Pancreatic islet cell carcinoma presenting with concurrent Cushing's and Zollinger-Ellison syndromes: case series and literature review

Eur J Gastroenterol Hepatol. 2010 Feb;22(2):246-52. doi: 10.1097/MEG.0b013e3283314827.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenalectomy
  • Adult
  • Aged
  • Antineoplastic Agents / therapeutic use
  • Carcinoma, Islet Cell / complications*
  • Carcinoma, Islet Cell / secondary
  • Carcinoma, Islet Cell / therapy
  • Combined Modality Therapy
  • Cushing Syndrome / etiology*
  • Cushing Syndrome / pathology
  • Cushing Syndrome / therapy
  • Embolization, Therapeutic
  • Fatal Outcome
  • Female
  • Gastrointestinal Agents / therapeutic use
  • Hormone Antagonists / therapeutic use
  • Humans
  • Liver Neoplasms / secondary
  • Pancreatic Neoplasms / complications*
  • Pancreatic Neoplasms / pathology
  • Pancreatic Neoplasms / therapy
  • Treatment Outcome
  • Zollinger-Ellison Syndrome / etiology*
  • Zollinger-Ellison Syndrome / pathology
  • Zollinger-Ellison Syndrome / therapy


  • Antineoplastic Agents
  • Gastrointestinal Agents
  • Hormone Antagonists