B-small lymphocytic lymphoma/chronic lymphocytic leukemia in cranio-orbital region with osteolytic performance

Abstract

Objective To report a case of B-small lymphocytic lymphoma (SLL)/chronic lymphocytic leukemia (CLL) with osteolytic performance invading the intracranial and orbital part, and to analyze the clinical manifestations, imaging features, histological patterns and immunohistochemical phenotypes, diagnosis and treatment strategies of this disease combined with review of literatures.  Methods and Results A 60-year-old female presented with left orbital swelling with intermittent headache. Head MRI showed space-occupying lesions invading left frontotemporal lobe, left greater wing of sphenoid bone, left lateral wall of sphenoid sinus, left lateral and upper orbital wall. Three-dimensional reconstructed CT showed extensive bone destruction in left frontal, temporal and sphenoid bone. The patient underwent tumor resection under general anesthesia. Histologically, the tumor cells were diffusely distributed. The nuclei were small, round and hyperchromatic, with sparse nucleoli and cytoplasm. The membrane of tumor cells were diffusely positive for CD5, positive for CD20 and CD43, partially positive for CD23, focally positive for CD138, sparsely positive for CD38 and sporadically positive for MUM1. The membrane and cytoplasm of tumor cells were positive for epithelial membrane antigen (EMA). The cytoplasm was positive for immunoglobulin κ-chain. Cyclin D1, CD10, CD56, Bcl-6, glial fibrillary acidic protein (GFAP), synaptophysin (Syn) and immunoglobulin λ-chain were negative. Ki-67 labeling index was about 70% . Final pathological diagnosis was B-SLL/CLL. The patient was treated by postoperative chemotherapy, and the 6-month follow-up showed a fine survival.  Conclusions The clinical manifestations of central nervous system (CNS) lymphomas are various, and the imaging features are atypical. A definite diagnosis depends on histopathological diagnosis. B-SLL/CLL should be differentiated from CNS metastatic tumors, other primary CNS tumors and other hematological diseases. Tumor resection guided by neuronavigation combining with chemotherapy and radiotherapy may achieve a good effect.

DOI: 10.3969/j.issn.1672-6731.2016.08.007