title: Peptide receptor radionuclide therapy with (90)Y/ (177)Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours).
abstract: OBJECTIVE
  Head and neck paragangliomas (HNPGLs) are rare tumours arising from autonomic nervous system ganglia. Although surgery offers the best chance of complete cure, there is associated morbidity due to the crucial location of these tumours. Radiotherapy arrests tumour growth and provides symptomatic improvement, but has long-term consequences. These tumours express somatostatin receptors (SSTR) and hence peptide receptor radionuclide therapy (PRRT) is now a treatment option. We assessed the molecular, morphological and clinical responses of inoperable HNPGLs to PRRT.
  
  
  METHODS
  Nine patients with inoperable HNPGL assessed between June 2006 and June 2014 were included. Four patients had a solitary lesion, four had multifocal involvement and one had distant metastases (bone and lungs). The patients were treated with PRRT using (90)Y/(177)Lu-labelled peptides after positive confirmation of SSTR expression on (68)Ga-DOTATOC PET/CT. All patients received two to four courses of PRRT. Subsequent serial imaging with (68)Ga-DOTATOC PET/CT was carried out every 6 months to assess response to treatment. Clinical (symptomatic) response was also assessed.
  
  
  RESULTS
  Based on molecular response (EORTC) criteria, four of the nine patients showed a partial molecular response to treatment seen as significant decreases in SUVmax, accompanied by a reduction in tumour size. Five patients showed stable disease on both molecular and morphological criteria. Six out of nine patients were symptomatic at presentation with manifestations of cranial nerve involvement, bone destruction at the primary site and metastatic bone pain. Molecular responses were correlated with symptomatic improvement in four out of these six patients; while two patients showed small reductions in tumour size and SUVmax. The three asymptomatic patients showed no new lesions or symptomatic worsening.
  
  
  CONCLUSIONS
  PRRT was effective in all patients, with no disease worsening seen, either in the form of neurological symptoms or distant spread. Though these are preliminary results, PRRT shows promise as a good treatment option for HNPGL, and hence study in a larger patient cohort is essential to establish its place in the management algorithm.
authors.affiliation: THERANOSTICS Centre for Molecular Radiotherapy and Molecular Imaging, ENETS Center of Excellence, Zentralklinik Bad Berka, Robert Koch Allee, Bad Berka, 99437, Germany.
authors.forename: Ameya D
authors.initials: AD
authors.lastname: Puranik
authors.forename: Harshad R
authors.initials: HR
authors.lastname: Kulkarni
authors.forename: Aviral
authors.initials: A
authors.lastname: Singh
authors.forename: Richard P
authors.initials: RP
authors.lastname: Baum
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006258:Head and Neck Neoplasms / Q000532:radiotherapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015282:Octreotide / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009942:Organometallic Compounds / Q000009:adverse effects / Q000627:therapeutic use*; D010235:Paraganglioma / Q000532:radiotherapy*; D019275:Radiopharmaceuticals / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: C539274:90Y-octreotate, DOTA(0)-Tyr(3)-Thr(8)-; D009942:Organometallic Compounds; D019275:Radiopharmaceuticals; C447941:lutetium Lu 177 dotatate; D015282:Octreotide
references.citation: Semin Nucl Med. 2012 May;42(3):190-207
references.pmid: 22475428
references.citation: J Clin Endocrinol Metab. 2009 Jan;94(1):5-9
references.pmid: 19001511
references.citation: J Nucl Med. 2006 Oct;47(10):1599-606
references.pmid: 17015894
references.citation: Eur J Cancer. 1999 Dec;35(13):1773-82
references.pmid: 10673991
references.citation: Clin Oncol (R Coll Radiol). 2010 Jun;22(5):382-9
references.pmid: 20206483
references.citation: J Neurosurg. 2012 Aug;117(2):246-54
references.pmid: 22680240
references.citation: Am J Surg. 1990 Apr;159(4):389-93
references.pmid: 2156465
references.citation: Recent Results Cancer Res. 2013;194:457-65
references.pmid: 22918775
references.citation: Theranostics. 2012;2(5):437-47
references.pmid: 22768024
references.citation: Eur J Cancer. 2009 Jan;45(2):228-47
references.pmid: 19097774
references.citation: Ann N Y Acad Sci. 2006 Aug;1073:21-9
references.pmid: 17102068
references.citation: J Med Genet. 2002 Sep;39(9):617-22
references.pmid: 12205103
references.citation: Endocr Relat Cancer. 2013 Aug 23;20(5):R291-305
references.pmid: 23921204
references.citation: Horm Metab Res. 2012 May;44(5):411-4
references.pmid: 22566197
references.citation: Laryngoscope. 2005 Aug;115(8):1381-4
references.pmid: 16094109
references.citation: Hum Pathol. 1979 Mar;10(2):191-218
references.pmid: 422190
references.citation: Laryngorhinootologie. 2006 Sep;85(9):649-56
references.pmid: 16612748