PDF(Pubmed)
Abstract：
Gastrointestinal (GI) involvement of plasma cell neoplasms is extremely rare. Herein, we describe the case of a 74-year-old Caucasian woman who came to our attention with abdominal pain, food vomiting, and weight loss of 10 kg over 1 year. A computed tomography scan of the abdomen revealed circumferential thickening of terminal ileum, for which the patient underwent an urgent 20-cm-long ileal resection. Histopathological and immunophenotypic analysis revealed a plasma cell neoplasm of the ileum. Subsequent investigations found a serum monoclonal immunoglobulin A component, an osteolytic lesion of the left jaw, and a clonal bone marrow plasma cell infiltrate carrying 1q21 amplification. Given the final diagnosis of plasma cell myeloma (PCM), the patient underwent a VMD (bortezomib, melphalan, and dexamethasone) chemotherapy regimen, achieving a complete remission after a 12-month treatment. For disease relapse, two further chemotherapy regimens were later attempted. At the last follow-up 4 years after the diagnosis, the patient is still alive. This case draws attention to the extramedullary presentation of plasma cell neoplasms, even if rare, as a prompt diagnosis seems to result in a better prognosis. In addition, it highlights the relevance of a multidisciplinary approach, involving gastroenterologists, hematologists, and pathologists, to the diagnosis and management of these neoplasms.
摘要：
浆细胞肿瘤的胃肠道（GI）参与极为罕见。在这里，我们描述了一个74岁的高加索女人，她因腹痛引起我们的注意，食物呕吐,和体重减轻10公斤超过1年。腹部计算机断层扫描显示末端回肠周围增厚，患者接受了紧急20厘米长的回肠切除术。组织病理学和免疫表型分析显示回肠的浆细胞肿瘤。随后的调查发现了血清单克隆免疫球蛋白A成分，左颌的溶骨性病变,克隆性骨髓浆细胞浸润携带1q21扩增。鉴于浆细胞骨髓瘤（PCM）的最终诊断，患者接受了VMD(硼替佐米，melphalan,和地塞米松）化疗方案，12个月治疗后完全缓解。对于疾病复发，随后尝试了另外两种化疗方案.在诊断后4年的最后一次随访中，病人还活着。此病例引起了对浆细胞肿瘤的髓外表现的关注，即使罕见，作为一个及时的诊断似乎导致更好的预后。此外,它强调了多学科方法的相关性，涉及胃肠病学家，血液学家,和病理学家，这些肿瘤的诊断和治疗。
