Mucosa-associated lymphoid tissue is the most unique histological feature of DL. Due to its rarity, clinical characteristics and treatments for DL have not been fully elucidated to date.
A 31-year-old man with no past medical history presented with numbness in his left upper limb. Magnetic resonance imaging revealed a dural-based tumoral lesion and cervical lymphadenopathies. The lesion was diagnosed radiologically as a meningioma and a tumor resection was planned. However, intraoperative pathological diagnosis showed neoplastic lymphocytes. The planned total tumor resection was subsequently halted. Histologically, the tumor was characterized by areas of poorly defined follicular architecture consisting of medium- and large-sized centroblasts. These tumor cells were immunohistologically positive for CD10 and CD20, and negative for BCL2 and BCL6. Fluorescence in-situ hybridization did not show evidence of an IGH/BCL2 fusion. The lesion was subsequently diagnosed as a pediatric-type follicular lymphoma (PTFL). Six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) plus 2 cycles of rituximab were administered. There was no evidence of relapse 12 months after diagnosis, and the patient is still undergoing follow up.
Yamaguchi et al., described a very rare case of DL, which was diagnosed as PTFL. The tumor could be treated by immunochemotherapy alone 1).