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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.
OncoReview
|
2016
|
vol. 6
|
issue 1
A37-42
EN
Neuroendocrine tumours (NET) are a rare and heterogeneous group of neoplasms. The majority of patients are diagnosed with locally advanced or metastatic disease, and curative surgery is rarely an option. Treatment approaches involving targeted therapy, including the use of agents inhibiting the mTOR signalling pathways involved in neuroendocrine tumourigenesis, provide new therapeutic options for patients with NETs.
OncoReview
|
2012
|
vol. 2
|
issue 4
255-261
EN
Approximately two-thirds of malignant GEP-NENs are metastatic at discovery. Chemotherapy with platinum and etoposide is a standard therapy for poorly differentiated GEP-NEC. For well-differentiated pancreatic GEP-NEN association of streptozotocin/doxorubicin and/or 5-Fu is a standard. However, it requires comparison with other established therapies. International recommendations have suggested abandoning the use of classical cytotoxics in the treatment of metastatic midgut tumours in favour of more suitable options when applicable.
PL
Nowotwory neuroendokrynne układu pokarmowego w chwili rozpoznania choroby są w stadium rozsianym u ok. 2/3 chorych. Chemioterapia z użyciem schematu cisplatyna + etopozyd pozostaje standardem w przypadku zaawansowanych postaci niskozróżnicowanych raków neuroendokrynnych (NEC G3 wg klasyfikacji WHO z 2010 r.). W przypadku dobrze zróżnicowanych nowotworów neuroendokrynnych trzustki streptozotocyna, doksorubicyna i/lub 5-Fu są uważane za chemioterapię standardową, mimo że nie ma randomizowanych badań porównujących ją z innymi terapiami. Międzynarodowe wytyczne nie rekomendują stosowania chemioterapii w rozsianych nowotworach neuroendokrynnych pochodzących ze środkowego odcinka prajelita (midgut). Chorzy ci powinni być kwalifikowani do innego typu leczenia.
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.
EN
Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant hereditary disorder characterised by coexistence of pancreatic neuroendocrine tumours (pNETs) with parathyroid and pituitary tumours. PNETs, including mostly non-functioning tumours, gastrinoma and insulinoma, occur in nearly 95% of MEN1 patients and account for over 50% of disorder-related mortality. Therefore, early initiation of screening for pNET using biochemical and imaging tests as well as appropriate surgical and systemic treatment are of particular importance for this group of patients. Currently, there are no clearly defined guidelines which determine the optimal methods for detection and treatment of pNET in MEN1. Caution should be exercised when applying the guidelines designed for patients with sporadic pNET to MEN1 patients as the clinical course of the disorder is slightly different, involving multifocality of lesions and younger age of patients at onset. This paper discusses the distinctive features and challenges in diagnosing and treating pNETs in MEN1 patients.
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