Orbital recurrence of XXXX .

@entity1 @entity5 @entity262 @entity204 @entity995
There were 16 cases of orbital recurrence in a consecutive series of 317 @entity1 with @entity995 referred to a specialist centre. The incidence of orbital relapse amongst @entity1 treated at the centre from the outset of their disease was 2.5%. In every case the @entity1 was the first affected family member. The @entity5 was therefore not anticipated and had commonly been detected at an advanced stage. Systemic staging investigations detected extraorbital spread in six of the 16 and none of these @entity1 survived. One of the remaining ten @entity1 with no evidence of dissemination at the time of diagnosis of orbital recurrence is a long-term survivor. No @entity1 survived after orbital exenteration or radical orbital radiotherapy alone. Three @entity1 received a combination of radiotherapy and adjuvant chemotherapy. Only one of the three was free from @entity262 at relapse and this @entity1 is the only survivor. In @entity1 apparently free from widespread @entity995 at diagnosis of orbital recurrence, distant relapse was the commonest cause of @entity204 though several @entity1 died from direct intracranial extension. It is advocated that orbital recurrence of @entity995 is treated by excision biopsy of the @entity5 mass followed by radical orbital radiotherapy to a dose of 50 Gy. This should be combined with adjuvant chemotherapy and, where a risk of direct intracranial extension exists, by neuraxis irradiation. No other @entity1 in this series with evidence of local extraocular extension of @entity995 at enucleation and who had received radical orbital radiotherapy to a full dose of 50 Gy subsequently recurred in the orbit.(ABSTRACT TRUNCATED AT 250 WORDS)
