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OncoReview
|
2014
|
vol. 4
|
issue 2
A76-82
EN
We reported a 58-year-old woman with tumor in the tail and body of pancreas measuring 70 mm in diameter who underwent distal pancreatectomy and splenectomy. Examination of a specimen of the pancreatic mass obtained histopathological features of a well-differentiated neuroendocrine carcinoma (WHO 2000 r. NECLM group 2, MIB < 2%). Immunohistochemical staining showed that the tumor cells were positive for chromogranin and synaptophysin. The tumor was radical resected; there were 9 lymph nodes without metastases. The patient was attending routine follow-up 3 years after resection, when ultrasonography detected hepatic tumor with a low echoic area, confirmed as at least 3 lesions in CT. The patient presented with symptoms of general malaise, anorexia, weight loss, diarrhea, and diabetes mellitus. The diagnosis including of the histopathological features resected specimen and symptoms suggested a somatostatinoma. The patient denied the surgery treatment so she was treated with good clinical and biochemical (normalization of chromogranin A) response to octreotide LAR. During follow-up 4 months after, Computer Tomography showed progression. The patient refused suggested chemotherapy streptozotocin combined with doxorubicin. We continued treatment with octreotide LAR, taking into consideration lack of symptoms and stabilization in chromogranin A level, with good result and stabilization in following Computer Tomography. Somatostatinoma originates from delta cells and is a rare neoplasm, accounting for about 1% of gastroenteropancreatic endocrine neoplasms. About half of somatostatinomas originate in the pancreas, and the remainders originate in other parts of the gastrointestinal tract, mainly in the duodenum. Measurement of the plasma somatostatin concentration is useful for making a diagnosis of somatostatinoma, however is very difficult to perform this examination in our country. Successful treatment with long-acting somatostatin analogues (octreotide LAR) has been reported after progression.
EN
Neuroendocrine tumours are a rare and heterogeneous group of neoplasms. Most of the patients are diagnosed with locally advanced or metastatic disease and curative surgery is rarely an option. Somatostatin analogues have been shown to control the symptoms and growth of well-differentiated metastatic neuroendocrine tumours. Octreotide LAR is one of the treatment options.
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