Sarcoma primario pancreático

Abstract

A 53-year-old woman with a past medical history of appendectomy and neurinoma on the left side of the neck in 1986 was admitted for asthenia and itching of recent onset, with absence of abdominal pain or any alterations on physical examination. A blood test showed: alkaline phosphatase, 1,499 IU/L, gamma-glutamyl transpeptidase, 1,163 IU/L, total bilirubin, 1.62 mg/dl, and CA-19.9, 533 IU/ml (normal value < 37 IU/ml). Doppler-US and abdominal CT scans were performed, which revealed a mass measuring 6 cm in diameter located in the head of the pancreas, with dilated intraand extra-hepatic bile ducts. The lesion had cystic areas with solid septa and papillary epithelium, some of which stained very brightly (Figs. 1 and 2); no evidence of vascular invasion or adenopathies was observed. With a diagnostic impression of resectable cystic neoplasm of the pancreas with no surgical contraindications a cephalic duodenopancreatectomy was performed. In the surgically removed tissue a solid mass of whitish color and elastic consistency was observed. The mass was located in the head of the pancreas. Although not encapsulated, it showed well-defined borders and was easily removed from the adjacent pancreas. An immunohistochemical study showed that it was positive for vimentin marker, and negative for cytokeratins (AE1-AE3), S-100, chromogranin, CD 34, C-KIT, and actin. Furthermore, a low cell proliferation index (MIB-I) was also observed (Fig. 3). These data from the histological study suggested the presence of a low-malignancy-grade fusocellular sarcoma. After a 2-year follow-up, the patient remained asymptomatic and without recurrent disease.