MyPACS.net: Radiology Teaching Files > Case 767450

previously visited METASTATIC HEPATOBLASTOMA
Contributed by: Dr Phillip Silberberg, Children's Hospital Omaha, Radiologist, Omaha Childrens, Creighton University and UNMC, Nebraska, USA.
Patient: 12 year old female
History: 12 year old female patient with metastatic hepatoblastoma. Recent admission for radical resection of her right ilium on 06 October, 2004.
Images:[small]larger

Fig. 1: Initial Diagnosis --involves anterior segment of right lobe and entire left lobe and the caudate lobe.

Fig. 2: T1 weighted MRI.Hypointense on T1 with hyperintense foci secondary to hemorrhage.

Fig. 3: T2 weighted MRI. Hyperintense with hypointense bands on T2 images.

Fig. 4: Coronal T1 weighted MRI with gadolinium shows enhancement of the tumour.

Fig. 5: MR angiogram shows hypervascular lesion

Fig. 6: 08/23/04. CT post op Left partial hepatectomy

Fig. 7: 08/23/04. Right iliac destructive lesion

Fig. 8: 08/30/04. MR pre and post Gadolinium right iliac destructive expansile lesion enhances

Fig. 9: 08/30/04. MR pre and post Gadolinium of right iliac lesion shows invasion of the soft tissues

Fig. 10: Coronal T1. Hypointense metastasis right illium.

Fig. 11: Coronal T2. Hyperintense metastasis right illium.

Fig. 12: 08 Dec 2005 T2 Sag 512 Slice 7

Fig. 13: 08 Dec 2005 T1 Sag Post Slice 9

Fig. 14: 08 Dec 2005 STIR HI TE Slice 14

Fig. 15: 08 Dec 2005 STIR HI TE Slice 16

Fig. 16: 13 Jan 2006 Lateral C-spine

Fig. 17: 26 Jan 2006 AP Humerus

Fig. 18: Needle biopsy of liver with hepatoblastoma-4/02

Fig. 19: High power tumor -4/02

Fig. 20: Rib resection with metastatic hepatoblastoma-8/03

Fig. 21: Metastatic tumor and normal bone marrow-8/03

Fig. 22: Tumor and megakaryocyte-8/03

Fig. 23: Tumor through periosteum, negative soft tissue margin-8/03

Fig. 24: Tumor in bone - right ilium - 10/04

Fig. 25: Metastatic tumor-10/04

Fig. 26: High power tumor -10/04

Fig. 27: Tumor in separate soft tissue-10/04

Fig. 28: 1/2006 Cervical tissue with metastatic hepatoblastoma

Fig. 29: 1/2006 with hepatoblastoma
Findings:

8/23/04 MRI Whole Body Stir 1. Redemonstration of high signal intensity abnormality within the posterior superior medial right ilium which is more accurately measured on CT scan accomplished the same day as this examination.

8/2/04 CT 1. Interval incrase in size in lytic bone lesion within the right ilium which now measures 3.0 x 1.7 cm.
2. No significant change in size in 5 mm left ilium lytic lesion.
3. Postoperative changes within the posterior thorax including posterior 7th, 8th and 9th rib resections with adjacent scarring within the left lung base.
4. Status post left hepatic lobe resection and cholecystectomy.

8/30/04 MRI 1. Lesion in the right iliac wing, consistent with her history of hepatoblastoma. It measures larger than on the previous CT. It is unclear whether it has grown since the prior study of only one week ago or if it is just better seen and there is more accurate measurement on the MRI.
2. THe other small 5 mm lesion within the left iliac wing likely represents a fatty lesion.

Diagnosis: Metastatic hepatoblastoma
Discussion:

-Most common primary hepatic tumor of childhood, 3rd most common abdominal tumor
-Increased incidence with Beckwith-Weidemann syndrome, hemihypertrophy
-66% less than 2 years old, peak age 16-24 months
-60% right lobe
-Paucity of early symptoms leads to large size at time of discovery
-May see precocious puberty and elevated alpha-fetoprotein
-Metastasizes to the lung. rarely metastasizes to bone.
-Radiographs show calcifications in up to 50%
-U/S: Large heterogeneous echogenic mass. Heterogeneity reflects areas of hemorrhage and necrosis. Hypervascular.
-CT: Lower attenuation than liver parenchyma on unenhanced images and enhances less than normal liver parenchyma. Rim enhancement.
-MRI: Hypointense on T1 with hyperintense foci secondary to hemorrhage. Hyperintense with hypointense bands on T2 images
-Differential Diagnosis: hemangioendothelioma, metastatic neuroblastoma, mesenchymal hamartoma, hepatocellular carcinoma ( ref1.)

Metastases at diagnoses occur in 10%-20% of patients with the lung being the predominant site of metastases both at presentation and relapse. Other sites of distant metastases, including brain and bone, are rare and usually occur in the setting of relapsed disease . A higher incidence of nonpulmonary metastases has also been reported in congenital hepatoblastoma .Although pulmonary metastases are usually accompanied by an increase in AFP, recurrence of pulmonary metastases has been reported to occur without such an increase


References: 1.Kirks, Donald. Practical Pediatric Imaging. Lipincott-Raven, 1998.
Blickman, Hans. Pedicatric Radiology: The Requisites. Mosby, 1998.
2.Dahnert, Wolfgang. Radiology Review Manual: 4th Edition. Williams and Wilkins, 1999.
3.Herzog CE, Andrassy RJ, Eftekhari F. Related Articles, Links
Childhood cancers: hepatoblastoma.
Oncologist. 2000;5(6):445-53. Review.
The Oncologist

Contributed by Bruce Shroeder, M.D., Department of Radiology, Chidren's Hospital, Omaha, NE
Phillip Silberberg,M.D.,Department of Radiology, Chidren's Hospital, Omaha, NE
Chad Eicher, M.D., Radiology Resident, Creighton University Medical Center, Omaha, NE.
Deborah Perry and Chris Reyes M.D., Department of Pathology, Children's Hospital, Omaha, NE
Benjamin Silberberg

Cody Gronsten, Creighton University; Undergraduate B.S.

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Additional Details:

Case Number: 767450Last Updated: 04-08-2007
Anatomy: Gastrointestinal (GI)   Pathology: Neoplasm
Modality: CT, MR, PathologyExam Date: 01-01-1985Access Level: Readable by all users
Keywords: metastatic hepatoblastoma

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