- Email: [email protected]
Progression of an Unusual Primary Liver Tumor
Accepted Manuscript Natural Course of Unusual Primary Liver Tumor Yang-Yuan Chen, M.D, Cheng-Che Chen, M.D.
PII: DOI: Reference:
S0016-5085(17)36092-4 10.1053/j.gastro.2017.08.059 YGAST 61405
To appear in: Gastroenterology Accepted Date: 29 August 2017 Please cite this article as: Chen Y-Y, Chen C-C, Natural Course of Unusual Primary Liver Tumor, Gastroenterology (2017), doi: 10.1053/j.gastro.2017.08.059. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Natural Course of Unusual Primary Liver Tumor
Division of Gastroenterology,
RI PT
Yang-Yuan Chen, M.D., Cheng-Che Chen, M.D.
M AN U
SC
Changhua Christian Hospital, Changhua, Taiwan
TE D
Correspondence and Reprints:
Yang-Yuan Chen, M.D.
Changhua Christian Hospital
AC C
EP
3 Lane 138 Tai-An 2nd Street Changhua, 500 TAIWAN
Fax: +886-4-7228289
E-mail: [email protected]
Financial Support: The authors received no grants for this research. Disclosures: The authors have no potential conflicts of interest to disclose. Writing Assistance: None.
ACCEPTED MANUSCRIPT
Yang-Yuan Chen, M.D.
RI PT
Writing and final approval of this manuscript. Department of Gastroenterology, Changhua Christian, Taiwan.
Cheng-Che Chen, M.D. Writing of this manuscript.
M AN U
SC
E-mail: [email protected]
Department of Gastroenterology, Changhua Christian, Taiwan.
AC C
EP
TE D
E-mail: [email protected]
ACCEPTED MANUSCRIPT A 72-year-old woman presented at the emergency room (ER) with diffuse abdominal tenderness. Her medical history revealed end-stage renal disease with
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hemodialysis for 10 years. She had previously received bilateral nephroureterectomy for left renal pelvis urothelial carcinoma and total thyroidectomy for papillary thyroidal cancer. She was regularly followed up after bilateral nephroureterectomy.
SC
Abdominal computed tomography (CT) revealed a low-density liver nodule over
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segment 7 (Figure 1). No interval size change was observed in the abdominal CT scan 3 months later. An abdominal CT scan 13 months later revealed greater enlargement of the tumor with mild enhancement after contrast injection (Figure 2). Physical examination was unremarkable. Findings of chronic hepatitis B or C were negative.
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Hepatic function tests revealed that aspartate aminotransferase, total bilirubin, and γ-glutamyl transferase were within normal limits. The carcinoembryonic antigen level
EP
was 4 ng/mL, and the alpha-fetoprotein level was 3 ng/mL. A liver tumor biopsy was
AC C
performed. She declined further resection of the liver tumor due to poor prognosis and body performance. She experienced sudden onset diffuse abdominal rebounding pain and visited the ER. An emergency CT scan revealed a large right tumor with a considerable amount of contrast medium and much ascites (Figure 3).
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What was the diagnosis?
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ACCEPTED MANUSCRIPT
Answer: Natural course of primary hepatic angiosarcoma
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with rupture.
ACCEPTED MANUSCRIPT The pathology demonstrated slitlike vascular channels lined by spindle-shaped endothelial cells with large and hyperchromatic nuclei (Figure 3). Primary hepatic
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angiosarcoma (PHA) was diagnosed. The patient declined operation because of poor prognosis and body performance. Ascites tapping was performed, and the fluid was bloody. The patient received blood transfusion and expired 2 days later.
SC
PHA is an aggressive malignant tumor that originates from the endothelium of liver
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blood vessels. It is a rare condition and accounts for 0.6%–2.0% of all primary liver tumors and less than 5% of all angiosarcomas.1
Most PHAs exhibit no obvious symptoms and signs, particularly when they are small. As the disease progresses, symptoms and signs including abdominal pain,
TE D
weakness, fatigue, weight loss, hepatomegaly, and ascites appear. The spontaneous rupture of PHA was reported in 15%–27% of patients in a previous study.2 As in our
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case, no obvious symptoms were noted in the early stage, and spontaneous rupture
AC C
was observed in the later stage.
A CT scan of PHA reveals multiple nodules or a dominant mass and a diffusely
infiltrating lesion. The tumor is composed of low-density lesions with small heterogeneous hypervascular foci.3 In our patient, a CT scan revealed a hepatic tumor that had a low density when it was small and multiple heterogeneous hypervascular foci when it grew large.
ACCEPTED MANUSCRIPT PHAs are very aggressive tumors, and most cases are diagnosed at an advanced stage. The median survival was reported to be 6 months without treatment. Complete
RI PT
resection with clear margins is the choice of treatment; however, the prognosis is poor even after complete resection.2 In our case, operation after diagnosis was declined because of the patient’s poor prognosis and body performance. She lived for 18
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rupture was well presented in our case.
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months after the diagnosis. The entire natural course of PHA from initial diagnosis to
ACCEPTED MANUSCRIPT
References: 1. Zheng YW, Zhang XW, Zhang JL, Hui ZZ, Du WJ, Li RM, Ren XB. Primary
RI PT
hepatic angiosarcoma and potential treatment options. J Gastroenterol Hepatol. 2014; 29: 906-11.
2. Cawich SO, Ramjit C. Herald bleeding from a ruptured primary hepatic
SC
angiosarcoma: A case report. Mol Clin Oncol. 2015; 3: 1063-1066.
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3. Huang IH, Wu YY, Huang TC, Chang WK, Chen JH. Statistics and outlook of primary hepatic angiosarcoma based on clinical stage.Oncol Lett. 2016; 11:
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3218-3222.
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ACCEPTED MANUSCRIPT
PII: DOI: Reference:
S0016-5085(17)36092-4 10.1053/j.gastro.2017.08.059 YGAST 61405
To appear in: Gastroenterology Accepted Date: 29 August 2017 Please cite this article as: Chen Y-Y, Chen C-C, Natural Course of Unusual Primary Liver Tumor, Gastroenterology (2017), doi: 10.1053/j.gastro.2017.08.059. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Natural Course of Unusual Primary Liver Tumor
Division of Gastroenterology,
RI PT
Yang-Yuan Chen, M.D., Cheng-Che Chen, M.D.
M AN U
SC
Changhua Christian Hospital, Changhua, Taiwan
TE D
Correspondence and Reprints:
Yang-Yuan Chen, M.D.
Changhua Christian Hospital
AC C
EP
3 Lane 138 Tai-An 2nd Street Changhua, 500 TAIWAN
Fax: +886-4-7228289
E-mail: [email protected]
Financial Support: The authors received no grants for this research. Disclosures: The authors have no potential conflicts of interest to disclose. Writing Assistance: None.
ACCEPTED MANUSCRIPT
Yang-Yuan Chen, M.D.
RI PT
Writing and final approval of this manuscript. Department of Gastroenterology, Changhua Christian, Taiwan.
Cheng-Che Chen, M.D. Writing of this manuscript.
M AN U
SC
E-mail: [email protected]
Department of Gastroenterology, Changhua Christian, Taiwan.
AC C
EP
TE D
E-mail: [email protected]
ACCEPTED MANUSCRIPT A 72-year-old woman presented at the emergency room (ER) with diffuse abdominal tenderness. Her medical history revealed end-stage renal disease with
RI PT
hemodialysis for 10 years. She had previously received bilateral nephroureterectomy for left renal pelvis urothelial carcinoma and total thyroidectomy for papillary thyroidal cancer. She was regularly followed up after bilateral nephroureterectomy.
SC
Abdominal computed tomography (CT) revealed a low-density liver nodule over
M AN U
segment 7 (Figure 1). No interval size change was observed in the abdominal CT scan 3 months later. An abdominal CT scan 13 months later revealed greater enlargement of the tumor with mild enhancement after contrast injection (Figure 2). Physical examination was unremarkable. Findings of chronic hepatitis B or C were negative.
TE D
Hepatic function tests revealed that aspartate aminotransferase, total bilirubin, and γ-glutamyl transferase were within normal limits. The carcinoembryonic antigen level
EP
was 4 ng/mL, and the alpha-fetoprotein level was 3 ng/mL. A liver tumor biopsy was
AC C
performed. She declined further resection of the liver tumor due to poor prognosis and body performance. She experienced sudden onset diffuse abdominal rebounding pain and visited the ER. An emergency CT scan revealed a large right tumor with a considerable amount of contrast medium and much ascites (Figure 3).
TE D
What was the diagnosis?
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
Answer: Natural course of primary hepatic angiosarcoma
AC C
EP
with rupture.
ACCEPTED MANUSCRIPT The pathology demonstrated slitlike vascular channels lined by spindle-shaped endothelial cells with large and hyperchromatic nuclei (Figure 3). Primary hepatic
RI PT
angiosarcoma (PHA) was diagnosed. The patient declined operation because of poor prognosis and body performance. Ascites tapping was performed, and the fluid was bloody. The patient received blood transfusion and expired 2 days later.
SC
PHA is an aggressive malignant tumor that originates from the endothelium of liver
M AN U
blood vessels. It is a rare condition and accounts for 0.6%–2.0% of all primary liver tumors and less than 5% of all angiosarcomas.1
Most PHAs exhibit no obvious symptoms and signs, particularly when they are small. As the disease progresses, symptoms and signs including abdominal pain,
TE D
weakness, fatigue, weight loss, hepatomegaly, and ascites appear. The spontaneous rupture of PHA was reported in 15%–27% of patients in a previous study.2 As in our
EP
case, no obvious symptoms were noted in the early stage, and spontaneous rupture
AC C
was observed in the later stage.
A CT scan of PHA reveals multiple nodules or a dominant mass and a diffusely
infiltrating lesion. The tumor is composed of low-density lesions with small heterogeneous hypervascular foci.3 In our patient, a CT scan revealed a hepatic tumor that had a low density when it was small and multiple heterogeneous hypervascular foci when it grew large.
ACCEPTED MANUSCRIPT PHAs are very aggressive tumors, and most cases are diagnosed at an advanced stage. The median survival was reported to be 6 months without treatment. Complete
RI PT
resection with clear margins is the choice of treatment; however, the prognosis is poor even after complete resection.2 In our case, operation after diagnosis was declined because of the patient’s poor prognosis and body performance. She lived for 18
AC C
EP
TE D
M AN U
rupture was well presented in our case.
SC
months after the diagnosis. The entire natural course of PHA from initial diagnosis to
ACCEPTED MANUSCRIPT
References: 1. Zheng YW, Zhang XW, Zhang JL, Hui ZZ, Du WJ, Li RM, Ren XB. Primary
RI PT
hepatic angiosarcoma and potential treatment options. J Gastroenterol Hepatol. 2014; 29: 906-11.
2. Cawich SO, Ramjit C. Herald bleeding from a ruptured primary hepatic
SC
angiosarcoma: A case report. Mol Clin Oncol. 2015; 3: 1063-1066.
M AN U
3. Huang IH, Wu YY, Huang TC, Chang WK, Chen JH. Statistics and outlook of primary hepatic angiosarcoma based on clinical stage.Oncol Lett. 2016; 11:
AC C
EP
TE D
3218-3222.
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EP
TE D
M AN U
SC
RI PT
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