Aggressive primary hepatic epithelioid hemangioendothelioma: a case report and literature review

Abstract

A new case of epithelioid hemangioendothelioma is reported to have occurred to a 67-year-old patient who consulted for right-sided chest pain. The work-up showed multiple right pulmonary lesions associated with bilateral moderate pleural effusion and left-sided pleural thickening and three hypodense nodules in the right lobe of the liver, peritoneal thickening, ascites, and multiple vertebral lytic lesions. The diagnosis of an epithelioid hemangioendothelioma was concluded through a histological examination of a computed tomography scan guided biopsy of the liver. The patient received a primary mono-chemotherapy with Adriamycin (75 mg/m2 every three weeks) and intravenous bisphosphonates without response and general status impairment. The patient died after 16 months of follow-up.

Laparoscopic resection of hepatic epithelioid hemangioendothelioma: report of eleven rare cases and literature review

“…Primary HEHE refers to the invasion of the liver by a tumor originating in the liver mesenchyma rather than in other organs. HEHE consisting of a single lesion is considered to be the early stage of epithelioid hemangioendothelioma [ 3 ]. HEHE develops more frequently in males than in females, and appears to be associated with infections by hepatitis B- or C-viruses [ 10 ].…”

“…In the majority of HEHE patients, multiple nodules are secondary and result from a systemic spreading of the disease. Epithelioid hemangioendothelioma occurs most frequently in the liver [ 2 ], lung [ 3 ], and bone [ 4 ]. HEHEs are extremely rare borderline tumor, while primary single nodule HEHE is even rarer due to its high metastatic potential.…”

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

“…Initial symptoms have included pain in the right upper abdomen, ascites, weight loss, anorexia and jaundice in the late stage[ 3 ] as well as portal hypertension[ 2 ]. Some cases even presented with liver failure caused by a very large tumor[ 14 ], spontaneous rupture of the tumor[ 15 ] or corresponding symptoms caused by metastatic lesions, such as pleural effusion[ 16 ]. The tumor may appear as a single or multifocal tumor, and cases with a single lesion accounted for only 13%-18% of HEHE cases in the study by Mehrabi et al[ 3 ].…”

<p>Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?</p>

“…However, in the few cases reported and treated with Doxorubicin; best response ranged from PR to SD or PD. 10,18,39,40 A possible explanation for the response of the two cases of metastatic hEHE described herein; may be the biologic behavior of this tumor type, which progresses slowly. Therapeutic targeting of angiogenesis appears important; supporting a potential benefit of Olaratumab addition to the therapeutic approach of this rare tumor.…”