Plasmacytoma of Skull Mimicking Meningioma and Metastasis

Introduction

Plasmacytoma is malignant proliferation of plasma cell, mainly arise from proliferation of a single clone of B lymphocyte 3% of which present as solitary lesion [1]. The international myeloma working group lists three types of plasmacytoma: solitary plasmacytoma of bones, extramedullary plasmacytoma & multiple plasmacytoma. Solitary plasmacytoma occurs as lytic lesion. An osteolytic plasmacytoma lesion in skull with no systemic involvement is extremely rare [2]. Multiple myeloma, extramedullary plasmacytoma and solitary bone plasmacytoma all are the three subgroup of plasma cell tumor [3].We present a case of plasmacytoma present in frontoparietal region.

Case report : A 77 years male admitted to our department with complaints of gradual enlargement of a painless swelling on his vertex for one year. On examination we found a painless mass in left frontoparietal region measuring about 8.5 × 6.4 cm in diameter which was firm in consistency, not mobile, fixed with underlying & overlying structure, on general physical examination no other abnormality detected, neurological examination was also normal. He has no significant past medical or surgical illness. On MRI of brain revealed a iso to hypointense extra axial lesion involving the left frontoparietal region measuring about 8 × 6 cm causing mass effect over the brain parenchyma.

Figure 1: MRI of brain in t1wi showing iso intense lesion

Figure 2: Scalp swelling about 8.6 × 6.5 cm

Figures 3 and 4: After intravenous gadolinium strong enhancement of lesion make it confused with meningioma.

After Intravenous gadolinium showed strong enhancement of the lesion. On CT scan of brain revealed there is a lytic lesion in left frontoparietal region causing a bone defect (Figures 5 and 6).

Figures 5 and 6: CT scan of skull in bone window shows erosion of frontoparietal part of skull.

On magnetic resonance venogram displayed the superior sagittal sinus is compressed by the tumor. Complete blood count all are within normal range.as our initial diagnosis was may be a case of meningioma or metastasis so we did all metastatic work up. Example: Thyroid scan, Ultrasonography of whole abdomen and tumor marker but all were normal, He underwent craniectomy peroperative tumor was found to involve the subcutaneous tissue to bone up to dura & which was completely extradural, tumor was moderately vascular, total removal of tumor with involved bone was removed (Figures 7 and 8).

 

Figures 7 and 8: Peroperative tumor removal, after careful dissection of skin tumor found attached with underlying bone & after craniectomy.Cranioplasty was done with bone cement (Figures 9 and 10).

Figures 9 and 10: At 4th post-operative day, en bloc removal of tumor without any residual tumor with cranioplasty.

Histopathology revealed plasmacytoma .his post-operative period was uneventful, as there was no other site of lytic lesion and we removed tumor in en bloc with part of dura so radiotherapy was not performed.

Discussion

Plasma cell tumor are divided in three type solitary plasmacytoma of bone (SPB), extramedullary plasmacytoma & multiple myeloma. Multiple myeloma is systemic disease which involved multiple osteolytic lesions, atypical plasma cell in biopsy , amyloid deposit & abnormalities in immunoglobulin production, and the others are local form of plasma cell tumor , According to Bataille and Sany, the diagnostic criteria for SPB include an isolated tumor composed of malignant plasma cells; absence of other lesions on skeletal radiographic survey; absence of plasmacytosis in the bone marrow, absence of anemia, hypercalcemia, or renal involvement & the Vertebrae and pelvic bones are mostly involved by SPB [3].Solitary plasmacytoma of skull is rare disease and considered to be curable with resection & radiotherapy [4].Due to its rarity most of the time preoperatively it is misdiagnosed as meningioma or metastasis . As compared to SPB the prognosis of solitary plasmacytoma of skull is good if it is diagnosed on strict criteria [1].So making the appropriate diagnosis is necessary for further management & follow up, though from some literature they think that solitary plasmacytoma is the initial presentation of multiple myeloma with progression of time this may convert into multiple myeloma [5].

We went through published literature (Table 1) of patients diagnosed as a case of solitary plasmacytoma of skull received en bloc removal of tumor including cranioplasty [4, 6-12].

Study

Age & Gender

Location of Tumor

Surgery

Cranioplasty

Radio-therapy

Follow up

Recurrence

 

Arienta et al., [7]

64, F

Parietal

GTR

Yes (titanium mesh)

No

3 years

No

 
 

Du Preez et al., [6]

30,F

Frontotemporal

GTR

Yes

No

1.5

No

 

Barone et al., [8]

55, F

Frontal

GTR

Yes autograft

No

9 months

No

 
 

Madsuda et al., [9]

55, F

Temporal

GTR

Yes autograft

Yes

2 years

No

 

Tanaka et al., [10]

55, M

Frontal

GTR

Yes

Yes

7 months

No

 

Gürbüz et al., [11]

63, M

Parietooccipital

GTR

Yes autograft

Yes

 

No

 

Mankotia et al., [12]

36, M

Frontal

GTR

Yes cement

Yes

3 months

No

 

Kuo et [4]

40, M

parietooccipital

GTR

Yes cement

No

1 year

No

 

Table 1: Patients with solitary plasmacytoma of skull receiving surgery published in literature.

All of those patients are histopathologically proven plasmacytoma of skull among them four patients receive only surgery, no post-operative radiotherapy or chemotherapy, and four patients received surgery along with post-operative radiotherapy, and on follow up among them seven patient has no recurrence except one patient whom post-operative follow up is not available.

Radiotherapy is the definitive treatment for solitary plasmacytoma of bone, surgery along with radiotherapy is the treatment of choice based on tumor location and type of removal of tumor, chemotherapy is not needed until there is systemic involvement as like multiple myeloma [4]. If there is gross total resection of tumor in case of isolated local tumor radiotherapy is needed or not is still in quarry, but as sometimes solitary plasmacytoma is the initial presentation of multiple myeloma regular follow up is necessary in all cases.

Conclusion

Isolated solitary plasmacytoma is a very rare tumor, en bloc tumor removal with removal of involved bone up to macroscopic healthy margin with cranioplasty is a treatment option but as radiotherapy is another option so regular follow up should be carried out.

References( create separate box for references)

  1. Gill MK, Makkar M, Bains SP (2013) Solitary plasmacytoma of skull: a rare cytological diagnosis. J Clin Diagn Res 7(8): 1702.
  2. Dong L, Zhang X, Zhang H, Song R, Gu X, et al. (2013) Solitary plasmacytoma of the skull: Two case reports. Oncol Lett 5(2): 479-482.
  3. Lae ME, Vencio EF, Inwards CY, Unni KK, Nascimento AG, et al. (2003) Myeloma of the jaw bones: a clinicopathologic study of 33 cases. Head & Neck 25(5): 373-381.
  4. Kuo Y, Huang W, Wu J (2018) Surgical Treatment for a Giant Solitary Plasmacytoma with Skull Erosion. Cureus 10(11): e3535.
  5. Yang X, Ma M, Li L, Zhang Y (2017) Case Report Solitary plasmacytoma of the skull: case report. Int J Clin Exp Pathol 10(6): 7112-7115.
  6. Du Preez JH, Branca EP (1991) Plasmacytoma of the skull. Neurosurgery 29(6): 902-906.
  7. Arienta C, Caroli M, Ceretti L, Villani R (1987) Solitary plasmacytoma of the calvarium: two cases treated by operation alone. Neurosurgery 21: 560-563.
  8. Barone CM, Jimenez DF, Argamaso RV (1992) Solitary calvarial plasmacytoma. J Craniofac Surg 3(2): 108-112.
  9. Gürbüz MS, Akmil MU, Akar E, Aker FV (2013) Solitary plasmocytoma of the skull. BMJ case reports bcr2013200379.
  10. Mankotia DS, Borkar SA, Kaur K, Suri V, Sharma BS, et al. (2017) A rare case of giant solitary calvarial plasmacytoma: can it grow bigger than this? Neurol India 65: 420-422.
  11. Matsuda M, Nakazawa T, Kizuki H, Matsumura K, Nakasu S, et al. (1996) Solitary plasmacytoma of the skull vault–case report. Neurol Med Chir (Tokyo) 36: 388-392.
  12. Tanaka M, Shibui S, Nomura K, Nakanishi Y (1998) Solitary plasmacytoma of the skull: a case report. Jap J Clin Oncol 28(10): 626-630.
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