Article

A Pancreatic Polypeptide-Producing Pancreatic Tumor Causing WDHA Syndrome

Department of Surgery, Philipps University Marburg, Marburg, Germany.
Case Reports in Gastroenterology 07/2008; 2(2):238-43. DOI: 10.1159/000142739
Source: PubMed

ABSTRACT

We report the case of a 46-year-old female patient with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome caused by a pancreatic polypeptide-producing tumor in the head of the pancreas. Whereas VIP and other pancreatic endocrine hormones were in the normal range, only serum levels of pancreatic polypeptide were elevated. Imaging studies identified a pancreatic tumor in the head of the gland. After laparotomy, the tumor of 3 cm in size was enucleated. Final pathology documented a pancreatic endocrine tumor with immunohistochemical staining demonstrating the presence of pancreatic polypeptide. The patient remained cured after a follow-up of more than three years. The present case illustrates that, although rare, WDHA syndrome may be associated with a pancreatic polypeptide-secreting endocrine tumor of the pancreas.

Download full-text

Full-text

Available from: Jens Waldmann
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Pancreatic neuroendocrine tumors (pNETs) comprise with gastrointestinal carcinoids, the main groups of gastrointestinal neuroendocrine tumors (GI-NETs). Although these two groups of GI-NETs share many features including histological aspects; over-/ectopic expression of somatostatin receptors; the ability to ectopically secrete hormones/peptides/amines which can result in distinct functional syndromes; similar approaches used for tumor localization and some aspects of treatment, it is now generally agreed they should be considered separate. They differ in their pathogenesis, hormonal syndromes produced, many aspects of biological behaviour and most important, in their response to certain anti-tumour treatment (chemotherapy, molecular targeted therapies). In this chapter the clinical features of the different types of pNETs will be considered as well as aspects of their diagnosis and medical treatment of the hormone-excess state. Emphasis will be on controversial areas or recent advances. The other aspects of the management of these tumors (surgery, treatment of advanced disease, tumor localization) are not dealt with here, because they are covered in other chapters in this volume.
    Full-text · Article · Dec 2012 · Best practice & research. Clinical gastroenterology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Neuroendocrine tumors (NETs) are slow-growing neoplasms capable of storing and secreting different peptides and neuroamines. Some of these substances cause specific symptom complexes, whereas others are silent. They usually have episodic expression, and the diagnosis is often made at a late stage. Although considered rare, the incidence of NETs is increasing. For these reasons, a high index of suspicion is needed. In this article, the different clinical syndromes and the pathophysiology of each tumor as well as the new and emerging biochemical markers and imaging techniques that should be used to facilitate an early diagnosis, follow-up, and prognosis are reviewed.
    No preview · Article · Oct 2015 · Hematology/Oncology Clinics of North America