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Unusual Presentations of Malignancy

Case 2. Breast Plasmacytoma in a Patient with Human Immunodeficiency Virus

James A. Haley Veterans Affairs Hospital, Tampa, FL

A 44-year-old man noticed a mass on his right breast approximately the size of a pea 10 weeks before seeking medical evaluation. When the mass enlarged to the size of a golf ball, he went to his primary physician. He had also noted difficulty breathing and reported a few episodes of hemoptysis. He denied weight loss, fever, and night sweats. Past medical history was significant for 12 years of human immunodeficiency virus (HIV)–positive disease with a CD4 lymphocyte count of 89 and viral load of 411,000 copies.

Examination showed a firm 4 x 3-cm palpable right breast mass; the remainder of his physical examination was unremarkable. Chest x-ray revealed a 4 x 5-cm right suprahilar mass, which a thorax computed tomography (CT) scan confirmed. The scan showed that the mass was causing extrinsic compression of the right upper lobe pulmonary artery. The CT scan also demonstrated a right breast mass causing indentation of the pectoralis muscle. A mammogram documented a 3.5 x 2.5-cm retroareolar eccentric mass suggestive of malignancy. An excisional biopsy of the breast mass and an open lung biopsy using the Chamberlain procedure (anterior mediastinoscopy) were performed. The breast biopsy specimen was consistent with a plasmacytoma (Fig 1), whereas the suprahilar mass revealed an anthracotic lymph node–negative form malignancy. Subsequent evaluation for multiple myeloma showed a monoclonal spike of 2.1 g/dL identified as immunoglobulin A lambda. A skeletal survey was negative for lytic lesions. Bone marrow biopsy showed hypercellular marrow with extensive (approximately 90%) replacement with plasma cells. Results of the histologic examination of the breast biopsy specimen were similar to the bone marrow pathologic findings. Subsequently, additional subcutaneous firm nodules developed on the patient’s upper torso and axilla. A diagnosis of multiple myeloma with extramedullary plasmacytoma was made, and treatment with melphalan and prednisone was initiated.

View larger version (156K): [in this window] [in a new window]   Fig 1.

The prevalence of multiple myeloma is higher in patients older than 60 years of age. Patients with HIV and plasma cell dyscrasia are, in general, younger. Several theories have been postulated to explain the role of HIV in the pathogenesis of plasma cell malignancy. Konrad et al³ suggested that a causal relation between antigenic stimulation and myeloma is based on the fact that in HIV-infected patients with myeloma, the paraprotein is specifically directed against the p24 antigen of HIV.

Another possibility is that infection with the Epstein-Barr virus causes clonal expansion of plasma cells,⁴ analogous to the proposed pathogenesis of HIV-associated non-Hodgkin’s lymphoma. Although Goeder et al⁵ estimated a 4.5-fold increased risk of multiple myeloma in HIV patients, plasma cell malignancy is not usually entertained in the differential diagnosis of HIV-associated malignancies and therefore can be missed. More cases need to be documented to determine the incidence and clinical course of this entity in the HIV-infected population.

REFERENCES

1. Lallemand F, Fritsch L, Cywiner-Golenzer C, Rozenbaum W: Multiple myeloma in an HIV-positive man presenting with primary cutaneous plasmacytomas and spinal cord compression. J Am Acad Dermatol 39: 506-508, 1998[Medline]

2. Nosari AM, Landonio G, Cantoni S, et al: Multiple myeloma associated to HIV infection: Report of two patients. Eur J Haematol 56: 98-99, 1996[Medline]

3. Konrad RJ, Kricka LJ, Goodman DB, et al: Myeloma-associated paraprotein directed against the HIV-1 p24 antigen in an HIV-1-seropositive patient. N Engl J Med 328: 1817-1819, 1993[Free Full Text]

4. Voelkerding KV, Sanders LM, Kim HC, et al: Plasma cell malignancy in the acquired immunodeficiency syndrome: Associated with Epstein-Barr virus. Am J Clin Pathol 93: 222-228, 1989

5. Goeder T, Cote TR, Virgo P, et al: Spectrum of AIDS-associated malignancy disorders. Lancet 351: 1833-1839, 1998[Medline]

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Miranda, E. G. Articles by Doll, D. C. Search for Related Content PubMed PubMed Citation Articles by Miranda, E. G. Articles by Doll, D. C. Social Bookmarking                            What's this?