PROGRESSIVE SYSTEMIC-SCLEROSIS ASSOCIATED WITH PRIMARY SMALL-CELL CARCINOMA OF THE STOMACH

A 64-year-old man with a 5-year history of progressive systemic sclero sis (PSS) was hospitalized because of melena. Radiological and endosco pic examinations showed an ulcerative lesion with sharply demarcated a nd raised margins in the fornix of the stomach, Tumor markers-serum ca rcinoembryonic antigen (CEA, 11.3 ng/ml) and neuron-specific enolase ( NSE, 38.9 ng/ml) were elevated, Histological examination of endoscopic biopsy specimens (and of necropsy specimens) showed proliferation of atypical small round cells. Immunohistological examination of these ce lls showed they were positive for epithelial membranous antigen (EMA), and neuron-specific enolase (NSE). but negative for UCHL1, leukocyte common antigen (LCA), anti-leukocyte B-cell (MB1), and antileukocyte T -cell (MT1) antigens. Based on these histological and immunohistologic al tests, a definite diagnosis of small cell carcinoma of the stomach with PSS was established. Our case is a rare combination of PSS and ga stric small cell carcinoma. We also reviewed the literature for the as sociation between PSS and gastric cancer in Japanese patients.