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Vol 60, No 3 (2009)
Case report
Submitted: 2013-02-15
Published online: 2009-04-27
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A jejunal stromal tumour in a patient with metastatic neuroendocrine cancer of unknown origin; a rare coexistence, diagnostic and therapeutic challenge

Ziad El Ali, Katarzyna Iwanik, Jerzy Sowiński, Ewelina Swora, Marian Grzymislawski
Endokrynol Pol 2009;60(3):216-220.

open access

Vol 60, No 3 (2009)
Case report
Submitted: 2013-02-15
Published online: 2009-04-27

Abstract

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.

Abstract

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.

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Keywords

metastatic neuroendocrine tumour of unknown origin; jejunal GIST; coexistence; radionuclide therapy

About this article
Title

A jejunal stromal tumour in a patient with metastatic neuroendocrine cancer of unknown origin; a rare coexistence, diagnostic and therapeutic challenge

Journal

Endokrynologia Polska

Issue

Vol 60, No 3 (2009)

Article type

Case report

Pages

216-220

Published online

2009-04-27

Page views

617

Article views/downloads

1283

Bibliographic record

Endokrynol Pol 2009;60(3):216-220.

Keywords

metastatic neuroendocrine tumour of unknown origin
jejunal GIST
coexistence
radionuclide therapy

Authors

Ziad El Ali
Katarzyna Iwanik
Jerzy Sowiński
Ewelina Swora
Marian Grzymislawski

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