Orbital Manifestation of Multiple Myeloma--A Rare Case Report

眼眶轉移性多發性骨髓瘤－罕見案例報告

吳昶儒(Chang-Ju Wu) ； 陳慶恩(Ching-En Chen) ； 石育仲(Yu-Chung Shih)

《臺灣整形外科醫學會雜誌》 30卷4期 (2021/12) Pp. 266-271

摘要

Background: Solitary extramedullary plasmacytoma (SEMP) is known as a rare plasma cell tumor which arises from outside of the bone marrow and categorized into primary and secondary. However, secondary SEMP has ever been mentioned in patients who have a history of multiple myeloma (MM) or in asymptomatic cases where the diagnosis was not yet established and tends to metastasize aggressively. Aim and Objectives: We herein present an incidentally found rare case of orbital manifestation of SEMP with confirmed relapse of MM. Material and Methods: A 75-year-old female with history of systemic MM, IgG and Lamda type, noticed a rapidly growing mass at right lower lid after the confirmation of disease relapse. Excision biopsy was performed smoothly and the final diagnosis was compatible with secondary SEMP. Results: Histopathologic slides resulted plamacytoid neoplastic cells, and the plasma cells were positive for CD138 and showed a lambda-predominant immunoreactivity, which is compatible with SEMP, or so called plasma cell myeloma. So far, the treatment of SEMP is radiotherapy with 30-60 Gy on the involved area with or without surgery resection according to clinical symptoms. Conclusion: Solitary extramedullary plasmacytoma has been mentioned in patients who have a history of MM or in asymptomatic cases where the diagnosis was not yet established. Clinicians may need to consider this rare but dreadful disease entity (SEMP) in the differential diagnosis, especially for those who had previously documented multiple myeloma.

關鍵字

multiple myeloma ； orbital ； plasma cell myeloma ； solitary extramedullary plasmacytoma

並列摘要

背景：單一性髓外漿細胞瘤在多發性骨髓瘤的病例中很罕見，並可分為原發性及繼發性。而繼發性的病灶會表現出高度侵犯性及體內轉移機率。目的及目標：本篇提出一位眼眶繼發性多發性骨髓瘤病例報告，希望透過作者診斷及治療這位患者的經驗，討論醫學文獻，讓臨床醫師熟悉此疾病的鑑別診斷。材料及方法：本篇病例報告提出一位75歲女性已診斷有多發性骨髓瘤達五年，於追蹤期間多發性骨髓瘤復發，且發現右下眼處腫瘤。經腫瘤切除手術後，病理確診為繼發性眼眶多發性骨髓瘤。並利用關鍵字眼眶多發性骨髓瘤搜尋PubMed，整理相關醫學文獻。結果：腫瘤切除後，病理報告發現許多漿細胞樣的腫瘤細胞，使用CD138免疫分析發現細胞表現出CD138，並呈現單株免疫球蛋白病變。因此診斷為繼發性單一性髓外漿細胞癌。結論：病人罹患單一性髓外漿細胞瘤時，通常已診斷有多發性骨髓瘤，但也可能先前未曾被發現有罹患多發性骨髓瘤。而臨床醫師如果發現多發性骨髓瘤患者有眼周軟組織腫瘤時，應當特別留意此單一性髓外漿細胞瘤的鑑別診斷。