Introduction: Gliosarcoma is a rare but clinically significant high grade malignant tumor considered as a variant of glioblastoma. However, inspite of resembling glioblastoma in its clinical course, gliosarcoma is known to show a distinct and characteristic biphasic architecture consisting of both mesenchymal (sarcomatous) and glial components making it distinct from glioblastomas on histopathology. Case Presentation: We present a case of a 56 year old female who presented with chief complaints of inability to speak and seizures. While clinical and radiological examination pointed towards a broad differential of a high grade glioma, strategic use of histopathological and immunohistochemical examination led to a definitive diagnosis of a rare but high grade Gliosarcoma.Conclusion: Our case highlights the importance of recognizing gliosarcoma as a separate entity on histopathology by highlighting its sarcomatous and mesenchymal components with strategic use of special stains such as reticulin and immunohistochemical markers such as GFAP and Vimentin. This is essential for predicting patient outcome and clinical course of the disease thus aiding the refinement of therapeutic strategies.
Background: The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) was introduced in 2018 to provide a uniform, evidence-based framework for reporting salivary gland FNAC, with each diagnostic category linked to an estimated Risk of Malignancy (ROM) and management.Methods: For each FNAC of salivary gland lesions, whether received in-house or ultrasound-guided, a comprehensive clinical examination of the patient was performed. Descriptive statistical mean and frequency (%) is used to depict the baseline characteristics of demographic data of the study participants. ROM were calculated in each MSRSGC categories and performance analysis with Sensitivity, Specificity, Positive Predictive Value, Negative Predictive Value and Diagnostic accuracy. Result: This study evaluated 104 salivary gland FNAC cases using the MSRSGC and correlated cytological findings with histopathology wherever available. Benign neoplasms constituted the largest diagnostic category, followed by malignant and non-neoplastic lesions. A male predominance was observed (69.2%), with a peak incidence in the 61–70-year age group. Radiology–cytology concordance was noted in 82.5% of evaluated cases. Histopathological correlation was available in 44 of 104 FNAC cases, as only these patients underwent subsequent surgical excision/biopsy at our institution. The risk of malignancy was calculated for each MSRSGC Categories Conclusion: Overall, MSRSGC is a simple, user-friendly, and reliable reporting system that enhances communication between pathologists and clinicians and aids in appropriate patient management.
Introduction: Hepatocellular adenoma (HCA) is rare benign liver neoplasm. HCA can be single or multiple, and hepatic adenomatosis (HA) is defined as the occurrence of more than or equal to ten HCAs. Hepatocyte nuclear factor 1 alpha inactivated HCA (HHCA), Beta-catenin activated HCA (β HCA) Inflammatory HCA (IHCA), β catenin activated inflammatory adenoma (β IHCA) and Unclassified HCA (UHCA) are types based on correlation of genetic mutation and histological features.Materials and Methods: A 10 yrs retrospective study of HCA was done. Histological features scored as, 0, 1, 2 and 3. IHCA was diagnosed based on the presence of sinusoidal dilatation, pseudo portal tracts, and inflammatory infiltrate and any 2 or more features, with significant score were considered. β HCA was diagnosed on the any score of atypia, HHCA on the presence of steatosis with significant score and UHCA when no specific features were present. Glutamine synthetase (GS)/ β catenin stainingwas done.Results: There were 10 cases of HCA. 3 cases had more than 10 adenomas, most were well delineated (80%) and unencapsulated. There were 3 cases of HHCA, 1 of β HCA, 2 of IHCA, 2 of β IHCA and 2 of UHCA.Conclusions: Adenomas are rare liver neoplasm. The detection frequency of HA appears higher among HCA cases in India, thus further studies are essential. Though these tumors are reported to be non-capsulated, partial/complete capsule can be seen. Certain microscopic features help in classification, Immunohistochemistry/molecular testing is essential for exact typing and β catenin has low sensitivity in comparison to GS.
Background: Adult glial tumours continue to be a challenge for medicine for its poor prognostic effects. Due to its invasiveness and resistance to therapy it causes a lot of mortality and morbidity. The recent 5th edition WHO has changed the understanding of the spectrum of glial tumours through cytogenetic advancement. The diagnostic approach to spectrum of glial tumours has been changed due to the inclusion of molecular genetics. Molecular alterations are now the most important factor for diagnosis, classification and prognosis. Aims & Objectives: In the present study we aim to study spectrum of adult glial tumours along with immunohistochemical (IHC) study with IDH1, ATRX and p53 in a tertiary care centre. In this study we compared the histopathological features of these tumours with IHC expression to ascertain any existing correlation between two. This study also looks into any existing relation between the IHC expression and biology of the tumour. Material & Methods: This prospective study was done on 42 patients over a period of one and a half year. Patients with clinical symptoms of brain tumour were selected. The necessary neurosurgical procedure was done followed by histopathological diagnosis of the resected specimen. IHC study with IDH1, ATRX and p53 was done on the glial tumour tissue. All the data including clinic-radiological features were collected and statistical analysis was done using software.Results: Among 42 cases 66.67% of male and 33.33% female. Majority of the cases were glioblastoma. Most of the patients were affected in 4th decade of life. IDH1 expression was inversely related with WHO grade (p=.056) indicating that with increasing grade preponderance of IDH wild type increase and IDH1 mutant decreased. Significant correlation was found between histopathological subtype and IDH1 expression (p=0.000). ATRX expression showed no significant correlation with WHO grade (0.373) and with histopathological subtype. p53 expression also showed no correlation with WHO grade and histopathological subtype.Conclusion: This study was an institution-based study on 42 adult glial tumours. It was carried out during one-and-a-half-year period of January of 2021 to July of 2022. This study has showed that immunohistochemistry (IHC) could be used as a partial surrogate to genetic analysis in low resource setting. However, for more extensive analysis genetic testing is essential. IDH1 IHC in this study corroborated well with histological grade of adult gliomas.
This article reports a case of juvenile myelomonocytic proliferation. Juvenile Myelomonocytic Proliferation is an entity that includes many disorders like reactive myelomonocytic proliferation after viral sequelae, myelomonocytic proliferation associated with genetic syndromes like Noonan's syndrome and leukaemia like juvenile myelomonocytic proliferation. It mainly affects paediatric population. This case reports a similar case in a 4 year old female child who presented with generalised weakness and distention of abdomen since 15 days. She was referred from a private hospital as she was not responding to symptomatic management. On examination, patient in our centre had pallor, hepatosplenomegaly and inguinal lymphadenopathy. A complete blood count revealed Haemoglobin-5.5gm/dl, TLC-1,77,000/ul, Platelets-14000/ul. Peripheral smear with flow cytometry was reported as juvenile myelomonocytic proliferation. The patient did not have any phenotypic findings that could suggest syndromic associations. Based on above laboratory findings this was thought to be a case of juvenile myelomonocytic leukaemia and patient was given two units of Packed red cells (PRCs) for improving haemoglobin level and tablet hydroxyurea and allopurinol and IV Rasburicase with the intent of cytoreduction. Unfortunately, patient deteriorated clinically and hence further workup was not possible as the patient succumbed to death due to haemorrhagic shock following disseminated intravascular coagulation. Hence a detailed and timely evaluation of juvenile myelomonocytic proliferation is paramount for accurate diagnosis and appropriate management.
