164 RETINOBLASTOMA – FAMILIAL INCIDENCE, CYTOGENETIC STUDY AND SPINAL FLUID AND BONE MARROW EXAMINATIONS
| Autor: | Helena Zygulska-Machowa, Jacek J Pietrzyk, Grayna Miklaszewska, Ewa Kostyk, Boguslawa Krzykwa |
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| Rok vydání: | 1990 |
| Předmět: | |
| Zdroj: | Pediatric Research. 28:304-304 |
| ISSN: | 1530-0447 0031-3998 |
| DOI: | 10.1203/00006450-199009000-00188 |
| Popis: | From January 1989 through January 1990 a group of 51 children (25 boys and 26 girls) affected with retinoblastoma (Rb) were ascertained by means of a nation-wide referring system of this disease in Polish population. All children were diagnosed below 3 years of age. Bilateral tumors were observed in 28 cases, whereas 23 children were affected unilaterally. In 4 families additional four cases of Rb were found among the probands' parents (3) and sib (1). All affected children showed bilateral tumors. In addition, second degree relatives of 9 probands revealed other malignancies of mesenchymal origin known to be associated with Rb-1 chromosomal locus. Cytogenetic examinations performed in all familial cases revealed normal karyotypes among probands, their parents and sibs. In 20 probands spinal fluid and bone marrow aspirates were examined for early metastases as an attempt to define the indications for chemiotherapy. In only one case an increased percentage of blast cells indicating bone marrow metastases was observed. In two cases, the examination of spinal fluid revealed the macrophages with significant necrobiotic changes, but without any signs of neoplastic cells. Conclusions: 1. In addition to significant familial clustering, Rb reveals marked association with other mesenchymal tumors. 2. Familial as well as sporadic cases of Rb were not accompanied by interstitial deletion of chromosome 13. 3. Spinal fluid and bone marrow examinations might be of some value for the introduction of adjunct therapy for surgical treatment of Rb. |
| Databáze: | OpenAIRE |
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