Non-secreting benign glucagonoma diagnosed incidentally in a patient with refractory thrombocytopenic thrombotic purpura: report of a case.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder, which may be idiopathic or secondary to a variety of diseases. However, there are very few reports of TTP in the context of pancreatic neoplasms. We report a case of relapsing TTP after initial treatment with plasmapheresis, corticosteroids, and rituximab, in a 59-year-old woman. During diagnostic work-up, a pancreatic lesion 35 × 25 mm in size was discovered incidentally and splenopancreatectomy was performed. The pathological diagnosis was benign glucagonoma. The hematological symptoms resolved completely after the procedure and 3 years later, the patient is well with no sign of recurrence of TTP or glucagonoma. To our knowledge, this represents the first documented case of a non-secreting benign pancreatic neuroendocrine tumor (glucagonoma) associated with TTP that is refractory to standard treatment.

• Authors: Georgios K Georgiou, Ioannis Gizas, Konstantinos P Katopodis, Christos S Katsios
• Journal: Surgery today (Surg Today) Vol. 45 Issue 10 Pg. 1317-20 (Oct 2015) ISSN: 1436-2813 [Electronic] Japan
• PMID: 25373364 (Publication Type: Case Reports, Journal Article)

Chemical References

• Rituximab
• Prednisolone
• prednylidene

Topics

• Female
• Glucagonoma (complications, diagnosis, pathology, therapy)
• Humans
• Incidental Findings
• Middle Aged
• Pancreatectomy
• Pancreatic Neoplasms (complications, diagnosis, pathology, therapy)
• Plasmapheresis
• Prednisolone (analogs & derivatives, therapeutic use)
• Purpura, Thrombotic Thrombocytopenic (etiology, therapy)
• Recurrence
• Rituximab (therapeutic use)
• Splenectomy
• Treatment Failure