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The clinical course in pediatric solid tumor patients with focal nodular hyperplasia of the liver
Authors:Kiminobu Sugito  Shota Uekusa  Hiroyuki Kawashima  Takeshi Furuya  Kensuke Ohashi  Mikiya Inoue  Taro Ikeda  Tsugumichi Koshinaga  Ryouichi Tomita  Hideo Mugishima  Toshiya Maebayashi
Affiliation:Department of Pediatric Surgery, Nihon University School of Medicine, 30-1, Ohyaguchi-Kamimachi, Itabashi-ku, Tokyo, 173-8610, Japan. sugitou.kiminobu@nihon-u.ac.jp
Abstract:

Background

Focal nodular hyperplasia (FNH) of the liver is a rare benign lesion that may be related to the vascular and hepatic damage induced by completion of tumor therapy and a reaction to localized vascular abnormality. The aim of this study was to analyze the clinical course in pediatric solid tumor patients with FNH.

Methods

We analyzed thirty-two patients with pediatric solid tumors who received multiagent chemotherapy (15 advanced neuroblastomas, 7 hepatoblastomas, 5 rhabdomyosarcomas, 2 nephroblastomas, 1 rhabdoid tumor of the kidney, 1 clear cell sarcoma of the kidney and 1 pancreatoblastoma). All of them had been previously treated at our hospital, and have been alive for over 3 years without recurrence.

Results

FNH lesions were discovered in three (9.4%) of 32 patients, and were neuroblastoma (NB) stage 4. All 3 patients received induction chemotherapy and high-dose alkylating agents, and developed grade 3 (National Cancer Institute Common Toxicity Criteria; NCI-CTC) liver dysfunction during completion of tumor therapy without veno-occlusive disease. Two of the 3 patients received the same induction chemotherapy and high doses of alkylating agents with total body irradiation for cytoreductive agents prior to peripheral blood cell transplantation. FNH lesions in both female patients who received estrogen replacement therapy after completion of tumor therapy have expanded and are increasing.

Conclusion

FNH appears to be a late complication of iatrogenic disease in NB stage 4 patients. The therapeutic agents for NB stage 4 and estrogen replacement therapy should be considered as risk factors for the development of FNH.
Keywords:
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