clinical characteristics and prognosis of hepatoblastoma ...hepatoblastoma is a rare pediatric...
TRANSCRIPT
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= Abstract=
Clinical Characteristics and Prognosis of Hepatoblastoma in Children
Min Young Kim, M.D., Dae Yeon Kim, M.D., Hyo Seop Ahn, M.D.*,
Chong Jai Kim, M.D.**, In One Kim, M.D.***, Sung Eun Jung, M.D.,
Seong Cheol Lee, M.D., Kwi Won Park, M.D., Woo Ki Kim, M.D.
Department of Surgery, Pediatrics*, Pathology**, Radiology***
Seoul National University College of Medicine
Seoul, Korea
Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage. With the neoajuvant chemotherapy, improved resectability and survival have been reported. Twenty children with biopsy proven hepatoblastoma were treated during the period between January 1987 and June 1995. Median age at diagnosis was 13 months(2 months to 7 year and 10 months) , and 13 were male. Histologic profile was 13 epithelia1(5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial and 2 of undetermined type. Chemotherapy effectively reduced the tumor volume(p'= O. J08), and' was able to convert 7 out of 9 initially unresectable cases(78%) to resectable ones. Twelve radical and 2 palliative operations were done with or without adjuvant chemotherapy. The Median follow up period was 33 months and the median survival was 26 months. The group with curative resection had a 61.1 % 5 year survival rate, but none of palliative resection grpup survived more than 13 months(p= 0.000l). In univariate analysis for prognostic factors revealed, large tumor size at diagnosis and abscence of thrombocytopenia were associated with poor survival, but these differences were not statistically significant. Histological pure fetal type did not mean a better prognosis. Even with a recent neoadjuvant chemotherapy, the strategy should be focused on the radical resection as early as possible.
Index Words: Hepatoblastoma, Curative resection, Neoadjuvant chemotherapy, Prognosis,
Thrombocytopenia
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Table 1. Histology of Hepatoblastomas
Epithelial 13(65 %) undetermined 3 (15 %) fetal 5 (25 %) mixed feta l & embryonal 4(20 %) embryonal 1 ( 5 %)
Mixed mesenchymal & epithelial 5 (25 %)
Anaplastic O( 0%)
Undetermined 2(10 %)
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No. of Pts
10~---------------------
9 ! I'!l---i ____________ ~~L___ ~ -Ulilili~: -liiiil-il _ ________ _
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3 5 7 9 Age (Yr)
Fig. 1. Age and Sex.
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Table 2. Chief Complaints and Laboratory Findings at Admission
Chief co mpla nit
mass di s t ' nsion pain others (fever, cough , diarrhea)
8(40 %) 6(30 %) 3(15 %) 3(15 %)
Hematologic & biochemical features
increased aFP hypercholesterolemia anemia th rom bocy tosis jaundice
20000 %) 13(65 %) 11 (55 %) 6(30 %) 5(25 %)
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20Pts I~
/
7 Unresectable : ~ Death Refuse Tx (3)
{I Op (14)
,--:;- PreOp Chemo (10) )
I~ Death Chemo Cx [2/ Refuse Tx 1
rl Palliative (2) I~ r l Curative (12) I~ Death Death I Alive (9) I Recur (3~ Recur (1) Unknown 1)
Tx;treatment, Chemo; chemotherapy, Op; operation, Cx;complication
Fig. 2. Clinical course of 20 patients with Hepatoblastoma.
tl ~s:.'T'-~ -'g-0J~ A~l.21 ~ t.+u-j Al 3 i!1lojjJ'1 ~ A~
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V score (x104mm3)
250
200
150
100
50
o Before Chemo
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Median in tumor volume reduction rate (After I Before Chemo) : 78.7%
(p =0. 008)
After Chemo
Fig. 3. Effect of neoadjuvant chemotherapy on tumor volume in 8 hepatoblastoma patients.
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--- Overall Survival Rate (%) - - - - - - - _. Curative resection *
100 ........... Non-curative resection *
* p=O.0001
80 1_ - - - - - - - - - - - - - - - - - - - - _,
61.1% 60
40
20
o o 1 2 3
Fig. 4. Survival rate by type of operations.
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Table 3. Name of Operations
Tumorectomy
Anterior segmentectomy
Posterior segmentectomy
Anterior and medial segmentectomy
Left lobetomy
Right lobectomy
Trisegmentectomy
4
5
, ' _____________ _
39.5%
4 5 Years
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Table 4. F'actors Affecting Prognosis
F'actors 5yr SR P-value
Thrombocytosis
~600,000
<600,000
Initial V score
~ 700,000
<700,000
100 %(n=4)
38.9 %(n=8)
38.l %(n=7)
100 %(n=5)
0.08
0.07
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