Malignant fibrous histiocytoma of maxilla following radiotherapy for bilateral retinoblastoma

Abstract

A 20-year-old man developed a malignant fibrous histiocytoma in the right maxilla 19 years after irradiation for bilateral retinoblastoma.The incidence of second tumours in patients who survived bilateral retinoblastoma treated with radiation was 8.5 per cent. Malignant fibrous histiocytomas which arise in a site of prior radiation are fatal. The present case is presumed to have the autosomal dominant retinoblastoma gene, not associated with deletion of the q 14 band of chromosome 13. The patient succumbed to the second tumour.

Radiogen induziertes malignes fibröses Histiozytom des Oropharynx

“…Strahleninduzierte MFH treten am häufigsten im Bereich des Brustund Bauchraums nach Radiation von Mamma-und Zervixkarzinomen sowie Lymphomen auf [1,4,5,8]. Über ihr Vorkommen im Kopf-Hals-Bereich finden sich in der Literatur nur in sehr wenige Einzelfallberichte, wobei überwiegend intraossär lokalisierte Tumoren beschrieben sind [2,9,10,12,15,16].…”

Metastasis or second nonocular tumor in retinoblastoma

“…The reported incidence varies between 1 in 17000-30000 live births [13,17,19], has an equal male, female distribution and shows no racial variation [19], 20-40% of reported cases are bilateral [1,10,13,17,19,23]. Series published from Sweden [13] and the United States [10,19] report that 90% are diagnosed before the age of five years [13,19,21].…”

“…Series published from Sweden [13] and the United States [10,19] report that 90% are diagnosed before the age of five years [13,19,21]. Unilateral tumors tend to be discovered one year or more later than bilateral tumors [17,21,23].…”

“…The frequency of a family history varies from 6-36% in bilateral cases and 2-78% in unilateral cases [13,17,21]. This was initially thought to occur as an autosomal dominant gene with incomplete penetrance.…”

“…If the structure is paired such as the retina, bilateral neoplasms are highly likely. Pure double somatic mutations are characterized by unilateral neoplasms, low heritability and only 4-9% of offspring potentially effected [20], compared with familial inheritance, or a mutation involving geminal tissue when a retinoblastoma survivor has approximately a 50% chance of producing an affected child [10,17]. Subsequent work has demonstrated a retinoblastoma antioneogene at q 14 on Chromosome 13.…”