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Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 301-302
Plasmacytoma of the sphenoid sinus presenting as unilateral loss of vision


Command Hospital, Air Force, Bangalore-560007, India

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How to cite this article:
Suri A, Gill S S, Sarma Y S. Plasmacytoma of the sphenoid sinus presenting as unilateral loss of vision. Indian J Radiol Imaging 2002;12:301-2

How to cite this URL:
Suri A, Gill S S, Sarma Y S. Plasmacytoma of the sphenoid sinus presenting as unilateral loss of vision. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Nov 19];12:301-2. Available from: http://www.ijri.org/text.asp?2002/12/2/301/28468
Sir,

Extramedullary plasmacytomas are rare lesions to diagnose,either as a part of a generalized disease or a local entity,especially in a atypical age group. It is possible for an apparent solitary plasmacytoma to be the first presenting feature of generalised disease. We present one such case -a young patient who was found to harbour a skull base plasmacytoma,which underwent rapid transformation to multiple myeloma.

A twenty- five- years old male presented with history of blurring of vision in the right eye, which progressed to total blindness in one month. There was also orbital pain and mild headache of fifteen days duration. On examination of the right eye there was absent perception to light. Ocular movements in all directions were painful. Examination of the fundus showed optic atrophy. The other eye, the other cranial nerves and the rest of the central nervous system were normal. A CT scan of the orbits and brain showed a low attenuating mass (30HU) with homogenous moderate enhancement occupying the sphenoid sinus. There was extension of the lesion to the right parasellar region and the right orbital apex and the bony contours of the right lesser wing of the sphenoid and the right anterior clinoid were eroded [Figure - 1]. The bony margins of the floor of the sphenoid and the orbital canal were intact and there were no areas of calcification within the mass, any bony expansion or sclerosis normal. The mass was removed by frontal craniotomy and histopathology examination revealed a plasmacytoma. Further investigations for multiple myeloma were carried out.M band was present on serum protein electrophoresis and serum IgG was raised (>3 gm/dl). IgM (50mg/dl) and IgA (81mg/dl) were decreased. Bence Jones protein was absent in the urine. Bone marrow aspiration revealed well-defined lytic areas plasma cells greater than 30% of the cell population. A Skeletal survey revealed well-defined lytic areas seen in the skull, proximal humerus and the lumbar vertebral bodies.

A diagnosis of multiple myeloma stage III-A was made and the patient was subjected to chemotherapy and local radiotherapy. Although his vision in the right eye did not improve he showed good objective response.

Extramedullary plasmacytoma (EMP), solitary plasmacytoma of bone and multiple myeloma (MM) are all plasma cell neoplasms. EMP and SPB each comprise 3% of all plasma cell neoplasms[1].

Although EMP is known to frequently coexist in patients of MM at autopsy, clinically only 20% of the head and neck EMP present simultaneously [2]. Fewer than 2% cases of multiple myeloma are less than 40 years [3]. The majority occur in the in the head and neck especially the nasopharynx and the paranasal sinuses with clinical symptoms like nasal obstruction, epistaxis, headache and local pain.[1] The diagnosis of multiple myeloma requires cytological, radiological and laboratory criteria.

The differential diagnosis of lesions of the sphenoid sinus with extension to the orbital apex would include soft tissue tumors like lymphomas, metastasis, meningiomas, optic gliomas and local extension by adjacent tumors of paranasal sinuses. Local escape by tumor with perineural spread including squamous cell carcinomas and adenoid cystic carcinomas are also included in orbital apex lesions. Tumors of bone such as myeloma,Ewings sarcoma,osteosarcomas, chondrosarcomas and undifferentiated sarcomas associated with Pagets disease are possible tumors to affect the orbital apex.Non tumoral orbital apex lesions include granulomatous orbital inflammatory disease (pseudotumor), sarcoid, tuberculosis and fungal disease.Occasionally multiple sclerosis,radiation neuropathy or aneurysm can present with orbital apex like syndromes.

On CT plasmacytomas are well-demarcated tumors, occasionally aggressive with bone destruction and involvement of adjacent structures.On MR they are isointense on T1 weighted images and moderate signal intensity on T2 weighted images. There is significant contrast enhancement with central inhomogenity [4].

CT and MRI can define the extent of the disease and when possible, distinguish between inflammation and neoplasia. On MRI an EMP is indistinguishable from a solitary lytic metastasis. [4]

EMP of the skull base is at a high risk for transformation to multiple myeloma especially in the early postoperative period.

In conclusion, the case highlights the unusual clinical presentation and behaviour of an sphenoid sinus EMP with its differential diagnosis.

 
   References Top

1.NofsingerYC, Miza N,Rowan PT,Lanza D,Weinstein G.Head and Neck Manifestations of Plasma Cell Neoplasms.The Laryngoscope 1997;107:741-746  Back to cited text no. 1    
2.KapadiaSB, Desai U,Cheng VS.Extramedullary Plasmacytoma of the Head and Neck-A clinicopathological study of 20 cases.Medicine 1982; 51(5): 317-329.  Back to cited text no. 2    
3.Losa M,Terrini M,Tresoldi M,MarcattiM,Campi A,Triulzi ScottiG,Giovanelli.Solitary Plasmcytoma of the Sphenoid Sinus involving the Pituatary Fossa:Acase report and review of literature.Surg Neurol 1992;37:388-393.  Back to cited text no. 3    
4.Vogl T J, Steger W,Grevers G, et al . MR Characteristics of Primary Extramedullary Plasmacytoma in the head and neck. AJNR 1996;17:1349-1354.   Back to cited text no. 4    

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Correspondence Address:
A Suri
Command Hospital, Air Force, Bangalore-560007
India
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Source of Support: None, Conflict of Interest: None


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



 

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