Hepatic epithelioid haemangioendothelioma (HEHE) : 상피모양혈관내피종

Hepatic epithelioid hemangioendothelioma (HEHE)

 : 상피모양혈관내피종

a rare, intermediate grade malignant hepatic vascular tumur

흔하지 않은 혈관 종양이며, 중등도의 악성도를 지녔다. (Hemangioma 와 Angiosarcoma 의 중간) 양쪽 간엽에 다발성의 결절을 형성하며, 간세포암으로 오진하기도 한다. 1982년 처음 보고되었으며, 2:3 정도로 여성에게 조금더 많다. 대게는 milddle age 에 호발했다. (mean age 41.7세) 원인이 될 수 있는 위험요인은 아직 밝혀진바 없다.

There may be a greater female incidence (with reported male-to-female ratio, 3:2), with peak incidence thought to be around 30-40 year-old.

They tend to be multiple solid tumour nodules, located in a predominantly peripheral distribution, which coalescence to individual nodules. Lesions adjacent to the capsule often produce hepatic capsular retraction.

Ultrasound

Usually seen as hepatic lesions that are predominantly hyapoechoic

however, hepatic lesions can also have mixed echotexture or be predominantly hyperechoic.

CT

Typically seen as multiple hypoattenuating lesions in both hepatic lobes that coalesce to form larger confluent hypoattenuating regions in a peripheral or subcapsular distribution, with a halo or target pattern of enhancement in larger lesions. Subcapsular lesions often present with capsular retraction.

Calcifications are uncommon, but do occur on occasion.

MRI liver

T1: hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images

T2: heterogeneously increased signal intensity.

C+ (Gd): some lesions demonstrate either a peripheral halo or a target-type enhancement pattern after administration of a gadolinium-based contrast agent, with an occasional thin peripheral hypointense rim.

Ferumoxides-enhanced T2-weighted images may help physicians distinguish tumour margins

PET-CT

Moderate to intense FDG uptake

...

Treatment and prognosis

확립된 치료는 없다. 현재는 간이식 (44.8%), 항암치료나 방사선치료 (21%), 간절제 (9.4%), Supportive care (24.8%) 가 있다.

간절제술이 간이식보다 조금더 예후가 좋으나, 대부분의 경우 다발성으로 발현되므로, 간절제를 할 수 없는 경우가 대부분이다. 항암치료나 방사선치료에 반응이 좋지 않으므로, 수술이 불가능한 경우 Orthotropic liver transplantation 이 가능한 치료방법으로 고려될 수 있다. 예후인자에 대해서는 알려진바가 없으나, 증상, 고령, CA 19-9 증가 등이 불량한 예후를 시사한다는 보고가 있다.

The clinical course of these lesions can be variable with histologic analysis being of little value in predicting the clinical outcome. The overall prognosis is much more favourable than other hepatic malignancies.

Radical surgical resection or orthoptic liver transplantation are considered the treatments of choice. Due to the often multifocal nature of the tumour, transplantation may be the optimal treatment. Metastatic lesions have been reported in ~30% of patients at presentation and occur most commonly in the lungs . Other less common sites include the abdominal lymph nodes, omentum, mesentery, and peritoneum.

CT image 예 >

74세 남환, 기저 간질환이 없었던 분이며, 증상도 없었던 분. 

검진 상 간초음파에서 다발성 결절 확인되어 전원.

--> Precontrast image에서 low density를 보이고, enhancement시 일부는 globular enhancemet를 보이며, 대부분 delayed enhancement를 보임.

끝.

2018. 10. 28 - SJH