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Malignant presacral...
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Falkmer, Ursula G.
(author)
Malignant presacral ghrelinoma with long-standing hyperghrelinaemia
- Article/chapterEnglish2015
Publisher, publication year, extent ...
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2015-06-20
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Uppsala Medical Society,2015
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LIBRIS-ID:oai:lup.lub.lu.se:2e675ab2-8453-457d-8613-d6439739f12e
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https://lup.lub.lu.se/record/8525913URI
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https://doi.org/10.3109/03009734.2015.1054453DOI
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Language:English
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Summary in:English
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Subject category:art swepub-publicationtype
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Subject category:ref swepub-contenttype
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Background. A 57-year old man with low-back pain was found to have a 3 x 3 x 3 cm presacral neuroendocrine tumour (NET) with widespread metastases, mainly to the skeleton. His neoplastic disease responded well to peptide receptor radionuclide therapy (PRRT) with the radiotagged somatostatin agonist Lu-177-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination. Procedures. A resection of the rectum, with a non-radical excision of the adjacent NET, was made. In addition to computerized tomography (CT), receptor positron emission tomography (PET) with Ga-68-labelled somatostatin analogues was used. Observations. The NET showed the growth pattern and immunoprofile of a G2 carcinoid. A majority cell population displayed immunoreactivity to ghrelin, exceptionally with co-immunoreactivity to motilin. Somatostatin receptor scintigraphy and Ga-68-DOTATATE PET-CT demonstrated uptake in the metastatic lesions. High serum concentrations of total (desacyl-)ghrelin were found with fluctuations reflecting the severity of the symptoms. In contrast, the concentrations of active (acyl-)ghrelin were consistently low, as were those of chromogranin A (CgA).Conclusions. Neoplastically transformed ghrelin cells can release large amounts of desacyl-ghrelin, evoking an array of non-specific clinical symptoms. Despite an early dissemination to the skeleton, a ghrelinoma can be compatible with longevity after adequate radiotherapy.
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Gustafsson, Thomas
(author)
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Wenzel, Ralf
(author)
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Wierup, Nils
(author)
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Sundler, FrankLund University,Lunds universitet,Neuroendokrin cellbiologi,Forskargrupper vid Lunds universitet,Neuroendocrine Cell Biology,Lund University Research Groups(Swepub:lu)mphy-fsu
(author)
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Kulkarni, Harshad
(author)
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Baum, Richard P.
(author)
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Falkmer, Sture E.
(author)
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Neuroendokrin cellbiologiForskargrupper vid Lunds universitet
(creator_code:org_t)
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In:Uppsala Journal of Medical Sciences: Uppsala Medical Society120:4, s. 299-3040300-97342000-1967
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