Metastatic Hepatic Epithelioid Hemangioendothelioma In 10 Years Old Boy; A Rare Case Report

OBJECTIVES Epithelioid Hemangioendothelioma is an infrequent vascular neoplasm of intermediate malignant potential oddly affecting children. It is primarily noted in soft tissues, the stomach, breast, spleen, brain, and liver. No definite risk factor is identified in children. However, following WWTR1-CAMTA1 and YAP1-TFE3 gene fusions are frequently seen in these tumours. We present a case of very rare childhood Epithelioid Hemangioendothelioma of the liver in a ten-year-old boy. A 10-year-old boy presented with abdominal pain, mild weight loss, and abnormal liver function tests. Radiologically, hepatic, pulmonary, and bony lesions are noted. The biopsy report showed Epithelioid Hemangioendothelioma confirmed by an Immunohistochemical panel. Due to the lack of facilities in our centre, palliative treatment was given to the patient. Surgical resection, liver transplant, and radiofrequency ablation were impossible due to widespread hepatic and pulmonary disease. The patient was resistant to any medical treatment. The patient died at the seventh-month follow-up. HEHE is a scarcely seen tumour with no definite management protocol. Surgical resection is the preferred treatment for resectable tumours. In non-resectable extensive bifocal tumours, like in our case, the preferred treatment is radio-frequency ablation and hepatic transplant. The overall survival is trivial due to the non-compliant nature of the disease

Histopathological Assessment of Microvascular Invasion in Hepatocellular Carcinoma Resection Specimens and its Correlation with Tumor Size and Grade

OBJECTIVES To determine the histopathological assessment of microvascular invasion in Hepatocellular Carcinoma Resection Specimens and its correlation with tumour size and grade. METHODOLOGY This retrospective cross-sectional study included the biopsy-proven Hepatocellular (HCC) case with microvascular invasion (MVI) noted in the resected specimens evaluated by two independent consultants Histopathologists. The exclusion criteria were; all patients below 18 years, unfixed autolyzed samples, and incomplete requisition-filled forms. Numerical data, i.e., patient age and tumour size, are presented as mean with standard deviation. Categorical variables, i.e., tumour size, grade, and presence or absence of MVI, were submitted as numbers with percentages. Continuous variables, i.e., tumour size and differentiation grade, were assessed using the Chi-square test. A p-value of ≤ 0.05 was considered significant. RESULTSMost patients, 34.4%, fall into the age group of 47-70. Most patients were males, 63.6%, and microvascular invasion was noted in 49.09% of cases. Most cases were of moderate to poorly differentiated tumours, 80.0%. MVI was statistically significant with the grade of the tumour. CONCLUSION Microvascular invasion is an important prognostic marker noted in a surgical resection specimen. Although the exact definition and risk stratification is unclear, survival studies have proven that MVI is associated with poor outcomes

Clinicopathological features of different Histopathological Subtypes and Stages of Wilms Tumor

Introduction: Wilms tumor is the most common pediatric renal neoplasm. Following preoperative therapy, various histological subtypes, risks and clinical stages are determined as per following SIOP 2001 protocol. To determine the clinicopathological features of different histopathological subtypes and stages of Wilms tumor after completing course of preoperative as well as postoperative chemotherapy following SIOP 2001 protocol. Methodology: This is a retrospective cross-sectional study and sample size calculated by non-probability technique. Ninety-three patients with abdominal masses in the Hematology/Oncology Department were included. After radiological and histopathological diagnosis, four weeks of preoperative chemotherapy, given to patients, followed by partial and radical nephrectomies by surgical team. Specimens received in Histopathology Department grossed and microscopically examined for different post chemotherapy histological subtypes. Further risk categorization and clinicopathological staging in accordance with SIOP 2001, done after completion of treatment. Patients called for yearly follow up for the next five years. Categorical variables are presented as frequencies and percentages. Results: Mean age of ninety-three children was 44.4 months + 30.92with predominance of males (55.9%) and more common in right sided kidney (55.9%). Majority patients completed the entire course of treatment (77.4%). Majority were intermediate risk tumors (76.3%) and most common histological subtype was Mixed Tumor subtype (23.4%). In our study majority, tumors were stage III (48.3%) and patients died due to febrile neutropenia (9.6%) Conclusion: In our study the majority of patients completed the entire course of treatment and relapse was fairly less. Patients lost to follow up after nephrectomy and deaths at home caused by febrile neutropenia were our major challenges.