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Case Report

Malignant presacral ghrelinoma with long-standing hyperghrelinaemia

, , , , , , & show all
Pages 299-304 | Received 20 Jan 2015, Accepted 12 May 2015, Published online: 20 Jun 2015
 

Abstract

Background. A 57-year old man with low-back pain was found to have a 3 × 3 × 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 177Lu-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 68Ga-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 68Ga-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.

Funding: The investigations were supported by grants from the Swedish Society of Medicine, the Foundation for Clinical Cancer Research in Jönköping, the Swedish Research Council, the Gyllenstiernska Krapperup, Fredrik and Ingrid Thuring, Magnus Bergwall, and Albert Påhlsson research funds, as well as from the Dinse Research Foundation, Hamburg, Germany.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.