Antunes Maria Inês, Bujor Laurentiu, Grillo Isabel Monteiro
Radiotherapy Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal.
Radiotherapy Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal ; Faculty of Medicine, Lisbon University, Portugal.
Rep Pract Oncol Radiother. 2010 Dec 30;16(1):36-9. doi: 10.1016/j.rpor.2010.12.002. eCollection 2010.
Extramedullary plasmacytomas (EMP) are rare plasma cell tumors that arise outside the bone marrow. They are most often located in the head and neck region, but may also occur in the other locations. The lower gastrointestinal EMP represents less than 5% of all cases, and location in the anal canal is exceedingly rare.
We present an exceedingly rare case of anal canal plasmacytoma, aiming to achieve a better understanding of this rare entity.
We report a case of a 61-year-old man with a bulky mass in the anal canal. The lesion measured about 6 cm and invaded in all layers of the anal canal wall. The biopsy was performed and revealed a round and plasmocitoid cell population with a solid growth pattern and necrosis. The tumoral cells have express CD79a and CD138 with lambda chains. There was no evidence of disease in other locations and these features were consistent with the diagnosis of an extra-osseous plasmacytoma. The patient was submitted to conformal radiotherapy 50.4 Gy total dose, 1.8 Gy per fraction. After 24 months, the patient is asymptomatic and the lesion has completely disappeared.
EMP accounts for approximately 3% of plasma cell malignancies. The median age is about 60 years, and the majority of patients are male. The treatment of choice for extramedullary plasmacytoma is radiation therapy in a dosage of about 50 Gy. Patients should be followed-up for life with repeated bone marrow aspiration and protein studies to detect the development of multiple myeloma.
髓外浆细胞瘤（EMP）是一种罕见的起源于骨髓外的浆细胞肿瘤。它们最常位于头颈部区域，但也可能发生在其他部位。下消化道EMP占所有病例的比例不到5%，而位于肛管的情况极为罕见。
我们报告一例极为罕见的肛管浆细胞瘤病例，旨在更好地了解这种罕见疾病。
我们报告一例61岁男性患者，其肛管内有一巨大肿物。该病变大小约6厘米，侵犯了肛管壁的全层。进行了活检，结果显示为圆形和浆细胞样细胞群体，呈实体生长模式并有坏死。肿瘤细胞表达CD79a和CD138以及λ链。其他部位无疾病证据，这些特征符合骨外浆细胞瘤的诊断。患者接受了总剂量50.4 Gy、每次分割剂量1.8 Gy的适形放疗。24个月后，患者无症状，病变已完全消失。
EMP约占浆细胞恶性肿瘤的3%。中位年龄约为60岁，大多数患者为男性。髓外浆细胞瘤的首选治疗方法是剂量约为50 Gy的放射治疗。应终身随访患者，反复进行骨髓穿刺和蛋白研究以检测多发性骨髓瘤的发生。
