Dermatomyositis and peritoneal papillary serous carcinoma

We describe an unusual case of peritoneal papillary serous carcinoma (PPSC) arising in a female patient with dermatomyositis (DM). Despite periodic ex tensive searches for an underlying malignancy, no malignancy had been detec ted in this patient during the first 2.5 years after the diagnosis of DM. I t was only when the patient presented with pleural effusion and ascites tha t the underlying intra-abdominal malignancy was detected by laparoscopy. Tr eatment with four cycles of pre-operative chemotherapy (taxol and cisplatin ) resulted in tumor regression with amelioration in the muscular manifestat ion of the DM, but without parallelic amelioration in the skin manifestatio ns of the DM. Explorative laparotomy confirmed the presence of papillary se rous carcinoma in the omentum, surface of the left ovary and the retroperit oneal lymph nodes, and established the diagnosis of PPSC. Following two cyc les of postoperative chemotherapy, the patient is alive with no evidence of internal malignancy. However, although muscle strength and enzymes have re mained normal, no effect on the skin manifestation of DM has been observed. This case illustrates that, alongside the more frequently occurring ovaria n carcinoma, PPSC should also be considered in the differential diagnosis o f the underlying malignancy that may occur in the female patient with DM. ( C) 1999 Elsevier Science Ireland Ltd. All rights reserved.