Extramedullary plasmacytoma, also known as extraosseous plasmacytoma, are the less common form of solitary plasmacytoma, manifesting as isolated plasma cell tumors located at a non-osseous site. In contrast to multiple myeloma (MM), solitary plasmacytoma have little or no systemic bone marrow involvement.
They most commonly occur in the head and neck region, rarely along the digestive tract, lung, or extremities.
- solitary plasmacytoma overall represent only 3-6% of all plasma cell disorders (i.e. multiple myeloma, plasma cell leukemia) 4
- ~1/3 of solitary plasmacytoma are extramedullary (~2% of all plasma cell disorders overall) 4,5
- occur most commonly during the fourth through to seventh decades of life
- more common in men than women
Non-specific symptoms such as pain caused by local mass effect and compression of the surrounding organs by the extramedullary plasmacytoma 7.
A plasmacytoma lesion is composed of monoclonal plasma cells arranged in clusters or sheets. In contradistinction to MM, diagnosis of plasmacytoma requires exclusion of significant bone marrow involvement.
Although plasmacytoma more commonly occur within bone, they may also occur in other organs throughout the body.
Extramedullary plasmacytoma can involve almost any location outside the bone marrow, but the following are most often recognized:
- head and neck - by far most common 5,6
- mostly involving sinuses, nasopharynx, oropharynx 5
- digestive tract 6
- lungs 6
- upper/lower extremities 6
- skin 7
- hyperamylasemia may be an associated finding
Serum amylase levels may be used as a tumor marker for monitoring tumor progression and treatment response.
CT / MRI
Cross-sectional imaging demonstrates non-specific, well-circumscribed or infiltrative, homogeneously enhancing soft tissue masses or lymphadenopathy.
There may be a mass effect on other adjacent structures such as vessels, or there may be an invasion of adjacent organs.
Active lesions demonstrate uptake of FDG on PET imaging.
Treatment and prognosis
Treatment typically is complete surgical resection, sometimes in combination with radiation therapy 7.
Prognosis for extramedullary plasmacytoma is considered better than for solitary bone plasmacytoma 4,5.
The rate of progression to multiple myeloma (MM) varies from 10% to 30%.
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- 5. Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, Touzeau C, Abildgaard N, Terpos E, Heusschen R, Ocio E, Delforge M, Sezer O, Beksac M, Ludwig H, Merlini G, Moreau P, Zweegman S, Engelhardt M, Rosiñol L. Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. (2018) Journal of hematology & oncology. 11 (1): 10. doi:10.1186/s13045-017-0549-1 - Pubmed
- 6. Ozsahin M, Tsang RW, Poortmans P, Belkacémi Y, Bolla M, Dinçbas FO, Landmann C, Castelain B, Buijsen J, Curschmann J, Kadish SP, Kowalczyk A, Anacak Y, Hammer J, Nguyen TD, Studer G, Cooper R, Sengöz M, Scandolaro L, Zouhair A. Outcomes and patterns of failure in solitary plasmacytoma: a multicenter Rare Cancer Network study of 258 patients. (2006) International journal of radiation oncology, biology, physics. 64 (1): 210-7. doi:10.1016/j.ijrobp.2005.06.039 - Pubmed
- 7. Wang J, Li J, Zhang F, Zhang P. Retroperitoneal extramedullary plasmacytoma: A case report and review of the literature. (2018) Medicine. 97 (46): e13281. doi:10.1097/MD.0000000000013281 - Pubmed