSION: The existence of CXCL10 and CXCR3 with other CXC/CC chemokine signature in CP is suggestive of their vital role in the progression of chronic inflammation.

Unusual malignant tracheal tumour presenting with acute airway obstruction.

A 25-year-old male presented with features of acute airways obstruction. He was diagnosed to have a lower tracheal mass with near total tracheal obstruction and complete obstruction of the left main bronchus. The tumour was resected successfully using a two-step method of ventilation. Histopathology of the mass revealed it to be a spindle cell sarcoma. Subsequently, local irradiation and systemic chemotherapy was given.

Demyelinating disease simulating brain tumours: a histopathologic assessment of seven cases.

BACKGROUND: Demyelinating diseases can present as space occupying lesions with in the brain. It is clinically and radiologically difficult to differentiate them from primary neoplasms. Histopathologically they mimic astrocytic neoplasms closely and identifying these lesions correctly has a profound impact in treatment and prognosis of these patients. AIMS AND OBJECTIVES: The objective was to determine the histopathologic features of such acute focal demyelinating disease that clinically presented as brain tumors. MATERIAL AND METHODS: Seven cases were included for the study. Detailed histopathological examination including stains for myelin and axon were performed. The histopathological keys in arriving at the right diagnoses included a well demarcated lesion that contains uniform distribution of foamy macrophages in the absence of any associated coagulative necrosis, sheets of gemistocytic astrocytes in the white matter that show well-formed processes, perivascular chronic inflammatory cell infiltration and total absence of myelin with relative preservation of axons within these areas. CONCLUSION: The degree of suspicion (clinical, radiological and histopathological) should be high to diagnose these group of lesions. The above-mentioned diagnostic keys should help in arriving at the correct histopathological diagnoses of such cases.

Central neurocytoma: a clinico-pathological study of eight cases.

Central neurocytomas are benign neuronal tumours generally found in the lateral or third ventricles. They are rare, comprising < 1% of all brain tumours. It is frequently confused with other tumours of the central nervous system particularly oligodendroglioma. The present study was done to analyse the histopathological features including immunohistochemical profile of these rare tumours. Eight cases were taken up for the study. Seven of the cases had an intraventricular location and one was located outside the ventricles. Increased intracranial pressure was the most common presenting symptom. Microscopically all tumours were composed of small uniform cells with perinuclear halos and regular round nuclei. The tumour in extraventricular location showed atypical features. Immunohistochemistry showed positivity for neuronal markers. The present series highlights the characteristic clinical and pathological findings of this rare brain tumour. Immunostaining for neuronal markers are essential for distinguishing them from other small round cell tumours of the brain.

Acute respiratory distress syndrome in disseminated tuberculosis: an uncommon association.

OBJECTIVE: To analyse clinicopathological features of acute respiratory distress syndrome (ARDS) in disseminated tuberculosis (TB) at autopsy. METHODS: A retrospective analysis of an autopsy database of disseminated TB from 1990 to 2010 was conducted. ARDS cases were assessed for histological changes of diffuse alveolar damage (DAD) and other pathological pulmonary features. RESULTS: Disseminated TB was diagnosed in 196 cases. The clinical diagnosis of disseminated TB was made in 67% of cases. Of the 196 cases, 10 met the clinical criteria for ARDS, 60% of whom showed histological evidence of DAD. One case of DAD was diagnosed on histology alone. DAD was thus found in 7/196 cases of disseminated TB. Other pulmonary changes included necrotising granulomas (n = 10), tuberculous bronchopneumonia (n = 4), tuberculous vasculitis (n = 3), infarction (n = 1) and aspergilloma (n = 1). Histopathological diagnosis other than DAD was found in 4/10 cases and disseminated TB was presumed clinically in only 4/10 cases of ARDS. CONCLUSION: Disseminated TB may be clinically missed and diagnosed only post mortem. Disseminated TB is a relatively uncommon cause of ARDS; however, it should always be presumed clinically as it is a potentially treatable cause. DAD is a rare histological feature of disseminated TB and there may not always be a clinicopathological correlation between ARDS and DAD.

Fatal acute disseminated encephalomyelitis following treated snake bite in India.

Snake bite is an important cause of mortality and morbidity in India, with an estimated 35,000 to 50,000 fatal bites occurring annually. The neurological consequences of snake bite are predominantly the result of inhibition of neuromuscular transmission. We describe the first documented case of autopsy proven acute disseminated encephalomyelitis following treated snake bite in a young female.

Acetylcholinesterase histochemistry (AChE)--A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease.

BACKGROUND: Hirschsprung's disease (HD) is an anomaly characterized by the absence of myenteric and submucosal ganglion cells (GC) in the distal alimentary tract. Diagnosis of HD is made by the absence of GC and missing out on even a single ganglion cell can be very devastating. Acetylcholinesterase (AChE) histochemistry, done on frozen sections is said to be a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD. METHODS: To assess this, 73 samples from 42 suspected/known cases of HD were subjected to frozen section analysis with rapid haematoxylin and eosin, toluidin blue stain along with AChE histochemistry. The remnant sample was paraffin embedded for routine haematoxylin and eosin staining. RESULTS: On frozen section analysis, 33 samples showed absence of ganglion cells, AChE histochemistry showed a positive staining pattern in 17 samples and paraffin embedded routine, H&E stained sections showed absence of ganglion cells in 19 samples. Sensitivity and specificity of both tests ie frozen section rapid H&E/AChE histochemistry in the diagnosis of HD, were calculated taking paraffin embedded H&E stained sections as the gold standard. Sensitivity of frozen section rapid H&E in the diagnosis of HD is 57.57 % and specificity is 79.10 %. The p-value is <0.0001, which is significant. The sensitivity of AChE histochemistry in the diagnosis of HD is 90.47 % and specificity is 96.36 %. The p-value is <0.0001, which is significant. CONCLUSIONS: Acetylcholineesterase (AChE) histochemistry is a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD. It acts as a double check in the diagnosis of HD.

Intraglomerular metastasis: a necropsy study.

Intraglomerular metastasis is a rare phenomenon. Four cases are reported here. The primary malignancies in these four cases were squamous cell carcinoma of the lung, adenocarcinoma of pancreas, haematological malignancy with a malignant intrapulmonary teratoma, and undifferentiated pleural mesothelioma. There was no significant renal functional impairment in any case except for mild proteinuria in one. Histopathology showed tumour cells in the mesangium, in the glomerular tufts replacing the endothelial cells, as well as in an extracapillary location replacing the parietal cells. In one case, tumour cells formed crescent-like structures. Such tumour deposits were better appreciated with special stains. The presence of intraglomerular metastasis indicates dissemination of the malignancy and hence a poor prognosis even though there is no significant impairment of renal function.

Central nervous system complications in renal transplant recipients in a tropical environment.

Renal transplant recipients are at risk of developing various infectious and non-infectious complications affecting the central nervous system (CNS). There is paucity of data regarding the spectrum of CNS complications and the epidemiology of infective agents varies according to geographical location. We retrospectively studied the spectrum of CNS complications seen in 792 renal allograft recipients followed up at this tertiary care centre in north India over a 19-year period. Autopsy findings of 78 allograft recipients who died in the hospital were also reviewed and included. The brain was examined in 22 of these patients. Overall, 79 (10%) patients developed some form of CNS dysfunction with a mortality rate of 60.8%. CNS infections occurred in 31 renal allograft recipients (3.9% of total) and accounted for the largest group (39.2%). Fungi were the commonest etiological agents (21 patients) and were associated with a 70% mortality, with cryptococcal meningitis occurring in 12, mucormycosis in six, aspergillosis in one, and other unusual fungal infections in the remaining two patients. All patients with mucormycosis had a fatal outcome. The second largest group comprised of patients with non-uremic encephalopathies (23 patients, 29.1%) with metabolic encephalopathy occurring in 13, toxic encephalopathy in nine and hypertensive encephalopathy in one patient) and was associated with an overall mortality rate of 60.9%. Cerebrovascular accidents occurred in 12 patients (15.2%) and were associated with a mortality of 91.7%. Other CNS complications included treatment related complications in four (5.1%), primary CNS lymphomas in three (3.8%), and miscellaneous complications in six patients (7.6%). Patients with non-cryptococcal fungal infections of the CNS, hepatic and toxic encephalopathy and those with cerebrovascular accidents had the worst outcome. There was no relationship between the development of infection or stroke and the type of maintenance immunosuppression used. We conclude that complications involving the CNS occur in 10% of all renal transplant recipients and are associated a with high mortality, warranting early diagnosis and aggressive treatment.

Aseptic cerebral venous thrombosis associated with idiopathic ulcerative colitis: a report of two cases.

Two cases of cerebral venous thrombosis with ulcerative colitis with complete autopsy findings are reported. The diagnosis of cerebral venous thrombosis with ulcerative colitis was made during life in one case and at autopsy showed active chronic ulcerative colitis with recent cerebral venous thrombosis with cerebral infarction. In the other case active chronic ulcerative colitis and old cerebral venous thrombosis with cerebral infarction were diagnosed at autopsy. Although rare the importance of recognising cerebral venous thrombosis as a complication of ulcerative colitis has been emphasised in view of the high mortality of this complication.

Chondrosarcoma of calcanaeum in a 12-year-old male patient: a case report.

Chondrosarcoma is distinctly uncommon and tends to be located often in the extremities in young patients more than in its adult counterpart. Cartilaginous tumors involving the small bones of the hands and feet are benign tumors such as enchondromas, chondromyxoid fibroma (CMF), and chondroblastoma. Chondrosarcomas involving calcanaeum in young adults are largely covered in the literature as single case reports. A young 12-yr-male patient presented with complaints of pain and mild swelling in the ankle. Radiological examination revealed a dense irregular lesion in the calcanaeum. Fine-needle aspiration was performed and a possibility of chondrosarcoma was suspected, which later was confirmed on histopathological examination. The cytopathologist should be aware of the occurrence of malignant chondroid tumors in the younger age group at this rare site. Correlation to radiological findings is essential in these cases.

Desmoplastic ameloblastoma: a case report with fine-needle aspiration cytologic findings.

A case of desmoplastic ameloblastoma of the maxilla in a 25-yr-old woman is presented. Smears prepared from fine-needle aspiration cytology showed two populations of cellular elements: cohesive epithelial clusters with basaloid morphology present, mostly in bidimensional, irregularly outlined clusters with ill-formed palisading of nuclei at the periphery in some, and a mesenchymal component represented by 1) a sparse chunk of moderate-sized tissue fragments made up of spindle- or ovoid-shaped nuclei entrapped in mesenchymal matrix, and 2) many dissociated naked oval-to-spindle-shaped nuclei. The presence of epithelial and mesenchymal components and their benign nature lead us to consider the possibility of benign odontogenic tumors 1) of epithelial origin, such as ameloblastma with a stromal component, e.g., desmoplastic ameloblastoma; 2) of mesenchymal origin, such as odontogenic fibroma; and 3) of mixed epithelial and mesenchymal origin, such as ameloblastic fibroma. Excision and histopathological examination of this lesion confirmed the diagnosis of desmoplastic ameloblastoma. In the given clinical setting and radiological examination, the above cytological features suggest a benign odontogenic tumor, rather than precisely diagnosing any of the entities mentioned above. However, it is important to distinguish between these, since the treatment varies accordingly. The differential diagnosis is discussed.

Comparison of cytologic features of giant-cell tumor and giant-cell tumor of tendon sheath.

The cytologic features in twelve cases of giant-cell tumor (GCT) and five cases of giant-cell tumor of tendon sheath (GCTTS) diagnosed by fine-needle aspiration cytology (FNAC) are described. All of these cases were histopathologically confirmed. The aspirates of GCT are composed of a dual population of mononucleated spindle cell and multinucleated giant cells. The peripheral adherence of giant cells to the spindle cell is the feature of diagnostic significance in GCT. In GCTTS, the aspirate consists of a polymorphic population composed of mononuclear histiocyte-like cells, hemosiderin laden macrophages, foamy macrophages, and a few multinucleated giant cells. FNAC can be used as a diagnostic tool for an early and accurate detection of these two giant cell-rich lesions, since the cytologic features when evaluated in conjunction with the clinical and radiologic features are sufficiently diagnostic.