Nonfunctional pancreatic neuroendocrine carcinoma with metastasis to spleen in 17years old girl: rare case report abstract
Pancreatic neuroendocrine tumors (PNETs) have an incidence of one per 1,000,000 individuals per year. Based on the secretion status of hormones, the tumors are divided into functional and nonfunctional PNETs. Most nonfunctional PNETs are sporadic and asymptomatic. Lesions are found accidentally in first examination. Metastasis of PNET to the spleen is extremely rare, Due to our searches this is the second report of splenic metastasis of primary pancreatic neuroendocrine tumor. The spleen has been reported to be involved with
neuroendocrine tumor thrombus in the splenic vein without mass in the spleen.We present case of 17-year-old girl with intermittent upper abdominal pain specially in left upper quadrant (LUQ). Patient symptoms were anorexia, nausea, vomiting and early satiety and 10kg weight loss within last months. On physical examination she was pale, cachectic with splenomegaly.Laboratory assays showed anemia, elevated liver enzymes) SGOT, SGPT) and pancreatic enzymes(lipase, amylase). CT scan of abdomen and pelvis showed low-attenuation mass in the distal pancreatic tail measuring 5.8×5.4×7 cm with multiple splenic masses, scattered calcification and infiltration of the duodenal wall.Core needle biopsy of pancreatic tail and spleen showed cells with nesting growth and monotonous pattern. The majority of tumor cells had centrally located nucleus with moderate amounts of amphophilic cytoplasm. Nuclei were generally round to oval in shape, with coarsely clumped chromatin. Primary diagnose was nonfunctional pancreatic neuroendocrine carcinoma with metastasis to the spleen.PNETs have low incidence and currently, surgical resection with hormonal treatment is the best way to cure, so resection of the tumor (T3N0M1) was performed with deodenoctomy and splenectomy. This patient had no lymph node metastasis in initial presentation which is unusual in the presence of metastasis. The overall prognosis and long-term survival for PNET patients is related to tumor histological grade and distant metastasis.