Martins-Filho Sebastiao N, Putra Juan
Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
Department of Laboratory Medicine & Pathobiology, University of Toronto, Toronto, Ontario, Canada.
Hepat Oncol. 2020 Apr 7;7(2):HEP19. doi: 10.2217/hep-2020-0002.
This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.
肝间叶性错构瘤（HMH）和肝未分化胚胎性肉瘤（UESL）。HMH是一种主要见于生命最初2年的良性病变，而UESL是恶性的，通常在6至10岁的患者中发现。UESL可能在HMH的基础上发生，并且这种关联已得到相似染色体畸变（19q13.4）的支持。这两种病变的诊断主要基于组织学评估，因为其临床和放射学特征并不总是典型的。本文讨论了这两种病变的临床病理特征、发病机制、鉴别诊断及治疗。
