Vusqa Urwat Til, Asawa Palash, Fazal Salman, Samhouri Yazan
Department of Internal Medicine, Allegheny Health Network, Pittsburgh, PA, U.S.A.
Division of Hematology and Cellular Therapy, Allegheny Health Network Cancer Institute, Pittsburgh, PA, U.S.A.
Cancer Diagn Progn. 2022 Sep 3;2(5):549-552. doi: 10.21873/cdp.10140. eCollection 2022 Sep-Oct.
BACKGROUND/AIM: Extramedullary plasmacytoma (EMP) is defined as a localized plasma cell neoplasm that arises in tissues other than the bone. The most common sites of involvement of EMP are the upper airways followed by lymph nodes, gastrointestinal tract, thyroid gland, skin, brain, liver, and lungs. Testicular plasmacytoma has a very rare occurrence with about 70 cases reported in literature to date.
We describe a 52-year-old-male with a diagnosis of multiple myeloma presenting with lytic lesions of the axial skeleton. He had lambda light chain restricted, R-ISS stage II with high risk cytogenetics as he tested positive for t(4;14). He underwent four cycles of cyclophosphamide, bortezomib and dexamethasone followed by auto-peripheral stem cell transplantation. He was kept on ixazomib, lenalidomide and dexamethasone maintenance therapy, but relapsed soon after and was diagnosed with plasmacytoma of the left lung. Therapy was switched to daratumumab, carfilzomib and dexamethasone and the patient received radiation of his left lung. He then developed left painless testicular mass which was treated with six weeks course of antibiotics. However due to persistence of concerning features on scrotal ultrasound post-treatment, the patient underwent radical orchiectomy with pathology coming back positive for plasma cells.
The testes serve as a sanctuary site for hematological malignancies due to the presence of the testicular-blood barrier. Hence, it is imperative to keep a high index of suspicion for testicular plasmacytoma in the right clinical context when evaluating a patient with known multiple myeloma.
背景/目的:髓外浆细胞瘤(EMP)被定义为一种起源于骨骼以外组织的局限性浆细胞肿瘤。EMP最常见的受累部位是上呼吸道,其次是淋巴结、胃肠道、甲状腺、皮肤、脑、肝和肺。睾丸浆细胞瘤非常罕见,迄今为止文献报道约70例。
我们描述了一名52岁男性,诊断为多发性骨髓瘤,伴有中轴骨溶骨性病变。他的λ轻链受限,R-ISS分期为II期,细胞遗传学高危,因为他检测t(4;14)呈阳性。他接受了四个周期的环磷酰胺、硼替佐米和地塞米松治疗,随后进行了自体外周干细胞移植。他接受了伊沙佐米、来那度胺和地塞米松维持治疗,但很快复发,被诊断为左肺浆细胞瘤。治疗改为达雷妥尤单抗、卡非佐米和地塞米松,患者接受了左肺放疗。然后他出现了左侧无痛性睾丸肿块,接受了六周疗程的抗生素治疗。然而,由于治疗后阴囊超声检查仍存在可疑特征,患者接受了根治性睾丸切除术,病理检查显示浆细胞阳性。
由于存在血睾屏障,睾丸是血液系统恶性肿瘤的一个庇护所。因此,在评估已知多发性骨髓瘤的患者时,在正确的临床背景下,必须对睾丸浆细胞瘤保持高度怀疑。