PubMed · 9616067
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P Debourdeau, J Gligorov, C Couteau. 1998-06-04. Genetic abnormalities in synovial sarcoma.. https://doi.org/10.1056/nejm199806043382315
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Tumors of the salivary gland are very uncommon in children. Sialoblastoma is a rare, aggressive, blastomatous, and potentially malignant congenital tumor. Distant metastases are rare. We present a case of sialoblastoma with lung metastases that developed in a 4-year-old girl adjacent to a congenital nevus in the left cheek. The tumor was inoperable at diagnosis but the largest of the pulmonary metastases was removed surgically. The patient responded well to chemotherapy and underwent surgical excision of the primary tumor, followed by three more courses of chemotherapy.
Anaplastic large cell lymphoma (ALCL) accounts for approximately 15% of childhood NHL. Relapsed ALCL represents a formidable challenge because outcome is poor despite the use of high-dose chemotherapy regimens. We report two patients with relapsed T-type and 0-type ALCL who achieved long-term 3rd and 4th remissions with 4-weekly oral dexamethasone (DEX) and etoposide pulses for 2 years. This regimen also induced and maintained remission in a third patient with Nijmegen breakage syndrome (NBS) with secondary T-type ALCL. These patients demonstrate that low-intensity oral chemotherapy can induce long-term remissions and offer a curative perspective in refractory, relapsed and secondary ALCL.
Cystic partially differentiated nephroblastoma (CPDN) has low malignant potential. We report a 1-year-old with stage III CPDN of the right kidney that recurred following radical nephrectomy and chemotherapy. There was evidence of tumor spillage pre-operatively and intra-operatively. During chemotherapy the disease recurred in the omentum and the peritoneum. Pathology of the recurrent resected cysts revealed a more differentiated biphasic tumor without blastemal elements. It appears that spillage of CPDN in our patient led to dissemination of disease. Chemotherapy failed to prevent recurrence but only mature elements were present following this treatment. The intensity of therapy required to treat CPDN remains undefined.