The lack of precise and homogeneous criteria for the recognition of primary
cutaneous follicular lymphoma has hindered gaining data on the frequency a
nd clinical and molecular features of this entity. In the course of a revie
w of a series of primary cutaneous lymphoma from different Spanish hospital
s, we collected a series of 18 cases of primary cutaneous follicular lympho
ma and analyzed its clinical, morphologic, and biologic characteristics. In
this review only cases with a follicular pattern of growth, germinal cente
r cytology, and restriction to the skin in a minimum follow-up of 6 months
have been included. Cases of primary cutaneous follicular lymphoma were cha
racterized by the expression of classic markers of the germinal center, suc
h as bc16, CD10, and the presence of aggregates of follicular dendritic cel
ls. They frequently express bc12 protein, although classical t(14;18) was n
ot found in any of the cases analyzed. Analysis of the bc16 noncodifying fi
rst exon showed somatic mutations in two of four cases analyzed, as would b
e expected in lymphoma deriving from the germinal center. Clinically, most
cases showed initial involvement of the head and neck, with relapses in eig
ht cases (involving the skin in five cases, both skin and lymph node in two
cases, and lymph node in one case). No death attributable to the tumor was
recorded. These data seem to imply that follicular lymphoma may present in
itially in the skin, lacking the characteristic t(14;18) and having a relat
ively indolent course. Recognition of these tumors and elucidation of their
molecular alterations could lead to properly adapted staging and treatment
protocols for these patients.