Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy

Ryota Souzaki, Tatsuro Tajiri, Yoshiaki Kinoshita, Sakura Tanaka, Yuhki Koga, Aiko Suminoe, Toshiro Hara, Kenichi Kohashi, Yoshinao Oda, Tomoaki Taguchi

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6 Citations (Scopus)

Abstract

Background: Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). Case report: A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. Conclusion: This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.

Original languageEnglish
Pages (from-to)1045-1048
Number of pages4
JournalPediatric surgery international
Volume26
Issue number10
DOIs
Publication statusPublished - Oct 1 2010

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Pancreaticoduodenectomy
Pylorus
Radiation
irinotecan
Drug Therapy
alpha-Fetoproteins
Stem Cell Transplantation
Neoplasms
Induction Chemotherapy
Vincristine
Etoposide
Therapeutics
Reference Values
Serum
Mesenteric Veins
Melphalan
Superior Mesenteric Artery
Carboplatin
Inferior Vena Cava
Pancreatoblastoma

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. / Souzaki, Ryota; Tajiri, Tatsuro; Kinoshita, Yoshiaki; Tanaka, Sakura; Koga, Yuhki; Suminoe, Aiko; Hara, Toshiro; Kohashi, Kenichi; Oda, Yoshinao; Taguchi, Tomoaki.

In: Pediatric surgery international, Vol. 26, No. 10, 01.10.2010, p. 1045-1048.

Research output: Contribution to journalArticle

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title = "Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy",
abstract = "Background: Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50{\%} and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). Case report: A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. Conclusion: This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.",
author = "Ryota Souzaki and Tatsuro Tajiri and Yoshiaki Kinoshita and Sakura Tanaka and Yuhki Koga and Aiko Suminoe and Toshiro Hara and Kenichi Kohashi and Yoshinao Oda and Tomoaki Taguchi",
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T1 - Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy

AU - Souzaki, Ryota

AU - Tajiri, Tatsuro

AU - Kinoshita, Yoshiaki

AU - Tanaka, Sakura

AU - Koga, Yuhki

AU - Suminoe, Aiko

AU - Hara, Toshiro

AU - Kohashi, Kenichi

AU - Oda, Yoshinao

AU - Taguchi, Tomoaki

PY - 2010/10/1

Y1 - 2010/10/1

N2 - Background: Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). Case report: A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. Conclusion: This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.

AB - Background: Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). Case report: A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. Conclusion: This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.

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