Malignant presacral ghrelinoma with long-standing hyperghrelinaemia

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.

Gustafsson, Thomas (author)
Wenzel, Ralf (author)
Wierup, Nils (author)
Sundler, FrankLund University,Lunds universitet,Neuroendokrin cellbiologi,Forskargrupper vid Lunds universitet,Neuroendocrine Cell Biology,Lund University Research Groups(Swepub:lu)mphy-fsu (author)
Kulkarni, Harshad (author)
Baum, Richard P. (author)
Falkmer, Sture E. (author)
Neuroendokrin cellbiologiForskargrupper vid Lunds universitet (creator_code:org_t)

In:Uppsala Journal of Medical Sciences: Uppsala Medical Society120:4, s. 299-3040300-97342000-1967

By the author/editor: Falkmer, Ursula ...; Gustafsson, Thom ...; Wenzel, Ralf; Wierup, Nils; Sundler, Frank; Kulkarni, Harsha ...; show more...; Baum, Richard P.; Falkmer, Sture E ...; show less...

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Cancer and Oncol ...

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