Non-secreting benign glucagonoma diagnosed incidentally in a patient with refractory thrombocytopenic thrombotic purpura: report of a case.
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.
Department of Surgery, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece. georgmd7@gmail.com.
Georgios K
GK
Georgiou
Department of Surgery, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece.
Ioannis
I
Gizas
Department of Nephrology, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece.
Konstantinos P
KP
Katopodis
Department of Surgery, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece.
Christos S
CS
Katsios
D005260:Female; D005935:Glucagonoma / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D006801:Humans; D033162:Incidental Findings; D008875:Middle Aged; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D010956:Plasmapheresis; D011239:Prednisolone / Q000031:analogs & derivatives / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000628:therapy*; D012008:Recurrence; D000069283:Rituximab / Q000627:therapeutic use; D013156:Splenectomy; D017211:Treatment Failure
D000069283:Rituximab; D011239:Prednisolone; C036266:prednylidene
Glucagonoma; Pancreatectomy; Plasmapheresis; Splenectomy; TTP