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Unusual primary osseous Hodgkin lymphoma in rib with associated soft tissue mass: a case report and review of literature

机译:肋骨中伴有软组织肿块的原发性骨性霍奇金淋巴瘤:一例病例并文献复习

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摘要

Hodgkin lymphoma (HL) typically presents as nodal lesion and may involve extranodal sites during the progression of the disease. Primary osseous HL without any lymph node association is extremely rare and only a few such cases have been described in the literature. We present a case of unusual primary HL in rib occurring in a middle-aged female patient. Computed tomography (CT) scan revealed an osteolytic lesion was located at the right second rib and was associated with a large soft tissue mass. There was no regional lymph node involvement. CT scan of neck and abdomen was performed and showed no pathologic findings, particularly no lymphadenopathy and organomegaly could be observed. Histologically, typical binucleated Reed-Sternberg (RS) cells and lacunar cells were scattered in the background of reactive inflammation with infiltration of lymphocytes, histiocytes and eosinophilic granulocytes. By immunohistochemistry, RS cells and lacunar cells were positive for CD15 and CD30 with typical membrane and paranuclear dot-like staining pattern. However, these cells were negative for Epstein-Barr virus detection by in situ hybridization. A diagnosis of primary osseous HL was made. The patient received systemic chemotherapy and local radiotherapy, and was on regular follow-up for 24 months. There was no sign of recurrence of tumor and lymph node or bone marrow involvement. Because there is a possibility of secondary bone involvement by systemic HL, strict histological analysis and thorough radiographic examination are suggested to be necessary for accurately diagnosing this tumor when it presents as a solitary bone lesion.
机译:霍奇金淋巴瘤(HL)通常表现为淋巴结病变,在疾病发展过程中可能涉及淋巴结外部位。没有任何淋巴结相关性的原发性骨HL极为罕见,文献中仅描述了少数这种情况。我们提出了一例中年女性患者发生的肋骨异常原发性HL。计算机断层扫描(CT)扫描显示,溶骨性病变位于右第二肋骨,并伴有较大的软组织肿块。没有区域淋巴结受累。进行了颈部和腹部的CT扫描,未发现任何病理学发现,特别是未观察到淋巴结肿大和器官肿大。组织学上,典型的双核Reed-Sternberg(RS)细胞和腔隙细胞散布在反应性炎症的背景下,淋巴细胞,组织细胞和嗜酸性粒细胞浸润。通过免疫组织化学,RS细胞和腔隙细胞对CD15和CD30呈阳性,具有典型的膜和核旁点状染色模式。然而,这些细胞对于通过原位杂交检测爱泼斯坦-巴尔病毒是阴性的。诊断为原发性骨HL。患者接受了全身化学疗法和局部放疗,并定期随访24个月。没有肿瘤和淋巴结复发或骨髓受累的迹象。由于系统性HL可能会继发骨累及,因此,当该肿瘤表现为孤立性骨病变时,建议进行严格的组织学分析和彻底的放射学检查对于准确诊断该肿瘤是必要的。

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