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Distinct patterns of p27/KIP 1 gene expression in hepatoblastoma and
prognostic implications with correlation before and after chemotherapy.
Brotto M, Finegold MJ.
Department of Pathology, Texas Children's Hospital, Houston, TX. |
Hum Pathol 2002 Feb;33(2):198-205 Abstract quote
Over the past 3 years, numerous studies have examined the diagnostic
and prognostic significance of p27/Kip1 expression in various tumors.
Almost all studies report decreased p27 expression in more aggressive
tumors. Information about morphologic changes due to chemotherapy in
hepatoblastoma (Hbs) is limited, and so is information about distinct
patterns of p27 gene expression.
Twenty-nine hepatoblastomas were evaluated for possible prognostic
correlation between p27 expression in different histotypes of hepatoblastoma,
changes during chemotherapy, and outcome. These observations should
prompt prospective randomized studies designed to investigate the predictive
role of p27 expression in different Hbs histotypes. Patients were treated
according to the Children's Cancer Group and Pediatric Oncology Group
protocols, which included initial surgery before chemotherapy wherever
possible.
Follow-up ranged from 1 to 133 months. The results show that primary
well-differentiated fetal tumors without mitotic activity are strongly
p27 positive. The embryonal pattern displays a variable p27 protein
expression pattern, with focal positivity between completely negative
zones; p27 is positive where the mitotic activity is low or absent and
negative where the mitogenic activity is high. The vast majority of
small undifferentiated cell components are p27 negative. p27 protein
expression is downregulated after chemotherapy in the remaining fetal
well-differentiated component of Hbs.
Although this may imply selection of a more aggressive clone, all patients
with this histology were cured in this series. Aggressiveness and ultimate
prognosis for incompletely resected tumors after chemotherapy remain
indeterminate. |
| METASTASIS |
Lungs most frequent, present in 10-20% of initial diagnosis
Bone, brain, eye, and ovaries
May extend into hepatic vessels and inferior vena cava
Multiple resections and chemotherapy/radiation therapy may be successful
in treating pulmonary and brain metastases over a prolonged period |
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Analysis of treatment outcome for children with recurrent or metastatic
hepatoblastoma.
Matsunaga T, Sasaki F, Ohira M, Hashizume K, Hayashi A, Hayashi
Y, Mugishima H, Ohnuma N.
Department of Pediatric Surgery, Graduate School of Medicine,
Chiba University, Chiba, Japan.
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Pediatr Surg Int 2003 May 24; Abstract quote For better total survival
rate of children with hepatoblastoma, the therapeutic strategy for recurrent
or metastatic hepatoblastoma should be improved. From 1991 to 1999,
134 cases of hepatoblastoma were treated by surgery and combination
chemotherapy of cisplatin (CDDP) and THP-Adriamycin (THP-ADR) based
on the JPLT-1 protocol. In 114 non-metastatic cases, 90 primary liver
tumors were resected completely by partial hepatectomy, but 12 recurrences
were observed in the liver (4 cases) and the lungs (8 cases).
Distant metastases on the diagnosis were observed in 20 cases. The treatment
outcome of these 12 recurrent and 20 metastatic tumors was analyzed.
In four recurrent liver tumors, surgical resection was performed in
all four cases, and all the patients were alive and well. In eight recurrent
lung tumors, surgical resection was performed completely in six cases
with unilateral lung disease, and five of the six patients were alive
and well.
In stage IV tumors, the survival rate of the patients having primary
tumors within two hepatic sections was significantly higher than that
of the patients having primary tumors over three hepatic sections. Active
surgical intervention to lung metastases and a more intensive chemotherapy
to facilitate complete resection of primary hepatic tumor could improve
survival rate of children with refractory hepatoblastoma.
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Surgical resection and chemotherapy improve survival rate for patients
with hepatoblastoma.
Carceller A, Blanchard H, Champagne J, St-Vil D, Bensoussan AL.
Division of Pediatrics, General Surgery, and Hemato-Oncology, Ste-Justine
Hospital, Montreal, Quebec, Canada. |
J Pediatr Surg 2001 May;36(5):755-9 Abstract quote
BACKGROUND: The authors reviewed retrospectively their experience in
30 children with hepatoblastoma (HB). Despite an increased trend in
the incidence of HB during the last 2 decades, an encouraging cure rate
has been achieved with complete resection of the tumor and chemotherapy
before or after surgery with cisplatin plus doxorubicin (Adriamycin)
or cisplatin plus vincristine plus 5-Fluorouracil.
RESULTS: There were 10 female and 20 male patients. For the period
from 1963 to 1980 there were 8 patients, and for the period from 1981
to 1998 there were 22 patients. Their mean age at surgery was 16 months
(range, 3.5 months to 5.5 years). Tumors were localized to the right
lobe in 10 (42%), to the left lobe in 7 (29%), and in both lobes in
7 (29%) of the resected patients. Tumors were greater than 10 cm in
size in 16 (67%) of these patients. Twenty-four patients (80%), underwent
liver resection before or after chemotherapy. One patient (3%) with
an unresectable tumor received chemotherapy and a liver transplant.
In 5 patients (17%) the hepatic involvement was too extensive for resection.
The types of resection performed were right lobectomy in 7, left lobectomy
in 6, right trisegmentectomy in 8, left trisegmentectomy in 2, and middle
hepatectomy in 1. The overall survival rate for 35 years of the study
was 60% (18 of 30). With the association of surgery and chemotherapy
(1981 through 1998) survival rate is 82% (14 of 17). Overall median
follow-up in our study is 8 years (range, 2.5 to 24 years).
CONCLUSIONS: There has been a dramatic improvement in the results of
treatment of hepatoblastoma. Formerly, only 25% to 30% of patients were
cured, whereas today, with combination of chemotherapy and surgery,
75% to 80% may be cured. |
