A jejunal stromal tumour in a patient with metastatic neuroendocrine cancer of unknown origin; a rare coexistence, diagnostic and therapeutic challenge.
A 59-year-old woman presented to the Department of Gastroenterology complaining of progressing weight loss, unexplained diarrhoea, and, as revealed by abdominal ultrasound, numerous hyperechogenic foci in the liver. The immunohistochemical evaluations of the specimens from biopsy revealed well-differentiated hepatic neuroendocrine metastases. The biochemical marker levels, including serum chromogranin A (CGA) and urine 5-hydroxyindolacetic acid (5-HIAA) 24-hour excretion, were significantly elevated. Whole body somatostatin receptor scintigraphy showed tracer accumulation in the liver lesions, with no extrahepatic tumour, possibly the primary origin. Long-term somatostatin analog therapy was initiated and a peptide-receptor targeted radionuclide therapy decision was made parallel to this treatment. Therefore, a followed-up CT scan of the abdomen showed, as well as the metastatic changes within the liver, a wellvascularised jejunal tumour suspected to be the primary focus of the disseminated neuroendocrine neoplasm. Unexpectedly, the pathological examination revealed a positive cell reaction for CD 117, confirming the diagnosis of a rare jejunal stromal tumour. Two months later peptide-receptor therapy with 90Y/77Lu-DOTA-TATE was commenced.
El Ali
Ziad
Z
Department of Internal Diseases, Metabolic Disorders and Dietetics, University of Medical Sciences, Poznan, Poland. zio73@poczta.fm
Iwanik
Katarzyna
K
Sowiński
Jerzy
J
Swora
Ewelina
E
Grzymislawski
Marian
M
D014408:Biomarkers, Tumor / Q000097:blood / Q000652:urine; D053379:Chromogranin A / Q000097:blood; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000652:urine; D007580:Jejunal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D008113:Liver Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000556:secondary*; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000188:drug therapy; D009382:Neoplasms, Unknown Primary / Q000175:diagnosis* / Q000188:drug therapy; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000188:drug therapy / Q000556:secondary*; D035583:Rare Diseases
D014408:Biomarkers, Tumor; D053379:Chromogranin A; D006897:Hydroxyindoleacetic Acid