Progress in the diagnosis and treatment of extramedullary plasmacytoma in soft tissue_West China Medical Journal

Authors：

FENG Xiaoyue , XU Juan , TANG Wenjiao , LI Yan , PAN Ling , ZHENG Yuhuan ,  ZHANG Li

Department of Hematology and Institute of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

ZHANG Li, Email: drzhangli2014@sina.com

Keywords：

Extramedullary; soft tissue; plasma cell neoplasm; solitary

DOI：

10.7507/1002-0179.202412084

Video：

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Soft tissue plasmacytomas are classified into primary or secondary. Primary soft-tissue plasmacytomas predominantly occur in the head and neck region. When there is no bone marrow involvement, the prognosis is favorable. Genetically, they are primarily characterized by 1q amplification and del(13q). The preferred treatment is local radiotherapy or surgical excision; for extensive lymph node involvement, bortezomib is administered. Secondary soft-tissue plasmacytomas are typically seen in relapsed multiple myeloma, with a shorter survival time. They frequently harbor high-risk mutations such as del(17p) and t(4;14), requiring multi-agent intensive therapy and/or autologous hematopoietic stem cell transplantation. The prognosis of soft-tissue plasmacytomas is influenced by genetic alterations and affected anatomical sites. Future research should focus on targeting drug-resistance mechanisms and establishing diagnostic-therapeutic protocols.

Citation： FENG Xiaoyue, XU Juan, TANG Wenjiao, LI Yan, PAN Ling, ZHENG Yuhuan, ZHANG Li. Progress in the diagnosis and treatment of extramedullary plasmacytoma in soft tissue. West China Medical Journal, 2025, 40(10): 1696-1701. doi: 10.7507/1002-0179.202412084 Copy

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2.	Bansal R, Rakshit S, Kumar S. Extramedullary disease in multiple myeloma. Blood Cancer J, 2021, 11(9): 161.
3.	Kim JH, Page RD, Wang YC, et al. Soft tissue mass extramedullary plasmacytoma following radiation therapy for solitary bone plasmacytoma. Cureus, 2023, 15(8): e43927.
4.	Caers J, Paiva B, Zamagni E, et al. Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. J Hematol Oncol, 2018, 11(1): 10.
5.	Rosiñol L, Beksac M, Zamagni E, et al. Expert review on soft-tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations. Br J Haematol, 2021, 194(3): 496-507.
6.	Janjetovic S, Lohneis P, Nogai A, et al. Clinical and biological characteristics of medullary and extramedullary plasma cell dyscrasias. Biology (Basel), 2021, 10(7): 629.
7.	Billecke L, Murga Penas EM, May AM, et al. Cytogenetics of extramedullary manifestations in multiple myeloma. Br J Haematol, 2013, 161(1): 87-94.
8.	Zanwar S, Ho M, Lin Y, et al. Natural history, predictors of development of extramedullary disease, and treatment outcomes for patients with extramedullary multiple myeloma. Am J Hematol, 2023, 98(10): 1540-1549.
9.	Stork M, Sevcikova S, Minarik J, et al. Identification of patients at high risk of secondary extramedullary multiple myeloma development. Br J Haematol, 2022, 196(4): 954-962.
10.	Gao S, Dong F, Yang P, et al. 1q21+ is associated with poor prognosis in newly diagnosed multiple myeloma patients with extramedullary disease: a retrospective study. Ann Hematol, 2024, 103(6): 1979-1987.
11.	Besse L, Sedlarikova L, Greslikova H, et al. Cytogenetics in multiple myeloma patients progressing into extramedullary disease. Eur J Haematol, 2016, 97(1): 93-100.
12.	Jelinek T, Zihala D, Sevcikova T, et al. Beyond the marrow: insights from comprehensive next-generation sequencing of extramedullary multiple myeloma tumors. Leukemia, 2024, 38(6): 1323-1333.
13.	中华医学会血液学分会浆细胞疾病学组, 中国医师协会多发性骨髓瘤专业委员会. 中国髓外浆细胞瘤诊断与治疗专家共识(2024 年版). 中华血液学杂志, 2024, 45(1): 8-17.
14.	Kim D, Jeong S, Song K, et al. Genetic variations in multiple myeloma with extramedullary disease. Blood, 142(Suppl 1): 4742.
15.	Sun Z, Ji J, Li Y, et al. Identification of evolutionary mechanisms of myelomatous effusion by single-cell RNA sequencing. Blood Adv, 2023, 7(15): 4148-4159.
16.	Stork M, Ondrouskova E, Bohunova M, et al. Del(1p32) is an early and high-risk event in multiple myeloma patients with extraosseous disease. Blood Cancer J, 2024, 14(1): 146.
17.	Holler A, Cicha I, Eckstein M, et al. Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts-A follow-up. Cancer Med, 2022, 11(24): 4743-4755.
18.	Xing MH, Shaari AL, Beute JE, et al. Rare case of anaplastic plasmacytoma in the sinonasal tract. Head Neck, 2021, 43(10): E46-E50.
19.	Wu J, Chu E, Chase CC, et al. Anaplastic multiple myeloma: case series and literature review. Asploro J Biomed Clin Case Rep, 2022, 5(1): 1-11.
20.	Alencar RN, Martinez GA, Cordeiro MG, et al. Anaplastic multiple myeloma with amplification of the IGH-CCND1 gene fusion. Hematol Transfus Cell Ther, 2023, 45(4): 495-498.
21.	Qunaj L, Castillo JJ, Olszewski AJ. Survival of patients with CD20-negative variants of large B-cell lymphoma: an analysis of the National Cancer Data Base. Leuk Lymphoma, 2018, 59(6): 1375-1383.
22.	中国抗癌协会血液肿瘤专业委员会, 中华医学会血液学分会, 中国华氏巨球蛋白血症工作组. 淋巴浆细胞淋巴瘤/华氏巨球蛋白血症诊断与治疗中国指南(2022 年版). 中华血液学杂志, 2022, 43(8): 624-630.
23.	Druey KM, Arnaud L, Parikh SM. Systemic capillary leak syndrome. Nat Rev Dis Primers, 2024, 10(1): 86.
24.	李燕, 张欣怡, 唐文娇, 等. 静脉免疫球蛋白有效二级预防特发性系统性毛细血管渗漏综合征的系统评价. 中国循证医学杂志, 2023, 23(7): 797-801.
25.	Costa BA, Mouhieddine TH, Ortiz RJ, et al. Revisiting the role of alkylating agents in multiple myeloma: up-to-date evidence and future perspectives. Crit Rev Oncol Hematol, 2023, 187: 104040.
26.	Naymagon L, Abdul-Hay M. Primary extramedullary plasmacytoma with diffuse lymph node involvement: a case report and review of the literature. J Med Case Rep, 2019, 13(1): 153.
27.	Ji J, Guo R, Ma J, et al. Liquid extramedullary disease in multiple myeloma strongly predicts a poor prognosis and is associated with bortezomib resistance gene upregulation. Clin Chim Acta, 2023, 548: 117497.
28.	Kegyes D, Gulei D, Drula R, et al. Proteasome inhibition in combination with immunotherapies: state-of-the-art in multiple myeloma. Blood Rev, 2023, 61: 101100.
29.	Katodritou E, Dalampira D, Delimpasi S, et al. Central nervous system multiple myeloma: a real-world multi-institutional study of the Greek Myeloma Study Group. Am J Hematol, 2024, 99(10): 1897-1905.
30.	中国医师协会血液科医师分会, 中华医学会血液学分会. 中国多发性骨髓瘤诊治指南(2024 年修订). 中华内科杂志, 2024, 63(12): 1186-1195.
31.	Jelinek T, Sevcikova T, Zihala D, et al. Limited efficacy of daratumumab in multiple myeloma with extramedullary disease. Leukemia, 2022, 36(1): 288-291.
32.	Oka S, Ono K, Nohgawa M. Successful retreatment with elotuzumab for multiple myeloma with extramedullary relapse while being treated with lenalidomide and dexamethasone. Pathol Oncol Res, 2020, 26(3): 1993-1995.
33.	Gandhi UH, Cornell RF, Lakshman A, et al. Outcomes of patients with multiple myeloma refractory to CD38-targeted monoclonal antibody therapy. Leukemia, 2019, 33(9): 2266-2275.
34.	Nooka AK, Cohen AD, Lee HC, et al. Single-agent belantamab mafodotin in patients with relapsed/refractory multiple myeloma: Final analysis of the DREAMM-2 trial. Cancer, 2023, 129(23): 3746-3760.
35.	Baertsch MA, Fougereau M, Hielscher T, et al. Carfilzomib, lenalidomide, and dexamethasone followed by salvage autologous stem cell transplant with or without maintenance for relapsed or refractory multiple myeloma. Cancers (Basel), 2021, 13(18): 4706.
36.	Moreno DF, Clapés V, Soler JA, et al. Real-World evidence of daratumumab monotherapy in relapsed/refractory multiple myeloma patients and efficacy on soft-tissue plasmacytomas. Clin Lymphoma Myeloma Leuk, 2022, 22(8): 635-642.
37.	Togano T, Andoh S, Komuro M, et al. Bortezomib-thalidomide-dexamethasone-cisplatin-doxorubicin-cyclophosphamide-etoposide as a salvage and bridging regimen before hematopoietic stem cell transplantation for relapsed or refractory multiple myeloma. Intern Med, 2022, 61(22): 3329-3334.
38.	Ababneh HS, Yee AJ, Raje NS, et al. Radiation therapy as a bridging and salvage strategy in patients with relapsed or refractory multiple myeloma undergoing BCMA-targeted CAR T-cell therapy. Radiother Oncol, 2023, 189: 109933.
39.	Palavani LB, Bapat A, Batista S, et al. Extramedullary intracranial plasmacytomas: a systematic review of literature. Asian J Neurosurg, 2024, 19(2): 137-144.
40.	Coelho H, Badior M, Pinto P, et al. Multiple myeloma relapse as extramedullary plasmacytoma of the penis. Ann Hematol, 2023, 102(12): 3649-3651.
41.	Orzechowski M, Kowal P, Ratajczyk K, et al. Extramedullary plasmacytoma of the penis as a first manifestation of multiple myeloma: a case report. Urol Int, 2024, 108(2): 168-171.
42.	Yanagihara W, Beppu T, Ogasawara Y, et al. Primary plasmacytoma in the cerebellum: a case report and literature review. World Neurosurg, 2020, 134: 10-13.
43.	Kohut-Jackson AL, Goyal SD, Carpenter DH, et al. Solitary intracranial plasmacytoma of the brain treated with primary radiation therapy. Cureus, 2023, 15(12): e49798.
44.	McCormack S, Hamad E, Hamad A. Solitary plasmacytoma of the breast: a case of an uncommon breast neoplasm. Case Rep Hematol, 2023, 2023: 9622042.
45.	四川省医学会多发性骨髓瘤协作组. 四川省多发性骨髓瘤管理指南(2024 版). 成都: 四川科学技术出版社, 2024.

1. World Health Organization. WHO classification of tumours: haematolymphoid tumours. 5th ed. Lyon: International Agency for Research on Cancer, 2022.
2. Bansal R, Rakshit S, Kumar S. Extramedullary disease in multiple myeloma. Blood Cancer J, 2021, 11(9): 161.
3. Kim JH, Page RD, Wang YC, et al. Soft tissue mass extramedullary plasmacytoma following radiation therapy for solitary bone plasmacytoma. Cureus, 2023, 15(8): e43927.
4. Caers J, Paiva B, Zamagni E, et al. Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. J Hematol Oncol, 2018, 11(1): 10.
5. Rosiñol L, Beksac M, Zamagni E, et al. Expert review on soft-tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations. Br J Haematol, 2021, 194(3): 496-507.
6. Janjetovic S, Lohneis P, Nogai A, et al. Clinical and biological characteristics of medullary and extramedullary plasma cell dyscrasias. Biology (Basel), 2021, 10(7): 629.
7. Billecke L, Murga Penas EM, May AM, et al. Cytogenetics of extramedullary manifestations in multiple myeloma. Br J Haematol, 2013, 161(1): 87-94.
8. Zanwar S, Ho M, Lin Y, et al. Natural history, predictors of development of extramedullary disease, and treatment outcomes for patients with extramedullary multiple myeloma. Am J Hematol, 2023, 98(10): 1540-1549.
9. Stork M, Sevcikova S, Minarik J, et al. Identification of patients at high risk of secondary extramedullary multiple myeloma development. Br J Haematol, 2022, 196(4): 954-962.
10. Gao S, Dong F, Yang P, et al. 1q21+ is associated with poor prognosis in newly diagnosed multiple myeloma patients with extramedullary disease: a retrospective study. Ann Hematol, 2024, 103(6): 1979-1987.
11. Besse L, Sedlarikova L, Greslikova H, et al. Cytogenetics in multiple myeloma patients progressing into extramedullary disease. Eur J Haematol, 2016, 97(1): 93-100.
12. Jelinek T, Zihala D, Sevcikova T, et al. Beyond the marrow: insights from comprehensive next-generation sequencing of extramedullary multiple myeloma tumors. Leukemia, 2024, 38(6): 1323-1333.
13. 中华医学会血液学分会浆细胞疾病学组, 中国医师协会多发性骨髓瘤专业委员会. 中国髓外浆细胞瘤诊断与治疗专家共识(2024 年版). 中华血液学杂志, 2024, 45(1): 8-17.
14. Kim D, Jeong S, Song K, et al. Genetic variations in multiple myeloma with extramedullary disease. Blood, 142(Suppl 1): 4742.
15. Sun Z, Ji J, Li Y, et al. Identification of evolutionary mechanisms of myelomatous effusion by single-cell RNA sequencing. Blood Adv, 2023, 7(15): 4148-4159.
16. Stork M, Ondrouskova E, Bohunova M, et al. Del(1p32) is an early and high-risk event in multiple myeloma patients with extraosseous disease. Blood Cancer J, 2024, 14(1): 146.
17. Holler A, Cicha I, Eckstein M, et al. Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts-A follow-up. Cancer Med, 2022, 11(24): 4743-4755.
18. Xing MH, Shaari AL, Beute JE, et al. Rare case of anaplastic plasmacytoma in the sinonasal tract. Head Neck, 2021, 43(10): E46-E50.
19. Wu J, Chu E, Chase CC, et al. Anaplastic multiple myeloma: case series and literature review. Asploro J Biomed Clin Case Rep, 2022, 5(1): 1-11.
20. Alencar RN, Martinez GA, Cordeiro MG, et al. Anaplastic multiple myeloma with amplification of the IGH-CCND1 gene fusion. Hematol Transfus Cell Ther, 2023, 45(4): 495-498.
21. Qunaj L, Castillo JJ, Olszewski AJ. Survival of patients with CD20-negative variants of large B-cell lymphoma: an analysis of the National Cancer Data Base. Leuk Lymphoma, 2018, 59(6): 1375-1383.
22. 中国抗癌协会血液肿瘤专业委员会, 中华医学会血液学分会, 中国华氏巨球蛋白血症工作组. 淋巴浆细胞淋巴瘤/华氏巨球蛋白血症诊断与治疗中国指南(2022 年版). 中华血液学杂志, 2022, 43(8): 624-630.
23. Druey KM, Arnaud L, Parikh SM. Systemic capillary leak syndrome. Nat Rev Dis Primers, 2024, 10(1): 86.
24. 李燕, 张欣怡, 唐文娇, 等. 静脉免疫球蛋白有效二级预防特发性系统性毛细血管渗漏综合征的系统评价. 中国循证医学杂志, 2023, 23(7): 797-801.
25. Costa BA, Mouhieddine TH, Ortiz RJ, et al. Revisiting the role of alkylating agents in multiple myeloma: up-to-date evidence and future perspectives. Crit Rev Oncol Hematol, 2023, 187: 104040.
26. Naymagon L, Abdul-Hay M. Primary extramedullary plasmacytoma with diffuse lymph node involvement: a case report and review of the literature. J Med Case Rep, 2019, 13(1): 153.
27. Ji J, Guo R, Ma J, et al. Liquid extramedullary disease in multiple myeloma strongly predicts a poor prognosis and is associated with bortezomib resistance gene upregulation. Clin Chim Acta, 2023, 548: 117497.
28. Kegyes D, Gulei D, Drula R, et al. Proteasome inhibition in combination with immunotherapies: state-of-the-art in multiple myeloma. Blood Rev, 2023, 61: 101100.
29. Katodritou E, Dalampira D, Delimpasi S, et al. Central nervous system multiple myeloma: a real-world multi-institutional study of the Greek Myeloma Study Group. Am J Hematol, 2024, 99(10): 1897-1905.
30. 中国医师协会血液科医师分会, 中华医学会血液学分会. 中国多发性骨髓瘤诊治指南(2024 年修订). 中华内科杂志, 2024, 63(12): 1186-1195.
31. Jelinek T, Sevcikova T, Zihala D, et al. Limited efficacy of daratumumab in multiple myeloma with extramedullary disease. Leukemia, 2022, 36(1): 288-291.
32. Oka S, Ono K, Nohgawa M. Successful retreatment with elotuzumab for multiple myeloma with extramedullary relapse while being treated with lenalidomide and dexamethasone. Pathol Oncol Res, 2020, 26(3): 1993-1995.
33. Gandhi UH, Cornell RF, Lakshman A, et al. Outcomes of patients with multiple myeloma refractory to CD38-targeted monoclonal antibody therapy. Leukemia, 2019, 33(9): 2266-2275.
34. Nooka AK, Cohen AD, Lee HC, et al. Single-agent belantamab mafodotin in patients with relapsed/refractory multiple myeloma: Final analysis of the DREAMM-2 trial. Cancer, 2023, 129(23): 3746-3760.
35. Baertsch MA, Fougereau M, Hielscher T, et al. Carfilzomib, lenalidomide, and dexamethasone followed by salvage autologous stem cell transplant with or without maintenance for relapsed or refractory multiple myeloma. Cancers (Basel), 2021, 13(18): 4706.
36. Moreno DF, Clapés V, Soler JA, et al. Real-World evidence of daratumumab monotherapy in relapsed/refractory multiple myeloma patients and efficacy on soft-tissue plasmacytomas. Clin Lymphoma Myeloma Leuk, 2022, 22(8): 635-642.
37. Togano T, Andoh S, Komuro M, et al. Bortezomib-thalidomide-dexamethasone-cisplatin-doxorubicin-cyclophosphamide-etoposide as a salvage and bridging regimen before hematopoietic stem cell transplantation for relapsed or refractory multiple myeloma. Intern Med, 2022, 61(22): 3329-3334.
38. Ababneh HS, Yee AJ, Raje NS, et al. Radiation therapy as a bridging and salvage strategy in patients with relapsed or refractory multiple myeloma undergoing BCMA-targeted CAR T-cell therapy. Radiother Oncol, 2023, 189: 109933.
39. Palavani LB, Bapat A, Batista S, et al. Extramedullary intracranial plasmacytomas: a systematic review of literature. Asian J Neurosurg, 2024, 19(2): 137-144.
40. Coelho H, Badior M, Pinto P, et al. Multiple myeloma relapse as extramedullary plasmacytoma of the penis. Ann Hematol, 2023, 102(12): 3649-3651.
41. Orzechowski M, Kowal P, Ratajczyk K, et al. Extramedullary plasmacytoma of the penis as a first manifestation of multiple myeloma: a case report. Urol Int, 2024, 108(2): 168-171.
42. Yanagihara W, Beppu T, Ogasawara Y, et al. Primary plasmacytoma in the cerebellum: a case report and literature review. World Neurosurg, 2020, 134: 10-13.
43. Kohut-Jackson AL, Goyal SD, Carpenter DH, et al. Solitary intracranial plasmacytoma of the brain treated with primary radiation therapy. Cureus, 2023, 15(12): e49798.
44. McCormack S, Hamad E, Hamad A. Solitary plasmacytoma of the breast: a case of an uncommon breast neoplasm. Case Rep Hematol, 2023, 2023: 9622042.
45. 四川省医学会多发性骨髓瘤协作组. 四川省多发性骨髓瘤管理指南(2024 版). 成都: 四川科学技术出版社, 2024.

West China Medical Journal

Progress in the diagnosis and treatment of extramedullary plasmacytoma in soft tissue

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