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Archives francaises de pediatrie 1993-Dec

[Rapid efficacy of preoperative chemotherapy in nephroblastoma involving cardiac cavities].

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H Carla
C Schoepfer
J R Lusson
C de Riberolles
G Vanneuville
R Goddon
J L Kemeny
P Dechelotte
M Meyer
G Malpuech

Keywords

Abstract

BACKGROUND

Tumors involving the inferior vena cava and cardiac cavities are rare in nephroblastoma. When they do occur, the standard treatment is primary surgery; but this is often technically difficult.

METHODS

A 3 year-7-month-old girl was admitted because of an abdominal mass and signs of heart failure. Ultrasonography showed that this mass involved the right renal vein and the inferior vena cava including the right atrium. A CT scan showed no metastases. Echocardiography showed that the mass occupied two thirds of the right atrium and had blocked the tricuspid valve. The patient was given heparin (2 mg/kg/day), vincristine (1.5 mg/m2 on day 1) and actinomycin D (15 micrograms/kg on day 1 to 3). The heart failure disappeared and the abdominal mass decreased in volume. This improvement was confirmed by successive ultrasonographies. Chemotherapy was then completed and the patient was operated on six weeks later: right nephrectomy and removal of the cavoatrial tumoral thrombus. Pathological examination confirmed the nephroblastoma and the patient was given radiotherapy and chemotherapy for 28 weeks. She was also given aspirin for 17 weeks. Pulmonary metastases were detected 1 year after onset and were treated by a combination of surgery and chemotherapy. The patient is well 20 months after the onset of the disease.

CONCLUSIONS

Preoperative chemotherapy seems to be effective in nephroblastoma extending to cardiac cavities; it allows subsequent surgery and facilitates postoperative radiotherapy.

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