Transformation of primary cutaneous follicle centre lymphoma into primary cutaneous diffuse large B-cell lymphoma of other type

Non-Hodgkin lymphomas (NHL) arising from the skin with no evidence of extracutaneous involvement, at the time of diagnosis, are recognized as primary cutaneous lymphomas (PCL). Primary cutaneous lymphomas have to be distinguished from other nodal NHL affecting skin secondarily. Therefore, the European Society for Medical Oncology (ESMO) observed PCL as separate entities from their nodal/systemic counterparts defining distinct diagnostic, treatment and follow-up guidelines.
Skin is the second most affected organ by extranodal NHL with an estimated annual incidence of 1/100 000 in Western countries [1]. Within the group of PCL, cutaneous T-cell lymphomas (CTCL) and cutaneous B-cell lymphomas (CBCL) can be distinguished [2]. Geographically, their distribution varies among different world regions. Regarding data from the Western world, CTCL are the most common with an estimated incidence of 75–80%, while CBCL are found to be rare with an incidence of 20–25% [2, 3]. Specifically, the region of southeast Asian countries have a presumable high incidence of CTCL, in particular Epstein-Baar virus associated natural killer/T-cell lymphomas, while CBCL are very uncommon [4, 5].
According to the World Health Organization (WHO) and the European Organization for Research and Treatment of Cancer (EORTC), it has been proposed that CBCL should be divided into the following types: primary cutaneous follicle center lymphomas (PCFCL), cutaneous marginal zone lymphomas (PCMZL), and cutaneous large B-cell lymphomas (CLBCL). Furthermore, CLBCL can be divided to leg skin, the other, and exceedingly rare type CLBCL. A summarized review is presented in Table 1.
We report a rare case of CBCL with a long-term clinical course of an indolent PCFCL transforming to a more aggressive, rare variant, of other type (PC-DLBCL-O), stressing a natural biological event in lymphoma biology.
A 60-year-old Serbian male diagnosed with PC-DLBCL-O was admitted to our Institution for staging and decision on the treatment strategy.
The disease primarily appeared in 2001, 13 years prior to actual dissemination. It presented as a solitary erythematous macular-papular lesion of the middle chest skin, which was surgically removed. The pathohistology revealed low-grade LCA+, CD20+, MPO- CBCL composed of small to medium-sized lymphoid cells (centrocytes) some tissue specimens of which had a nodular growth pattern. According to the WHO 2008 classification, this lesion highly...


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