title: Treatment, prognostic markers and survival in thymic neuroendocrine tumours. a study from a single tertiary referral centre.
abstract: Thymic neuroendocrine tumours (TNETs) are uncommon but malignant neoplasms, usually associated with a poor prognosis. The number of cases reported is limited to a few hundreds and there are few prognostic factors available. All 28 patients (22 male, 6 female; median age 46.5 years) with thymic neuroendocrine tumour, treated at the Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden between 1985 and 2011 were studied. The overall 3, 5 and 10-year survival was 89%, 79% and 41% respectively. Ki67<10% (p=0.018) as well as surgical resection (p=0.001) and macroscopically radical primary surgery (p=0.034) was associated with increased survival. Staging & grading according to Masaoka and ENETS systems did not correlate with survival. However, a modified ENETS grading showed a positive correlation (p=0.015). Median time to progression was 20.5 months with Temozolomide and 18 months with platinum based therapy. Partial responses were noted in three patients (38%) treated with platinum based therapy and in two patients (20%) treated with Temozolomide based therapy. High proliferative rate, measured by Ki67 index, and absence of macroscopically radical primary resection as well as no surgical resection are three negative prognostic factors in patients with TNETs. Temozolomide or Platinum based chemotherapy should be considered as first-line medical therapy in patients with metastatic or non-resectable tumours.
authors.affiliation: Experimental Surgery, Department of Surgical Sciences, Uppsala University, Uppsala, Sweden. Joakim.Crona@surgsci.uu.se
authors.forename: Joakim
authors.initials: J
authors.lastname: Crona
authors.forename: Peyman
authors.initials: P
authors.lastname: Björklund
authors.forename: Staffan
authors.initials: S
authors.lastname: Welin
authors.forename: Gordana
authors.initials: G
authors.lastname: Kozlovacki
authors.forename: Kjell
authors.initials: K
authors.lastname: Oberg
authors.forename: Dan
authors.initials: D
authors.lastname: Granberg
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000378:metabolism; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D003606:Dacarbazine / Q000008:administration & dosage / Q000031:analogs & derivatives; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D017671:Platinum Compounds / Q000008:administration & dosage; D011379:Prognosis; D012017:Referral and Consultation; D013548:Sweden; D000077204:Temozolomide; D062606:Tertiary Care Centers; D013950:Thymus Gland / Q000187:drug effects / Q000473:pathology / Q000601:surgery; D013953:Thymus Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D017671:Platinum Compounds; D003606:Dacarbazine; D000077204:Temozolomide