Abstract
Background/ Purpose: Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience. Methods: A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted. Results: Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years. Conclusion: This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.
| Original language | English |
|---|---|
| Pages (from-to) | 2124-2128 |
| Number of pages | 5 |
| Journal | Journal of Pediatric Surgery |
| Volume | 45 |
| Issue number | 11 |
| DOIs | |
| Publication status | Published - Nov 2010 |
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Keywords
- Pediatric liver unit
- Pediatric tumors
- Primary hepatic sarcomas
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
Cite this
Primary hepatic sarcomas in children - A single-center experience over 19 years. / Upadhyaya, Manasvi; McKiernan, Patrick; Hobin, David; Kelly, Deirdre A.; Brown, Rachel; Lloyd, Carla; Buckels, John; Millar, Alastair J W; Deville Degoyet, Jean; Mirza, Darius F.; Sharif, Khalid.
In: Journal of Pediatric Surgery, Vol. 45, No. 11, 11.2010, p. 2124-2128.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Primary hepatic sarcomas in children - A single-center experience over 19 years
AU - Upadhyaya, Manasvi
AU - McKiernan, Patrick
AU - Hobin, David
AU - Kelly, Deirdre A.
AU - Brown, Rachel
AU - Lloyd, Carla
AU - Buckels, John
AU - Millar, Alastair J W
AU - Deville Degoyet, Jean
AU - Mirza, Darius F.
AU - Sharif, Khalid
PY - 2010/11
Y1 - 2010/11
N2 - Background/ Purpose: Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience. Methods: A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted. Results: Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years. Conclusion: This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.
AB - Background/ Purpose: Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience. Methods: A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted. Results: Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years. Conclusion: This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.
KW - Pediatric liver unit
KW - Pediatric tumors
KW - Primary hepatic sarcomas
UR - http://www.scopus.com/inward/record.url?scp=78049471689&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=78049471689&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2010.07.016
DO - 10.1016/j.jpedsurg.2010.07.016
M3 - Article
C2 - 21034932
AN - SCOPUS:78049471689
VL - 45
SP - 2124
EP - 2128
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 11
ER -