Carcinoid tumor of the jugulo-tympanic region.
Increased levels of 5-hydroxyindole acetic acid (5-HIAA) were found in a patient with a tumor arising in the middle ear. Iodine-123-metaiodobenzylguanidine ([123I]MIBG) scintigraphy and biochemical analysis showed evidence of serotonin production by the tumor. Immunohistochemistry of the tumor showed reactivity with antibodies directed against serotonin, chromogranin, leu-7 and neuron-specific enolase; S-100, met-enkephalin, leu-enkephalin and glial fibrillary acid protein were negative. This case suggests a close relationship between functioning paragangliomas and carcinoid tumors because a strong clinical and endocrinological resemblance exists. The hormonal activity found is discussed in relation to extra-adrenal paragangliomas. We recommend urinary screening not only for detection of increased levels of catecholamines, but also of 5-HIAA in all patients with paragangliomas of the head and neck. When elevated levels are found, [123I]MIBG scintigraphy should be performed to localize the areas of increased uptake in or outside the head and neck region.
Mandigers
C M
CM
Department of Internal Medicine, University Hospital and State University, Leiden, The Netherlands.
van Gils
A P
AP
Derksen
J
J
van der Mey
A G
AG
Hogendoorn
P C
PC
D019797:3-Iodobenzylguanidine; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000628:therapy; D003287:Contrast Media; D004428:Ear Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000628:therapy; D004432:Ear, Middle; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000652:urine; D007457:Iodine Radioisotopes / Q000627:therapeutic use; D007462:Iodobenzenes / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis; D011877:Radionuclide Imaging; D012701:Serotonin / Q000378:metabolism
D000970:Antineoplastic Agents; D003287:Contrast Media; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; D012701:Serotonin; D019797:3-Iodobenzylguanidine; D006897:Hydroxyindoleacetic Acid