CASE 4893 Published on 18.02.2009

Plasmocytoma of thoracic spine

Section

Musculoskeletal system

Case Type

Clinical Cases

Authors

Sias A, Balestrieri A, Politi C, Mallarini G.

Patient

73 years, female

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Clinical History

An elderly lady with severe back pain, treated with NSAIDS without significant results.

Imaging Findings

The patient had long standing, severe pain in the dorsal region. The patient had been treated with NSAIDS without significant results. She had undergone plain x-rays examination of the spine, which showed age-related changes, then a spinal MR examination was performed.

Discussion

Plasmacytoma is a rare group of plasma cell neoplasm arising from the bone marrow or other various soft tissue sites, [1] divided into two groups, according to location: solitary bone plasmacytoma (SBP), and solitary extramedullary plasmacytoma (SEP).
SBP arises from bone marrow plasma cells, SEP from plasma cells of mucosal surfaces [2]. SBP is described as a single area of bone destruction, bone marrow plasma cell infiltration not > 5% of all nucleated cells, and no evidence of
myeloma elsewhere [3,4].
SBP affects fewer than 5% of patients with plasma cell disorders [3, 4] with a male-female ratio of 2:1 and a mean age of 55 years, which is 10 years earlier than multiple myeloma [2,5-8].
Pain, resulting from bony erosion by infiltrating plasma cells, is the most common symptom [3, 9]. SBP’s most common localization is the axial skeleton, (spinal disease in 34-72%), more commonly in the thoracic vertebrae [4]. Ribs, sternum, clavicle and scapula are involved in 20% of cases [10]. Patients with SBP may present with POEMS (polyneuropathy, anomegaly, endocrinopathy, M protein, and skin changes) syndrome [11]. As differential diagnosis with Non-Hodgkin lymphoma should be considered.
On plain radiographs, SBP classically has a lytic appearance with clear margins [3].
Rarely, it appears as cystic, trabeculated or sclerotic lesions [12], this latter associated with POEMS syndrome [13]. On MRI, SBP usually exhibits a low T1 signal intensity, high signal on T2-weighted/STIR images [14].
The treatment of choice is local radiotherapy; chemotherapy may be considered for unresponsive patients [3, 4, 6, 8, 15]. Local control is achieved in 88-100% of patients. The local tumour recurrence rate is approximately 10%. The usefulness of surgery is controversial: some authors claim it is contraindicated in the absence of structural instability or neurologic compromise [15] while other authors state that the curative rates of radiotherapy are higher when combined with surgical excision [16].
Up to 84% of patients progress to multiple myeloma over 3-10 years, and up to 65-100% at 15 years [15, 17]. The median overall survival time is 10 years [6], with a 10-year disease-free survival rate of 15-46%. Prognostic features for conversion of SBP to multiple myeloma, include: lesion size =/> 5 cm, patients > 40 years, high M protein levels, and its persistence after treatment [8].

Differential Diagnosis List

Solitary bone plasmacytoma, SBP, Plasmacytoma

Final Diagnosis

Solitary bone plasmacytoma, SBP, Plasmacytoma

References

[1] Di Micco P, Di Micco B: Up-date on solitary plasmacytoma and its main differences with multiple myeloma. Exp Oncol. Mar 2005; 27(1):7-12. (PMID: 15812350)

[2] Wiltshaw E: The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine (Baltimore) 1976 May; 55(3): 217-38. (PMID: 1272069)

[3] Dimopoulos MA, Moulopoulos LA, Maniatis A, Alexanian R: Solitary plasmacytoma of bone and asymptomatic multiple myeloma. Blood 2000 Sep 15; 96(6): 2037-44. (PMID: 10979944)

[4] Dimopoulos MA, Kiamouris C, Moulopoulos LA: Solitary plasmacytoma of bone and extramedullary plasmacytoma. Hematol Oncol Clin North Am 1999 Dec; 13(6): 1249-57. (PMID: 10626148)

[5] Alexiou C, Kau RJ, Dietzfelbinger H, et al: Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts. Cancer 1999 Jun 1; 85(11): 2305-14. (PMID: 10357398)

[6] Hu K, Yahalom J: Radiotherapy in the management of plasma cell tumors. Oncology (Huntingt) 2000 Jan; 14(1): 101-8, 111; discussion 111-2, 115. (PMID: 10680152)

[7] Galieni P, Cavo M, Pulsoni A, et al: Clinical outcome of extramedullary plasmacytoma. Haematologica 2000 Jan; 85(1): 47-51. (PMID: 10629591)

[8] Liebross RH, Ha CS, Cox JD, et al: Clinical course of solitary extramedullary plasmacytoma. Radiother Oncol 1999 Sep; 52(3): 245-9. (PMID: 10580871)

[9] Kyle RA: Monoclonal gammopathy of undetermined significance and solitary plasmacytoma. Implications for progression to overt multiple myeloma. Hematol Oncol Clin North Am. Feb 1997; 11(1): 71-87. (PMID: 9081205)

[10] Burt M, Karpeh M, Ukoha O, et al: Medical tumors of the chest wall. Solitary plasmacytoma and Ewing\'s sarcoma. J Thorac Cardiovasc Surg 1993 Jan; 105(1): 89-96. (PMID: 8419714)

[11] Iwashita H, Ohnishi A, Asada M, et al: Polyneuropathy, skin hyperpigmentation, edema, and hypertrichosis in localized osteosclerotic myeloma. Neurology 1977 Jul; 27(7): 675-81. (PMID: 559975)

[12] Huvos AG: Multiple myeloma including solitary osseous myeloma. In: Bone Tumors: Diagnosis, Treatment, and Prognosis. Philadelphia, Pa: WB Saunders Co; 1992:653-667.

[13] Miralles GD, O\'Fallon JR, Talley NJ: Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med 1992 Dec 31; 327(27): 1919-23. (PMID: 1333569)

[14] Moulopoulos LA, Dimopoulos MA, Weber D, et al: Magnetic resonance imaging in the staging of solitary plasmacytoma of bone. J Clin Oncol 1993 Jul; 11(7): 1311-5. (PMID: 8315427)

[15] Soutar R, Lucraft H, Jackson G, et al: Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Clin Oncol (R Coll Radiol) 2004 Sep; 16(6): 405-13. (PMID: 15487132)

[16] P Strojan, E Soba, J Lamovec, A Munda. Extramedullary plasmacytoma: clinical and histopathologic study.International Journal of Radiation Oncology Biology Physics, July 2002; 53(3) : 692-701. (PMID: 12062614)

[17] Aviles A, Huerta-Guzman J, Delgado S, et al. Improved outcome in solitary bone plasmocytoma with combined therapy. Hematol Oncol. Sept 1996: 14(3): 111 - 117. (PMID: 9119355)

Case information

URL:	https://eurorad.org/case/4893
DOI:	10.1594/EURORAD/CASE.4893
ISSN:	1563-4086

Figure 1

T1-weighted sagittal image. Isointense soft tissue mass involving the spinous process of D6, and extending into the spinal canal, with mass-like effect.