Pancreatic islet cell carcinoma presenting with concurrent Cushing's and Zollinger-Ellison syndromes: Case series and literature review Journal Article
| Authors: | Said, R.; O'Reilly, E. M.; Blumgart, L.; Shia, J.; Abou-Alfa, G. K. |
| Article Title: | Pancreatic islet cell carcinoma presenting with concurrent Cushing's and Zollinger-Ellison syndromes: Case series and literature review |
| Abstract: | Cushing's syndrome and Zollinger-Ellison syndrome occur occasionally as a result of neuroendocrine cancers. The concurrence of the two syndromes has been considered to confer a poor clinical and therapeutic outcome. In this study, we are reviewing two patients with pancreatic islet cell carcinomas and with both Zollinger-Ellison and Cushing's syndromes, one followed up for more than 5 years, and the other still receiving therapy, 5 years since diagnosis. A literature review showed that surgery has limited utility as the majority of these patients had metastases at the time of diagnosis. Proton-pump inhibitors, ketoconazole, and somatostatin antagonists have a major role in controlling symptoms. Interferon and systemic chemotherapeutic agents play a role in the management of metastatic and fast-growing cases. Chemoembolization and bland embolization show encouraging Results in controlling liver metastases. The latter was used effectively and more than once in the two patients presented herein. On the basis of recent molecular genetics studies, target therapy may be helpful, however, ongoing trials will define it's utility. As the data confers a worse prognosis versus other pancreatic neuroendocrine tumors, the relatively favorable outcome of the two patients reported herein may reflect the impact of multiple therapeutic modalities. © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins. |
| Keywords: | adult; human tissue; treatment outcome; aged; unclassified drug; human cell; case report; doxorubicin; chemoembolization; drug withdrawal; antineoplastic agents; liver neoplasms; combined modality therapy; pancreatic neoplasms; antineoplastic agent; dacarbazine; metastasis; computer assisted tomography; dexamethasone; laboratory test; octreotide; ketoconazole; drug substitution; fatal outcome; liver biopsy; embolization, therapeutic; ulcer; somatostatin; epigastric pain; streptozocin; pancreas islet cell carcinoma; embolization; esomeprazole; zollinger ellison syndrome; adrenalectomy; cushing's syndrome; pancreatic islet cell carcinoma; mek 0646; pantoprazole; cushing syndrome; hypercortisolism; carcinoma, islet cell; gastrointestinal agents; hormone antagonists; zollinger-ellison syndrome |
| Journal Title: | European Journal of Gastroenterology and Hepatology |
| Volume: | 22 |
| Issue: | 2 |
| ISSN: | 0954-691X |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2010-02-01 |
| Start Page: | 246 |
| End Page: | 252 |
| Language: | English |
| DOI: | 10.1097/MEG.0b013e3283314827 |
| PUBMED: | 19770666 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Export Date: 20 April 2011" - "CODEN: EJGHE" - "Source: Scopus" |
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