Background: Primary thymic carcinomas are rare tumours with nonuniform mana
gement protocols and poor prognosis due to delayed diagnosis and highly mal
ignant behaviour. This review summarises the major clinicopathological stud
ies to assess more fully the prognostic importance of tumour histology and
staging as well as treatment benefit. Methods: A Medline search from 1966 t
o date was carried out and relevant references gleaned from these. Nineteen
clinicopathological studies form the core of this review. Demographic data
and clinical features were examined in all studies. Correlation of outcome
with histology was possible in fifteen of the studies and with tumour stag
ing in thirteen. Results: Thymic carcinomas occur most commonly in the fift
h to sixth decades of life, usually presenting with symptoms of space occup
ation or invasion. Paraneoplastic syndromes are rarely associated, except w
ith the well differentiated thymic carcinoma. Of 140 thymic carcinomas. 40
were squamous cell, 19 were spindle cell and 16 were lymphoepitheliomalike.
Completely resected stage I and II tumours demonstrated the best survival.
Conclusions: Complete surgical resection is taken to be the desired treatm
ent, but the continued high mortality and relapse rates and the variable be
nefit of adjuvant therapies challenge this strategy.