A 1-year-old girl was diagnosed with stage IV
alveolar rhabdomyosarcoma in 2008. A large
tumor was located in the central diaphragm and had infiltrated the liver and pericardium with peritoneal dissemination.
Chemotherapy was immediately started with six courses of
vincristine,
actinomycin-D and
cyclophosphamide (VAC) firstly, and secondly followed by 2 courses of
ifosfamide,
carboplatin and
etoposide (
ICE), but a large
tumor of 15 cm in size remained. The
tumor was inoperable because of its location, and photon
radiotherapy could not be performed due to limited liver tolerance. The patient was referred to our hospital and received PBT at a dose of 54 GyE in 30 fractions in June 2009. The
tumor quickly responded and 95 % of volume reduction was achieved at the end of PBT. However, marginal recurrence in the caudal part of the irradiated field, where we reduced the
proton dose because of the presence of the intestine, was detected in August 2010. The recurrent
tumor size was less than 1 cm.
Chemotherapy with VAC followed by
topotecan and
carboplatin (TC) was again tried, but the
tumor size was stable. Repeated PBT was not possible because of limited intestinal tolerance; therefore, intraoperative
radiotherapy was conducted with 20 Gy of electron beams in April 2011. The
tumor was subsequently well controlled, but secondary
myelodysplastic syndrome developed and the patient died of
hemophagocytic syndrome after umbilical cord blood
transplantation in May 2012.
CONCLUSION: PBT was performed safely and effectively for a 1-year-old girl with
alveolar rhabdomyosarcoma with liver and cardiac invasion that was resistant to surgery and
chemotherapy. This case illustrates that PBT can be useful in cases that are difficult to treat with conventional
radiotherapy.