Clinic Observation Of 25 Cases Of Extramedullary Plasmacytoma And Literature Review

Objective:To analyze the clinical features of extramedullary plasmacytoma (EMP), compare the therapeutic effect of various treatment methods for EMP, and investigate the influence of various clinical factors on EMP prognosis, in order to improve the level of diagnosis and treatment of this disease. In addition, by reviewing literature at home and abroad in recent years, we’ll analyze clinical features, evaluate the effectiveness of different treatment methods and discuss the prognostic factors of EMP.Methods:Collected and analyzed the clinical data of 25 cases with EMP from January 2005 to January 2015 in Qilu Hospital, and clinical manifestation, diagnosis, treatment and prognosis were retrospectively analyzed. All patients were diagnosed with UKMF (UK Myeloma Forum strictly, the Commonwealth myeloma forum guidelines Working Group) International Diagnostic criteria. The lab tests included blood routine, blood calcium, renal function, bone marrow aspiration and biopsy, tumor biopsy and so on. Imaging examinations used for EMP diagnosis were bone survey, CT, MRI, PET-CT, ECT and other tests. The main treatment methods included surgery alone, radiotherapy alone, chemoradiotherapy after surgery, radiotherapy after surgery. Regular corresponding imaging and laboratory examinations were adopted to monitor disease response, recurrence, progression to multiple myeloma (MM) and prognosis. In the treatment period, blood transfusion, anti infection and other symptomatic support therapy were given to the patients according to their condition.Results:Of the 25 patients,17 were male,8 female, and male to female ratio was 2:1. The age ranged from 25 to 84 years old, with a median age of 56 years. EMP can occur at any part of the body. The onset locations and case numbers of the 25 cases in present study were as follows:18 cases at head and neck (72.0%),2 cases in mediastinum (8.0%),2 case at limbs (4.0%),1 case at spinal canal (4.0%),1 case at stomach (4.0%),1 case at inguinal (4.0%). Clinical manifestations were not specific, most of them were mass generation and local compressive symptoms. The diagnosis of EMP mainly depends on pathology examination. All the 25 cases were pathologically confirmed plasmacytoma. According to the results of pathological grading, I grade was 17 cases (68%), grade II in 5 cases (20%) and grade III in 3 cases (12%). Imaging examinations should pay attention to the exclusion of solitary plasmacytoma of bone (SBP), immunohistochemistry and bone marrow aspiration to exclude MM. Pathological and immunohistochemical results were as follows:κlight chain restriction in 17 cases (68.0%), λlight chain restriction in 8 cases (32.0%). CD38 was positive in all cases, CD138 positive in 23 cases (92.0%), and 9 of them were weak positive or focally positive, CD79a positive in 17 cases (68.0%), EMA positive in 4 cases (4/19,21.2%), CD56、CD20 and CyclinD1 negative in all cases. Of the 25,6 cases (24.0%) were treated with operation alone,12 cases (48.0%) with postoperative radiotherapy,4 cases (16.0%) with postoperative radiotherapy and chemotherapy,3 cases (12.0%) with radiotherapy alone. Follow up period ranged from 17 to 98 months, with an average of 52.6 months. A total of 16 patients with EMP were followed up for more than 5 years, with an overall 5 year survival rate 56.3% (9/16); 7 patients died of EMP,6 patients with local recurrence(37.5%); 5 patients were converted to MM(31.3%).Conclusion:Extramedullary plasmacytoma(EMP) is a rare malignant tumor, and occurs mainly in middle-aged male. Head and neck are the most commonly invaded sites. Due to the unspecific clinical manifestations of EMP, it’s not easy to make early detection, diagnosis and treatment, and it’s easily misdiagnosed as other diseases for preliminary diagnosis. Ruling out MM and SBP while making the diagnosis mainly depends on the imaging and pathological examination. Recurrence may occur in some cases, and a number of cases can progress into MM and lead to poor prognosis. Local treatment is the main treatment in clinic nowadays. As EMP often occurs in the form of solid tumor, the clinical use of surgery followed with radiotherapy is the most frequent in China, and the treatment effect is quite ideal. Since it has a certain probability of recurrence and transformation, clinicians should pay attention to patient education and long-term follow-up.