Review of hepatic tumors

Nattinee Srisantiroj MD.*, Tharinee Lumluk MD.*, Eva Leinart MD., PhD.**

*Fetal-maternal medicine unit, Department of Obstetric and Gynecology, Rajavithi hospital, Bangkok,Thailand.
** Inner Vision Women's Ultrasound, Nashville, Tennessee, USA.
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Hepatic_hamartoma_6


Images 7,8: Doppler imaging of the tumor shows it's profound vascularization.

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Hepatoblastoma

Hepatoblastoma is the most common hepatic malignancy among neonates and in childhood. The most common initial finding is an elevation of α-fetoprotein. Unfortunately, this includes only half of the prenatally diagnosed cases. Other initial finding is a hepatic or abdominal mass detected on the ultrasound [1,8]. There are four main histological types: 1) fetal and 2) embryonal 3) fetal with embryonal and 4) fetal with embryonal and mesenchymal component [1,3].

Prevalence: very rare

Ultrasound findings

Hepatoblastomas are well-defined, lobulated, echogenic lesions, which may have internal structure like a spoked-wheel appearance. Most common site is the right lobe of the liver.

Differential diagnosis

Includes metastatic neuroblastoma and benign causes such as hemangioma, mesenchymal hamartoma, adenoma, and focal nodular hyperplasia [1].

Treatment and prognosis

Because of the rarity of this disease, the treatment protocols vary depending on the clinical findings. However, the treatment modalities include: primary chemotherapy and surgical resection, or chemotherapy alone [9]. If untreated it is usually fatal within 2 years.

Images 9,10: Transverse view of the fetal abdomen showing a hepatoblastoma. Note the well-defined, encapsulated lesion.

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Images 11,12: Doppler imaging showing the vascularization of the tumor mass.

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Classification of fetal and neonatal hepatic tumors and tumorlike conditions [1,13]:

  • Infantile hemangioma: Focal; Multifocal (Hemangioendothelioma type 1, Hemangioendothelioma type 2)
  • Mesenchymal hamartoma
  • Solitary unilocular cyst
  • Adenoma
  • Focal nodular hyperlasia
  • Hepatoblastoma: Epithelial (Fetal, Embryonal); Mixed: epithelial and mesenchymal; Anaplastic
  • Hepatocellular carcinoma
  • Germ cell tumors: Teratoma; Yolk sac tumor; Choriocarcinoma
  • Rhabdoid tumor
  • Hepatic sarcoma: Undifferentiated (embryonal) sarcoma; Rhabdomyosarcoma
  • Metastatic neoplasms: Neuroblastoma; Leukemia; Renal tumors; Yolk sac tumor; Rhabdoid tumor; Choriocarcinoma

References:

1.Isaacs H, Jr. Fetal and neonatal hepatic tumors. J Pediatr Surg. 2007 Nov;42(11):1797-803.

2.Mahony R, McParland P. Approaches to the management of antenatally diagnosed congenital tumours. Pediatr Radiol. 2009 Nov;39(11):1173-8.

3.Callen PW. Ultrasonography in obstetrics and gynecology. 5th ed. Philadelphia: Saunders Elsevier; 2008.

4.Sari N, Yalcin B, Akyuz C, Haliloglu M, Buyukpamukcu M. Infantile hepatic hemangioendothelioma with elevated serum alpha-fetoprotein. Pediatr Hematol Oncol. 2006 Dec;23(8):639-47.

5.Siddiqui MA, McKenna BJ. Hepatic mesenchymal hamartoma: a short review. Arch Pathol Lab Med. 2006 Oct;130(10):1567-9.

6.Stringer MD, Alizai NK. Mesenchymal hamartoma of the liver: a systematic review. J Pediatr Surg. 2005 Nov;40(11):1681-90.

7.Cornette J, Festen S, van den Hoonaard TL, Steegers EA. Mesenchymal hamartoma of the liver: a benign tumor with deceptive prognosis in the perinatal period. Case report and review of the literature. Fetal Diagn Ther. 2009;25(2):196-202.

8.Shih JC, Tsao PN, Huang SF, Yen BL, Lin JH, Lee CN, et al. Antenatal diagnosis of congenital hepatoblastoma in utero. Ultrasound Obstet Gynecol. 2000 Jul;16(1):94-7.

9.Davies JQ, de la Hall PM, Kaschula RO, Sinclair-Smith CC, Hartley P, Rode H, et al. Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases. J Pediatr Surg. 2004 Sep;39(9):1321-7.

10. Isaacs Jr H. Liver tumors. In: Isaacs Jr H, editor. Tumors of the fetus and infant: an atlas. New York (NY): Springer-Verlag; 2002. p. 303-36.

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