原发性中枢神经系统淋巴瘤(primary central ner-vous system lymphoma,PCNSL)是原发于脑、脊髓、软脑膜或眼的结外非霍奇金淋巴瘤(non-Hodgkin’s lym-phoma,NHL),无系统受累的证据。PCNSL较罕见,仅占颅内肿瘤的4%,占结外淋巴瘤的4%~6%...原发性中枢神经系统淋巴瘤(primary central ner-vous system lymphoma,PCNSL)是原发于脑、脊髓、软脑膜或眼的结外非霍奇金淋巴瘤(non-Hodgkin’s lym-phoma,NHL),无系统受累的证据。PCNSL较罕见,仅占颅内肿瘤的4%,占结外淋巴瘤的4%~6%[1]。PCNSL具有高度侵袭性,对比脑外淋巴瘤,预后较差,5年和10年生存率分别为29.9%和22.2%[2]。近年来,PCNSL的发病率不断上升,其年化发病率为0.4/10万~0.5/10万。展开更多
A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the...A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial work-up. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, th e CD8+epidermotropic cytotoxic subset comprises rare, highly aggressive forms c haracterized by metastatic spread to unusual sites such as the oral cavity, lung s, testis and the central nervous system but usually not to the lymph nodes. The se cases seem to be distinct from mycosis fungoides with CD8+phenotype, which s hows a nonaggressive clinical behavior.展开更多
文摘原发性中枢神经系统淋巴瘤(primary central ner-vous system lymphoma,PCNSL)是原发于脑、脊髓、软脑膜或眼的结外非霍奇金淋巴瘤(non-Hodgkin’s lym-phoma,NHL),无系统受累的证据。PCNSL较罕见,仅占颅内肿瘤的4%,占结外淋巴瘤的4%~6%[1]。PCNSL具有高度侵袭性,对比脑外淋巴瘤,预后较差,5年和10年生存率分别为29.9%和22.2%[2]。近年来,PCNSL的发病率不断上升,其年化发病率为0.4/10万~0.5/10万。
文摘A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial work-up. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, th e CD8+epidermotropic cytotoxic subset comprises rare, highly aggressive forms c haracterized by metastatic spread to unusual sites such as the oral cavity, lung s, testis and the central nervous system but usually not to the lymph nodes. The se cases seem to be distinct from mycosis fungoides with CD8+phenotype, which s hows a nonaggressive clinical behavior.
