Bonometti Arturo, Aronico Nicola, Santacroce Giovanni, Fraticelli Sara, Lucioni Marco, Cartia Claudio Salvatore, Vanoli Alessandro, Latorre Mario Andrea, Arcaini Luca, Paulli Marco, Di Sabatino Antonio
Unit of Anatomic Pathology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, University of Pavia, Pavia, Italy.
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Front Oncol. 2022 Aug 5;12:934566. doi: 10.3389/fonc.2022.934566. eCollection 2022.
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.
浆细胞肿瘤累及胃肠道极为罕见。在此,我们描述了一例74岁的白种女性病例,该患者因腹痛、食物呕吐以及1年内体重减轻10千克前来就诊。腹部计算机断层扫描显示回肠末端环形增厚,患者因此接受了紧急的20厘米长的回肠切除术。组织病理学和免疫表型分析显示为回肠浆细胞肿瘤。随后的检查发现血清单克隆免疫球蛋白A成分、左颌骨溶骨性病变以及携带1q21扩增的克隆性骨髓浆细胞浸润。鉴于最终诊断为浆细胞骨髓瘤(PCM),患者接受了VMD(硼替佐米、美法仑和地塞米松)化疗方案,经过12个月的治疗实现了完全缓解。对于疾病复发,后来又尝试了两种化疗方案。在诊断后的最后一次随访中,4年过去了,患者仍然存活。该病例提醒人们注意浆细胞肿瘤的髓外表现,尽管罕见,但及时诊断似乎会带来更好的预后。此外,它强调了多学科方法(涉及胃肠病学家、血液学家和病理学家)在这些肿瘤诊断和管理中的相关性。