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Syndrome Resembling Graft-Versus-Host Disease in a Patient with Disseminated Carcinoma1

W Jurecka MD D H McGibbon MRCP al. 1957, 1962, Andre et al. 1966). The polyps are usually hamartomas, although adenomas are also seen. One case of gallbladder polyps in P-J syndrome has been described (Foster & Foster 1980) and one case of a papillary (villous) adenoma of the pancreatic duct associated with bile duct carcinoma and tonisillar carcinoma (Bolwell & James 1979). The relationship of P-J syndrome to gastrointestinal carcinoma remains controversial. In 1957 Bartholomew et al. reviewed the literature on the P-J syndrome and concluded that those reports documenting carcinoma arising in P-J syndrome had been misinterpreted. Reid (1974) estimated that 2-3% of patients with the P-J syndrome develop gastrointestinal carcinoma. This figure is probably not significantly different from the normal population. On the other hand, many of those who did develop carcinoma, did so before the age of 40 (Reid 1974, Perzin & Bridge 1982, Bussey 1970). More recently, Linos et al. (1981) reported no evidence of decreased survival among 21 patients with P-J syndrome followed for up to 24 years compared to a matched general population. This was contrary to the Japanese survey by Utsunomiya et al. (1975). In the most recent review by Perzin & Bridge (1982) their conclusion is that carcinomas may arise within hamartomatous polyps but are possibly preceded by adenomatous change and dysplasia. In our patient it was important to ascertain that there was no evidence of malignancy in the polyps otherwise more radical surgery would have been necessary. In fact, as this was not the case, simple biliary bypass was considered appropriate. In conclusion, obstructive jaundice occurring in a patient suffering from P-J syndrome may be due. to polyposis of the common bile duct and the authors would recommend percutaneous transhepatic cholangiography as a means of preoperative diagnosis. In the absence of malignant change and in the presence of duodenal polyposis, biliary diversion by means of hepatodochojejunostomy should be employed.