Gianom D, Famos M, Marugg D, Oberholzer M
Medizinische Abteilung, Spital Oberengadin, Samedan.
Swiss Surg. 1999;5(1):6-10. doi: 10.1024/1023-9332.5.1.6.
Primary extramedullary plasmacytoma (EMP) is a solitary tumor that arises outside the bone marrow in patients without clinical evidence of coexisting multiple myeloma. EMP represents only 4% of all patients with plasma cell malignancies. The tumor generally occurs in the submucosal tissue of the upper airway or oral cavity. 10% of all EMP occur in the gastrointestinal tract. Only 8 cases of EMP in the duodenum have been reported in literature.
The authors present a case of EMP in the duodenum in a 53 year old men. The tumor was initially misdiagnosed as desmoid tumor. With a combined treatment of surgery and chemotherapy complete remission for 2 years was achieved, followed by a dissemination of the tumor in the testis, mandibular bone and ribs.
EMP is a rare differential diagnosis of an intraabdominal tumor. Diagnosis is difficult, and in most reported cases it is confirmed only postoperatively after immunohistochemical staining. Combined treatment of surgery and chemotherapy seem to offer the best potential for cure.
原发性髓外浆细胞瘤（EMP）是一种孤立性肿瘤，发生于无并存多发性骨髓瘤临床证据患者的骨髓外。EMP仅占所有浆细胞恶性肿瘤患者的4%。该肿瘤通常发生在上呼吸道或口腔的黏膜下组织。所有EMP中有10%发生在胃肠道。文献中仅报道过8例十二指肠EMP。
作者报告1例53岁男性十二指肠EMP病例。该肿瘤最初被误诊为硬纤维瘤。通过手术和化疗联合治疗实现了2年的完全缓解，随后肿瘤播散至睾丸、下颌骨和肋骨。
EMP是一种罕见的腹腔内肿瘤鉴别诊断。诊断困难，在大多数报道病例中仅在术后经免疫组化染色才能确诊。手术和化疗联合治疗似乎提供了最佳的治愈潜力。
