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Year : 2004  |  Volume : 14  |  Issue : 1  |  Page : 29-31
Extramedullary plasmacytoma of maxilla : Case report


Mohan Dai Oswal Cancer Hospital, G.T. Road Sherpur by Pass, Ludhiana, India

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Keywords: Plasmacytoma, Maxilla

How to cite this article:
Singh J P, Garg L, Shrimali R, Setia V, Gupta V. Extramedullary plasmacytoma of maxilla : Case report. Indian J Radiol Imaging 2004;14:29-31

How to cite this URL:
Singh J P, Garg L, Shrimali R, Setia V, Gupta V. Extramedullary plasmacytoma of maxilla : Case report. Indian J Radiol Imaging [serial online] 2004 [cited 2019 Jul 23];14:29-31. Available from: http://www.ijri.org/text.asp?2004/14/1/29/28550

   Introduction Top


Plasma cell neoplasms have been classified as multiple myeloma, solitary plasmacytoma and extramedullary plasmacytoma. The solitary plasmacytoma of maxilla is a rare condition. It is a single focus of myelomatous tissue with no dissemination to other parts of the skeleton [1]. Concurrent multiple myeloma should be ruled out by appropriate laboratory and radiologic examinations. Extramedullary plasmacytomas of the head and neck are very uncommon tumours and amongst them plasmacytoma of maxilla is extremely rare.

Local recurrence or dissemination can occur many years after the original lesion has been treated so that long term follow-up is mandatory. Plasmacytoma is a malignant tumour that is infrequently reported in extramedullary sites [2]. Such a case is being reported here for its rarity and the radiological findings which would be unusual for carcinoma, the commonest malignancy in this region.


   Case history Top


A 60 year old male came to the dept. of radiotherapy with complaints of swelling over left maxillary region and bleeding from the nose. There was no pain or tenderness. Routine blood investigations were done, Hb was 16 gm%, TLC 8400, DLC (N-58, Lym-38 and Eosin-4), Urea 25 mg/dl, creatinine 0.7 mg/dl. PNS radiograph [Figure - 1] showed a dense opacity overlying the left maxillary sinus with destruction of its floor and lateral walls. Also there is evidence of destruction of medial portion of zygomatic arch. Plain axial [Figure - 2] and coronal [Figure - 3] CT cuts were taken to know the extent of the soft tissue mass and the underlying bony destruction. An extensive soft tissue mass was seen involving the whole of the left maxillary sinus with extension in the nasal cavity medially and subcutaneous tissues of left cheek anteriorly after destruction of the medial and anterior walls respectively. The sinus cavity was expanded as a whole with remodelling of its osseous boundaries; although osseous erosion was visualised at places, it was disproportionately less for the size of the mass and for the extent of the expansion and remodelling; these features were thought to be unusual for carcinoma of maxillary sinus. So, a mitotic lesion other than carcinoma (e.g. Lymphoma) or a benign process was also considered on imaging grounds. Bone scan showed increased uptake in left maxillary, nasal and inferior orbital bone margins with intervening photopenic areas s/o destructive bone disease. No other area of increased uptake was seen. Bone marrow was normocellular with normal marrow constituents. Multiple polypoid pieces were resected and sent for histopathology. Sections showed [Figure - 4] sheets of round plasma cells which were monomorphic. Serum electrophoresis was done and it showed alb 25-4.10gm/dl, alpha 1- 0.27gm/dl, alpha 2- 0.88gm/dl, beta-0.87 and gamma-1.29. Myeloma band was not detected and urine examination showed no Bence Jones proteins. The above findings lead to the diagnosis of plasmacytoma and its further management by radiotherapy. The Patient received 30 Gy local RT and two cycles of chemotherapy with Melphalan 15 mg OD for 5 days. The tumour has regressed but there is still residual disease present, patient is now on follow-up.


   Discussion Top


Multiple myeloma is a neoplastic disorder of bone that originates from cells of bone marrow. Single focus of myelomatous tissue constitutes plasmacytoma. Plasmacytoma is a malignant tumour that is infrequently reported in extramedullary sites. It is most commonly seen within the age range of 50-80 years, cases under the age of 40 being rare. Men are affected more frequently than women. In the oral manifestations, the mandible is involved far more frequently than the maxilla, especially the most active hematopoietic areas-the ramus, angle and molar region of the mandible. Extraosseous lesions may result in paresthesia of soft tissue and gingival enlargement with bleeding tendency [3].

The nose, paranasal sinuses and nasopharynx are the most common areas involved. Seventy-five percent of extramedullary plasmacytomas arise in the upper respiratory tract, usually in the nose, nasopharynx or paranasal sinuses [4].

Plasmacytoma of the sinonasal tract appears on CT as a well defined mass, which often has expansile characteristics and is associated with bone remodelling, as well as bone erosion. There is moderate to marked enhancement after intravenous infusion of contrast material. On MR imaging T1-weighted images reveal a mass of low to intermediate signal intensity exhibiting moderate to marked contrast enhancement. On T2-weighted images, plasmacytoma demonstrates intermediate to high signal intensity [5].

The isolated plasmocytoma of the maxilla is an extremely rare tumor. It is classified in the group of low-grade B lymphomas. It's clinical and even radiological diagnosis remains difficult. Some authors, such as H.S. LOH in 1983, have attempted to regroup all reported cases in the literature, but many are incomplete, especially those which were first published. Most of the time, it is the pathological examination that rectifies the diagnosis and, currently, the immuno-chemical examination that confirms this diagnosis [6].

Microscopically the tumour is composed of densely packed round and polygonal cell structures which are scattered in relatively sparse stoma. The neoplastic cells have large, single eccentric nucleus, resembling typical plasma cells.

Good local tumour control is achieved with minimal surgery and moderate doses of radiation. Subsequent myelomatosis do occur but this is unusual and no more frequent with tumours arising in the maxilla and mandible than at extramedullary sites. It is recommended that all apparently solitary plasma cell tumours of the head and neck are treated by radiotherapy with curative intent and then followed-up carefully for life [7].

Survival rate with extramedullary plasmacytoma is good compared to multiple myeloma. However, it has been reported by Webb et al that survival rate is better in patients who develop multiple myeloma following an extramedullary plasmacytoma as compared to those who develop multiple myeloma denovo. A poor prognosis is evident in maxillary sinus plasma cell tumour as compared to other head and neck plasmacytomas. Prognosis is considered to be especially poor if radiographic examination of antrum shows bone destruction [8].

To conclude, although a rare diagnosis extramedullary plasmacytoma should also be thought of when an elderly patient presents with nose bleeding and CT shows a destructive soft tissue mass, especially when expansion and remodelling are more marked than bone destruction.

 
   References Top

1.Huang YL, Lin LM, Chen CH, Lin CC, Yan YH. Immunohistochemical detection and ultrastructural features of solitary plasmacytoma of maxillary bone-case presentation Gaoxiong Yi Xue Ke Xue Za Zhi 1990 Oct;6(10):565-71   Back to cited text no. 1    
2.Singh B, Lahiri AK, Kakar PK. Extramedullary plasmacytoma. Oral Surg Oral Med Oral Pathol 1984 Mar; 57(3):267-71.   Back to cited text no. 2    
3.Pisano JJ, Coupland R, Chen SY, Miller AS. Plasmacytoma of the oral cavity and jaws: a clinicopathologic study of 13 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997 Feb; 83(2):265-71.   Back to cited text no. 3    
4.Dolin S, Dewar JP. Extramedullary plasmacytoma. Am J Pathol 1956; 32: 83-103.   Back to cited text no. 4  [PUBMED]  
5.Rao VM, El-Noueam KI. Sinonasal imaging-Plasmacytoma. RCNA 1998; 36(5): 937.   Back to cited text no. 5    
6.Huang JS, Ho YP, Ho KY et al . Multiple myeloma with oral manifestations--report of two cases: Rev Stomatol Chir Maxillofac 1992; 93(6):377-80.   Back to cited text no. 6    
7.Gaffney CC, Dawes PJ, Jackson D. Plasmacytoma of the head and neck. Clin Radiol 1987 Jul; 38(4):385-8.   Back to cited text no. 7    
8.Chamyal PC, Mehta AK. Plasmacytoma of maxilla. J Indian medical association 1993; 91(7):183-4.  Back to cited text no. 8    

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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    Introduction
    Case history
    Discussion
    References
    Article Figures

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