Disseminated Multifocal Epithelioid Haemangioendothelioma- A Multimodality Approach for Diagnosis

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.

Keywords

• black target sign
• diffusion magnetic resonance
• dynamic contrast enhanced computed tomography
• white target sign