Spetsieris Nikolaos, Giannakopoulou Nefeli, Variami Eleni, Zervakis Konstantinos, Rougala Niki, Garefalakis Georgios, Skarlatou Vasiliki, Viniou Nora-Athina, Diamantopoulos Panagiotis
National and Kapodistrian University of Athens, Laikon General Hospital, 1st Department of Internal Medicine, Haematology Unit, Athens, Greece.
Medicine (Baltimore). 2018 Jan;97(3):e9608. doi: 10.1097/MD.0000000000009608.
Diffuse large B cell lymphoma (DLBCL) is a malignancy of the B cells with extranodal primary involvement being estimated at 30% to 40% of cases. Primary skeletal muscle presentation of DLBCL is extremely rare, with an estimated incidence of about 0.5% of extranodal lymphomas, presenting mostly in the lower extremities. The possible mechanisms of muscle involvement of DLBCL include primary extranodal disease, extension from adjacent organs (such as lymph nodes) or disseminated disease.
We report a case of a 70-year-old woman with an advanced initially nodal DLBCL, treated with R-CHOP, that presented with an enlargement of her left thigh and restricted mobility 3 months after completion of chemotherapy. Imaging studies were performed, which showed possible infiltration of the muscles of the left thigh, without any nodal disease present.
Muscle biopsy documented the recurrence of the lymphoma at the left thigh.
The patient started second-line treatment with gemcitabine and vinorelbine.
A partial response was achieved after the first cycle.
The remarkable element lies in the reappearance of the lymphoma at the left thigh muscles, with no radiographic or clinical evidence of involvement of lymph nodes, despite the extensive lymph node disease at initial presentation. The further management of such recurrences remains to be clarified, as the odd biological behavior of the malignant cells dictates a special handling of the disease.
弥漫性大B细胞淋巴瘤(DLBCL)是B细胞的恶性肿瘤,结外原发性受累估计占病例的30%至40%。DLBCL原发性骨骼肌表现极为罕见,估计发生率约为结外淋巴瘤的0.5%,主要发生在下肢。DLBCL累及肌肉的可能机制包括原发性结外疾病、从邻近器官(如淋巴结)蔓延或播散性疾病。
我们报告一例70岁女性,最初患有晚期淋巴结DLBCL,接受R-CHOP治疗,化疗结束3个月后出现左大腿肿大和活动受限。进行了影像学检查,结果显示左大腿肌肉可能有浸润,无任何淋巴结疾病。
肌肉活检证实左大腿淋巴瘤复发。
患者开始接受吉西他滨和长春瑞滨的二线治疗。
第一个周期后取得部分缓解。
值得注意的是,尽管最初表现为广泛的淋巴结疾病,但淋巴瘤在左大腿肌肉再次出现,影像学或临床均无淋巴结受累证据。由于恶性细胞异常的生物学行为决定了对该疾病的特殊处理方式,此类复发的进一步管理仍有待明确。