Malignant gastric ghrelinoma with hyperghrelinemia.
A characteristic feature of neuroendocrine tumors is production and release of peptide hormone. Ghrelin is a 28-amino acid hormone that stimulates GH release. In this paper, we describe a patient with a metastasizing gastric neuroendocrine tumor displaying intense immunoreactivity for ghrelin and extremely high circulating levels of ghrelin. Tumor tissue biopsies from the primary tumor and one liver metastasis were examined by immunohistochemistry. Ghrelin and several other hormones and tumor markers were measured in blood. The clinical course of the patient was followed. Tumor tissue biopsies showed immunoreactivity for cytokeratin, chromogranin A, human synaptic vesicle protein 2, synaptophysin, and ghrelin. Grossly elevated circulating levels of total ghrelin, 2100 microg/liter (reference interval < 5 microg/liter) and active ghrelin, 28 microg/liter (reference interval < 0.1 microg/liter) were found at presentation. Chromogranin A, chromogranin B, and calcitonin levels were also increased. Both total and active ghrelin increased, despite treatment, during follow-up of the patient. We have identified and characterized a patient with a malignant gastric neuroendocrine tumor secreting ghrelin as the main hormone. This might be a new tumor entity of the stomach, and it is suggested that patients with malignant gastric neuroendocrine tumors should be investigated for ghrelin production.
Tsolakis
Apostolos V
AV
Department of Medical Sciences, Section of Endocrine Oncology, Uppsala University Hospital, 751 85 Uppsala, Sweden. apostolos.tsolakis@medsci.uu.se
Portela-Gomes
Guida M
GM
Stridsberg
Mats
M
Grimelius
Lars
L
Sundin
Anders
A
Eriksson
Barbro K
BK
Oberg
Kjell E
KE
Janson
Eva T
ET
D014408:Biomarkers, Tumor / Q000097:blood; D018278:Carcinoma, Neuroendocrine / Q000097:blood* / Q000175:diagnosis / Q000473:pathology / Q000556:secondary; D017809:Fatal Outcome; D054439:Ghrelin; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000556:secondary; D008297:Male; D008875:Middle Aged; D036361:Peptide Hormones / Q000097:blood*; D013274:Stomach Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D014055:Tomography, Emission-Computed; D014057:Tomography, X-Ray Computed
D014408:Biomarkers, Tumor; D054439:Ghrelin; D036361:Peptide Hormones