Context:Spinal paragangliomas are rare neuroendocrine tumors of the extra-adrenal paraganglionic system.Aims:This study describes the clinicopathological features of eight cases of spinal paraganglioma and highlights the significance of important morphological features and immunohistochemistry in the diagnosis of paraganglioma at this unusual site.Material and Methods:All the cases of primary spinal paragangliomas diagnosed during the last six years (2008-2013) in the Department of Pathology at our hospital were reviewed.Results:There were six males and two females. The mean age at diagnosis was 50.4 years. All patients presented with low back pain. All tumors were located in the cauda equina or conus medullaris region. Magnetic Resonance Imaging and intraoperative appearance were that of a vascular, well-circumscribed intradural, extramedullary tumor suggestive of either schwannoma or ependymoma. All the patients underwent gross total resection of the tumor. Histopathology in five of the cases showed ‘ependymoma-like histology’ while only three cases had a predominant classic ‘zellballen’ pattern. Two cases had prominent ‘gangliocytic differentiation’. In the five cases with ‘ependymoma-like histology’, the diagnosis was confirmed on Immunohistochemistry (IHC).Conclusions:Even though relatively rare, paraganglioma should be considered in the differential diagnosis of spinal tumors and due to their clinical, radiological and histopathological similarity to schwannoma and ependymoma, the diagnosis should be based on close examination of the clinical, radiological and pathological findings.
Phosphaturic mesenchymal tumor (PMT) is a rare tumor causing oncogenic osteomalacia (OO). Most such tumors occur in soft tissue and bones of extremities and appendicular skeleton. Intracranial location and involvement of temporal–occipital bone is extremely rare. We report two unusual cases: The first was intracranial, involving the temporal bone, while the other was a skull base tumor arising from the occipital–temporal bone. Both of them presented with paraneoplastic syndrome of OO, resembled a meningioma radiologically, and underwent gross total resection of tumor. Histologically, both of them were diagnosed as PMT, mixed connective tissue variant.
Background: Non-cirrhotic portal hypertension includes primarily the cases of non-cirrhotic portal fibrosis (NCPF) and extra-hepatic portal vein obstruction, both showing no significant parenchymal disease. NCPF is a disease of uncertain etiology characterized by periportal fibrosis with small and medium sized portal vein branches involvement. Most study done in the past are clinical, hence we decided to analyse its histo-pathological features with clinical correlation. Methods: This is four and half year's retrospective study of liver biopsies of clinically diagnosed patients of NCPF. Fifteen patients of EHPVO served as controls. Biopsies were stained with Haematoxylin and eosin, reticulin and Masson's trichrome. Result: Total 978 benign specimens (excluding space-occupying lesions) received, of which 45 diagnosed as NCPF (4.6%). Most patients (68.88%) were between 20-40 years (M:F ratio 1:1.5), commonest symptoms long-standing splenomegaly (95%), anemia (78%) and variceal bleeds (42%) with duration varying from 3 months to 11 years. Commonest histopathological findings were portal tract fibrosis (77.78%), phlebosclerosis (68.89%), portal tract remnants (24.44%), more frequently seen in NCPF than EHPVO, septal fibrosis was seen in five. Follow up available in 91.11% cases showed no mortality or liver failure, but significant morbidity due to repeated episodes of variceal bleeds and hypersplenism. Conclusion: NCPF is still common in India. Distinctive features on histology are portal tract fibrosis, portal tract remnants, with phlebosclerosis which is being statistically significant when comparing with EHPVO. NCPF can show advanced fibrosis, can also present with features of decompensation mimicking cirrhosis, however its course remains stable with good long-term survival.
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