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Metastatic malignant rhabdoid tumor of the liver treated with tandem high-dose therapy and autologous peripheral blood stem cell rescue

✍ Scribed by Katzenstein, Howard M. ;Kletzel, Morris ;Reynolds, Marleta ;Superina, Riccardo ;Gonzalez-Crussi, F.


Publisher
John Wiley and Sons
Year
2003
Tongue
English
Weight
165 KB
Volume
40
Category
Article
ISSN
0098-1532

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✦ Synopsis


cyclophosphamide, carboplatin, and etoposide in another and an unknown combination in a third [4-6]. Only partial responses were seen. Another, 5-month-old boy received etoposide alone after a second microscopically incomplete resection. He was alive without disease 1 year later [7]. Dehner et al. report on a 2-months-old boy with a maxillary MNTI. The tumor was excised, recurred several times, and finally metastasized. At this stage neither radiation nor chemotherapy could stop the tumor growth and the boy died of disease [8].

Only one patient, a 2-month-old boy with a huge MNTI of the maxilla, was primarily treated with chemotherapy without resection like ours [9]. The chemotherapy regimen contained vincristine, cyclophosphosmide, etoposide, doxorubicin, and dactinomycin and it was comparable to the treatment our patient received. Some tumor shrinkage and, notably, calcification of the tumor resulted. No surgery was performed. The boy is now 6 years old without signs of tumor recurrence [Mittler U, personal communication]. Since our patient's tumor first responded after intensification of chemotherapy with etoposide, it may be speculated that etoposide induces either a terminal differentiation program or apoptosis in this embryonal neuroectodermal tumor.

In conclusion, a typical facial MNTI in an infant responded to combination chemotherapy and mutilating surgery was thus avoided.