title: Malignant presacral ghrelinoma with long-standing hyperghrelinaemia.
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 (177)Lu-DOTATATE. During almost 10 years he was fit for a normal life. He succumbed to an intraspinal dissemination.
  
  
  METHODS
  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 (68)Ga-labelled somatostatin analogues was used.
  
  
  METHODS
  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 (68)Ga-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.
authors.affiliation: a 1 Department of Oncology, University Hospital , Aalborg, Denmark.
authors.forename: Ursula G
authors.initials: UG
authors.lastname: Falkmer
authors.affiliation: b 2 Section of Biochemistry, Department of Clinical Chemistry, Karolinska University Hospital , Stockholm, Sweden.
authors.forename: Thomas
authors.initials: T
authors.lastname: Gustafsson
authors.affiliation: a 1 Department of Oncology, University Hospital , Aalborg, Denmark.
authors.forename: Ralf
authors.initials: R
authors.lastname: Wenzel
authors.affiliation: c 3 Department of Clinical Sciences, Lund University , Lund, Sweden.
authors.forename: Nils
authors.initials: N
authors.lastname: Wierup
authors.affiliation: c 3 Department of Clinical Sciences, Lund University , Lund, Sweden.
authors.forename: Frank
authors.initials: F
authors.lastname: Sundler
authors.affiliation: d 4 Department of Nuclear Medicine, Center for PET/CT, Zentralklinik Bad Berka, ENETS Center of Excellence , Bad Berka, Germany.
authors.forename: Harshad
authors.initials: H
authors.lastname: Kulkarni
authors.affiliation: d 4 Department of Nuclear Medicine, Center for PET/CT, Zentralklinik Bad Berka, ENETS Center of Excellence , Bad Berka, Germany.
authors.forename: Richard P
authors.initials: RP
authors.lastname: Baum
authors.affiliation: e 5 Department of Pathology, County Hospital Ryhov , Jönköping, Sweden.
authors.forename: Sture E
authors.initials: SE
authors.lastname: Falkmer
mesh_terms: D001707:Biopsy, Needle; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis / Q000556:secondary* / Q000601:surgery; D018450:Disease Progression; D017809:Fatal Outcome; D054439:Ghrelin / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D017116:Low Back Pain / Q000175:diagnosis / Q000209:etiology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D049268:Positron-Emission Tomography / Q000379:methods; D012004:Rectal Neoplasms / Q000473:pathology* / Q000601:surgery; D012445:Sacrococcygeal Region; D013125:Spinal Neoplasms / Q000503:physiopathology / Q000556:secondary*
chemical_list: D054439:Ghrelin
keywords: 177Lu therapy; Desacyl/acyl-ghrelin; ghrelinoma; hyperghrelinaemia; motilin; presacral carcinoid; skeletal neuroendocrine tumour dissemination; theranostics