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Indian Journal of Cancer
Medknow Publications on behalf of Indian Cancer Society
ISSN: 0019-509X EISSN: 1998-4774
Vol. 47, Num. 3, 2010, pp. 347-348

Indian Journal of Cancer, Vol. 47, No. 3, July-September, 2010, pp. 347-348

Letter To Editor

Unusual presentation of multiple myeloma

Department of Radiation Oncology, 1 Department of Medicine, Kasturba Medical College, Mangalore – 575 001, India

Correspondence Address: Dr. DS Suddharsan, Department of Medicine, Kasturba Medical College, Mangalore - 575 001, India
drsuddharsan@gmail.net

Code Number: cn10084

PMID: 20587918

DOI: 10.4103/0019-509X.64709

Sir,

Plasmacytoma may be osseous (medullary) or nonosseous (extramedullary) and primary or secondary to disseminated multiple myeloma (MM). The skull base is one of the rarest sites of solitary plasmacytoma of the bone. [1] We present here a rare case of a patient with skull base plasmacytoma as the presenting sign of MM.

A 58-year-old man with hypertension presented with a 3-month history of left-sided hearing loss, vertigo, and headache. On examination, there was left-sided sensory neural deafness. Laboratory tests showed normal hemogram, electrolytes, as well as renal and liver function. A possibility of cervical spondylosis was considered. Head and cervical spine magnetic resonance imaging [Figure - 1] revealed destruction of the clivus that was replaced by a moderately enhancing mass lesion, which was hypointense on unenhanced T1-weighted images and iso- to hypointense on T2-weighted images. Laterally, the lesion infiltrated both petrous apices, mostly on the left side, infiltrating the left jugular bulb and extending posterolaterally up to the left sigmoid sinus in the left cerebellopontine angle cistern. Computed tomography of Head [Figure - 2] showed an osteolytic lesion with areas of spotty calcification. Skeletal radiological work-up was normal. A transnasal biopsy of the lesion showed plasmacytoma of the bone [Figure - 3]. b2-Microglobulin was 2373.0 ng/mL and serum protein electrophoresis did not reveal M-band. Urine was negative for Bence Jones proteins. Peripheral smear was normal. A bone marrow aspiration study showed 41% of atypical plasma cells, binucleate forms, and plasma blast, suggestive for MM. The patient was treated with 6 cycles of high dose dexamethasone (40 mg/d intravenously for 4 d/cycle) and thalidomide (200 mg/d orally for 28 d/cycle), and local irradiation (40 Gy 20 fractions over 4 wk). After completion of this treatment, he has been referred for bone marrow transplantation.

Plasma cell neoplasm included MM, monoclonal gammapathies of unknown significance, plasmacytomas, and plasma cell leukemia. [2] MM is the most common type of plasma cell neoplasm. Plasmacytoma represents a local form of plasma cell neoplasm. [3] Plasmacytomas represent less than 1% of all head and neck tumors. [4] Primary extramedullary plasmacytomas represent less than 4% of all plasma cell tumors, most often involving the upper aerodigestive tracts and paranasal sinuses. [4],[5] Solitary intracranial plasmacytoma is exceedingly rare, [6] and may arise from the skull, leptomeninges, or the brain. When the skull is involved, most of the plasmacytomas occur in the calvarium and the skull base is rarely affected. Here, they may occur in the clivus, such as in the case presented herein, or in the petrous apex.

In conclusion, this is a rare clinical presentation since the patient did not have any other features of MM and it highlights the importance of extensive evaluation of patients with atypical neurological symptoms.

References

1.Goyal R, Gupta R, Radotra BD. Plasmacytoma of the clivus: A case report. Indian J Pathol Microbiol 2006;49:568-70.  Back to cited text no. 1  [PUBMED]  
2.Pollard JD, Young GA. Neurology and the bone marrow. J Neurol Neurosurg Psychiatry1997;63:706-18.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Ustuner Z, Basaran M, Kiris T, Bilgic B, Sencer S, Sakar B, et al. Skull base plasmacytoma in a patient with light chain myeloma. Skull Base 2003;13:167-71.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Nofsinger YC, Mirza N, Rowan PT, Lanza D, Weinstein G. Head and neck manifestations of plasma cell neoplasms. Laryngoscope 1997;107:741-6.  Back to cited text no. 4  [PUBMED]  
5.International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the International Myeloma Working Group. Br J Haematol 2003;121:749-57.  Back to cited text no. 5  [PUBMED]  
6.Cerase A, Tarantino A, Gozzetti A, Muccio CF, Gennari P, Monti L, et al. Intracranial involvement in plasmacytomas and multiple myeloma: A pictorial essay. Neuroradiology 2008;50:665-74.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]

Copyright 2010 - Indian Journal of Cancer


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