Disseminated Multifocal Epithelioid Haemangioendothelioma- A Multimodality Approach for Diagnosis
Journal Title: Journal of Clinical and Diagnostic Research - Year 2018, Vol 12, Issue 11
Abstract
ABSTRACT Epithelioid Haemangioendothelioma (EHE) is an extremely rare vascular tumour that develops in the soft tissues or in viscera such as liver, lung, bone, brain, spleen or even the small intestine. The tumour has an intermediate malignant potential. Hepatic Epithelioid Haemangioendothelioma (HEHE) is often not correctly diagnosed and may be confused with benign hepatic cavernous haemangioma or even with hepatic metastatic deposits. Imaging plays an important role in accurate diagnosis of this unusual entity with Dynamic contrast enhanced CT and MR showing the characteristic diagnostic features of HEHE and assist in differentiating it from other hepatic masses such as cavernous haemangioma, cholangiocarcinoma and metastasis. The differentiation is important for appropriate therapeutic triage as once detected these mesenchymal masses cannot be ignored, albeit need to be managed aggressively. Treatment modalities for this mesenchymal tumour include hepatic resection, orthotropic liver transplantation (even in cases with known metastases), radiotherapy and chemotherapy with Adriamycin, Doxorubicin or Interferon. Present case had an innocuous and beguiling clinical presentation and hepatic, pulmonary as well as unilateral adrenal masses were detected on dynamic CT and MR imaging. Metastases and cavernous haemangioma were ruled out based on the imaging appearance and advised vascular tumour markers to confirm the diagnosis of multifocal HEHE. After this, CD34 and CD31 markers were found positive, patient was referred for hepatic resection. A biopsy was conducted, that demonstrated cords of endothelial and spindle cells with large sinusoids, typical of EHE. Patient subsequently underwent a trisegmental right lobe resection (since right hepatic lesions were more numerous), followed by chemotherapy as well as radiotherapy.
Authors and Affiliations
Shibani Mehra, KSV Abhinetri, UC Garga
Keywords
Related Articles
- EP ID EP526231
- DOI 10.7860/JCDR/2018/34682.12363
- Views 50
- Downloads 0
Agreement of Axial Length Measurements by Three Different Biometric Devices
ABSTRACT Introduction: Accurate Intraocular Lens (IOL) power calculation is crucial for spectacle independence and patient satisfaction post cataract surgery. Of the various parameters measured to calculate IOL power, Ax...
Effect of Pharmacist Mediated Counselling on Knowledge, Attitude and Practice (KAP), Health Related Quality of Life (HR-QoL) and Glycaemic Control in Diabetic Patients on Insulin Therapy
ABSTRACT Introduction: Education of the patients regarding KAP management has shown to improve patient outcomes in various health care settings across the world. Aim: To assess the impact of patient counselling by the ph...
A Boy with a Congenital Sagittal Fissure of the Glans Penis Representing an Abortive Isolated Urethral Duplicatio
Images in Medicine
Prevalence, Characterisation and Diagnosis of Common Connective Tissue Diseases from Coimbatore Region in Tamil Nadu
ABSTRACT Introduction: Systemic autoimmune diseases include conditions where the immune system fails to recognise self antigens leading to production of “auto antibodies” and subsequent damage to several organs and tissu...
A Prevalence Study of Odontomas in a Brazilian Population
ABSTRACT Introduction: Odontomas are one of the most common odontogenic tumours and are classified as benign odontogenic tumours. According to radiographic, microscopic and clinical features there are two main types of o...