Fudala Madeline L, Karam Imad, Thida Aye M, Khan Imran, Hamadi Rachelle, Macapagal-Brown Natasha, Gupta Raavi, Preet Mohan
Hematology and Oncology, State University of New York, Downstate Health Sciences University, Brooklyn, USA.
Pathology, State University of New York, Downstate Health Sciences University, Brooklyn, USA.
Cureus. 2025 May 19;17(5):e84415. doi: 10.7759/cureus.84415. eCollection 2025 May.
Primary bone lymphoma (PBL) is a rare entity defined as a lymphoid neoplasm of bone that occurs in the bone without involvement of lymph nodes or other extranodal sites. In this report, we present a case of an elderly female with PBL who presented with regional lymphadenopathy and cutaneous lesions mimicking the primary cutaneous diffuse B-cell lymphoma (PCDBCL), leg type. This review describes the case of a 73-year-old female with primary bone lymphoma who presented with cutaneous lesions and regional lymphadenopathy. She was initially diagnosed with Paget's disease of bone but ultimately presented to the emergency room with a pathologic fracture of the left tibia and painful overlying cutaneous lesions. Imaging studies, including X-ray and computed tomography (CT) scans, along with a tibial biopsy, revealed findings consistent with PBL, characterized by large lymphoid cells that were positive for CD10, CD20, and Bcl6, with a high Ki-67 index. The diagnosis was confirmed despite the atypical cutaneous involvement. Due to her age, low-performance status, and extranodal disease, the patient was classified as high-intermediate risk according to the National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI) criteria. The presence of extranodal involvement also placed her at intermediate risk for central nervous system (CNS) disease. The clinical presentation of PBLs is often nonspecific, and diagnostic criteria noted in the literature vary, making for a difficult diagnosis in clinical practice. To our knowledge, this patient is the first documented case of PBL with concurrent cutaneous manifestations and regional lymph node involvement. This case report and literature review investigate the possibility of PBLs presenting with lesions extending beyond the initial area of bony involvement.
原发性骨淋巴瘤(PBL)是一种罕见的疾病,定义为发生于骨内的淋巴样肿瘤,不累及淋巴结或其他结外部位。在本报告中,我们呈现了一例患有PBL的老年女性病例,该患者表现为区域淋巴结肿大和皮肤损害,酷似原发性皮肤弥漫性B细胞淋巴瘤(PCDBCL)腿部型。本综述描述了一例73岁患有原发性骨淋巴瘤的女性病例,她出现了皮肤损害和区域淋巴结肿大。她最初被诊断为骨佩吉特病,但最终因左胫骨病理性骨折及疼痛的皮肤损害而就诊于急诊室。包括X线和计算机断层扫描(CT)在内的影像学检查以及胫骨活检显示结果与PBL一致,其特征为CD10、CD20和Bcl6阳性的大淋巴细胞,Ki-67指数高。尽管存在非典型的皮肤受累情况,但诊断仍得以证实。由于患者年龄、低体能状态及结外病变,根据美国国立综合癌症网络国际预后指数(NCCN-IPI)标准,该患者被归类为高中危。结外受累的存在也使其发生中枢神经系统(CNS)疾病的风险处于中度。PBL的临床表现通常不具有特异性,文献中提及的诊断标准也各不相同,这使得临床实践中的诊断较为困难。据我们所知,该患者是首例有皮肤表现并伴有区域淋巴结受累的PBL记录病例。本病例报告及文献综述探讨了PBL出现超出最初骨受累区域病变的可能性。