IntroductionPlasmacytomas are discrete, solitary masses of mono-

clonal plasma cells contained within the bone or soft tis-sues. The relationship to multiple myeloma (MM) remains unclear; however, plasmacytomas lie in a spectrum between monoclonal gammopathy of undetermined significance at one end and MM on the other. The diagnosis of an extra-medullary plasmacytoma is established when a plasma cell tumor in an extraosseous site occurs in the absence of MM on bone marrow examination, radiography, and studies of blood and urine.1 Although the etiology is unknown, viruses and inhaled irritants have been implicated as triggers for these tumors, because 80% occur in the upper aerodiges-tive tract.2,3 Plasmacytomas have been identified in the central nervous system, orbit, aerodigestive tract, stomach, intestines, liver, spleen, pancreas, lung, skin, testes, thyroid gland, parotid gland, mediastinum, as well as in the breast. In this regard, plasmacytoma is included in the differential diagnosis for unilateral or bilateral mammary masses. The first reported case of plasmacytoma in the breast in the

United States was in 1934 in a 49-year-old woman who later developed similar lesions in the larynx, the subcutaneous tissue of the chest wall, and the nostril.4 Our report is that of a patient with a history of MM and extensive plasma-cytic tumors that were responsive to chemotherapy until the development of plasmacytomas in the breast with rarely, if ever, reported axillary lymph node involvement.

Illustrative CaseThe patient is a 78-year-old white man with a history of

MM and plasmacytic tumors who presented with a growing mass in the left breast over a period of 3 months. His past history was significant in that he was exposed to chemi-cal irritants for several decades by pursuing photography as a hobby. He was diagnosed with immunoglobulin Gλ hyposecretory MM in January 2000. He was treated with 5 cycles of cyclophosphamide and dexamethasone and was considered to be in remission in September 2000. Biannual bone marrow biopsy results had been negative for myeloma since August 2000.

Between 2000 and 2004, the patient developed several plasmacytomas that fluctuated in size during trials of radia-tion and chemotherapy. They appeared in the left scapular region, right costal margin, scalp near the auricles bilater-ally, maxillary sinus, orbits bilaterally, right nipple, right supraclavicular lymph nodes, and on the back in 3 discrete areas. Beginning January 2004, the patient received continu-ous treatment with a potent thalidomide derivative CC-5013. During that treatment, the patient was treated with granu-locyte colony-stimulating factor when his white blood cell count reached a nadir of 2600/μL. All his plasmacytomas

Submitted: Jan 9, 2006; Revised: Feb 14, 2006; Accepted: Feb 28, 2006

1Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY2St. Vincent’s Comprehensive Cancer Center, New York Medical College, New York3New York University Clinical Cancer Center, New York University College of Medicine, New York

Address for correspondence: Deborah Axelrod, MD, New York University Clinical Cancer Center, 160 East 34th St, New York, NY 10016Fax: 212-731-6051; e-mail: axeldoc@comcast.net

Plasmacytoma in the Breast with Axillary Lymph Node Involvement: A Case Report

Plasmacytic tumors in the breast are extremely rare neoplasms that can mimic mammary adenocarcima. A literature review revealed approximately 43 reports worldwide of plasmacytic tumors in the breast since 1928, the majority with a synchronous diagnosis of multiple myeloma. We discuss management of a 78-year-old man with plasmacytoma in the breast who underwent a left total mastectomy and axillary lymph node dis-section. This report characterizes the histologic and immunochemical characteristics of plasmacytoma in the breast and reviews the consensus on treatment. This could be the first recorded account of plasmacytomas in the breast with axillary lymph node involvement and extracapsular extension of tumor.

Clinical Breast Cancer, Vol. 7, No. 1, 81-84, 2006Key words: Extracapsular extension, Multiple myeloma, Retroareolar mass

report

Abstract

case

Lesley Taylor,1 Mohamad Aziz,2 Paula Klein,2 Amitabha Mazumder,2 Sundar Jagannath,2 Deborah Axelrod3

Clinical Breast Cancer April 2006 • 81

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82 • Clinical Breast Cancer April 2006

Plasmacytoma of the Breast

decreased in size except for a mass in his left breast.The breast mass was rapidly enlarging, nonpainful, firm,

and there was no nipple discharge. Fine needle aspiration results were positive for a plasmacytic tumor (Figure 1). A bone marrow biopsy was performed, and results showed plasma cells accounting for 6% of the total cellularity. Bence Jones protein was not detected in the urine. Serum calcium was 9.1 mg/dL, albumin was 3.4 g/dL, lactate dehydrogenase was normal at 444 IU/L, and serum M-protein measured 0.59 g/dL. He had a white blood cell count of 4300/μL, hemo-globin 11.4 g/dL, hematocrit 35%, platelets 136,000/μL, abso-lute neutrophil count 2900/μL, and lymphocyte count 800/μL. The assessment was soft tissue plasmacytoma in the left breast refractory to treatment, and a left total mastectomy was subsequently planned.

At surgery, the left nipple was found to be erythematous and retracted. A smooth, movable, nontender, retroareolar mass measuring 6 cm × 1.5 cm was appreciated (Figure 2). No palpable or suspicious axillary lymph nodes were noted; however, an axillary lymph node dissection was performed subsequent to the intraoperative discovery of firm, suspi-cious lymph nodes.

The left breast specimen contained plasmacytomas at 2 sites. They were grossly circumscribed and fleshy in appear-ance. One mass measured 5 cm × 4.8 cm extending from the nipple to the deep fibromembranous fascia. The second mass was located 3 cm laterally to the other and measured 1.5 cm × 1 cm. Both tumors were identical microscopically with diffuse sheets of plasma cells, a high mitotic count, and abundant karyorrhexis. These findings were consistent with extramedullary plasmacytomas (Figure 3). Dermal skin was involved with plasmacytoma, but no involvement of epider-mis was noted.

Four of 6 level 1 left axillary lymph nodes were replaced by plasmacytoma with extension through the capsule to the perinodal fat. The largest lymph node measured 2.5 cm. All 5 nodes sampled near the thoracodorsal region were negative. In total, 4 of 11 axillary nodes were positive for plasmacy-toma, and all were negative for amyloid deposits after congo red staining. Both masses contained monoclonal plasma cell populations with cytoplasmic immunoglobulin Gλ restriction. The breast tumors were negative for cytokera-tin, S-100, actin, and B-cell antigen.

Four months after surgery, the patient developed several plasmacytomas that were clustered like grapes along the mastectomy scar line. A plasmacytoma also developed over the right chest wall near his Port-a-Cath® site. The patient survived 11 months after his initial presentation of plasma-cytoma in the breast. He died from complications of pneumo-nia 7 months after surgery.

DiscussionOur review of the literature yielded 43 reports of plas-

macytoma in the breast described worldwide, and only 9 patients were men (Table 1).4-45 The patient in this report

Fine Needle Aspiration Positive for Plasmacytic Tumor Figure 1

A fine needle aspiration revealing abundant dysplastic plasma cells and plasmablasts with large eccentric nuclei, multinucleation, perinuclear hof corresponding to the prominent Golgi region, and abnormal mitosis. High power magnification.

A Smooth, Movable, Nontender, Retroareolar Mass Figure 2

A clinical image of a left breast plasmacytoma with a firm central mass appearing fixed to the overlying skin and associated with raised erythematous patches. The patient has a Port-a-Cath® located in the right chest wall.

Histology Showing Infiltrating Dysplastic Plasma Cells and Plasmablasts

Figure 3

Hematoxylin and eosin staining shows sheets and aggregates of infiltrating dysplastic plasma cells and plasmablasts. The cells have an increased nuclear-to-cytoplasmic ratio, distinct nucleoli, dispersed nuclear chromatin, and increased abnormal mitotic activity. High power magnification.

Taylor CsRpt1.indd 2 5/4/06 9:22:42 AM

Lesley Taylor et al

was unusual in several aspects: he had a history of MM and classic focal lesions in the aerodigestive tract consistent with extramedullary plasmacytoma; he is the oldest man recorded with solitary mammary involvement at the time of surgery; he had 2 lesions in the breast; the plasma cells expressed λ paraprotein; the breast tumors did not respond to chemotherapy; and axillary lymph nodes were involved with extracapsular extension.

When evaluating a patient with a plasmacytoma in the breast, it is essential to distinguish a localized process or one synchronous with a systemic disease. Approximately 75% of the reported cases of plasmacytoma in the breast have occurred in women with bilateral or unilateral mammary involvement and concurrent MM.45 We found few reports of patients with truly solitary plasmacytic tumors in the breast without evidence of concurrent MM or other plasma-cytic tumors.5,6,8,9,12-14,16-19 Four patients who underwent surgical removal of solitary plasmacytomas in the breast remained disease free for > 1 year.6,8,17,18

Diagnostic procedures for the wide spectrum of plasma-cytic tumors in the breast follow the same protocol as for any suspicious breast mass. The differential diagnosis of a solitary breast mass or a mass presenting within the clinical context of plasma cell neoplasms includes primary epithelial neoplasms of the breast, plasma cell mastitis, non-Hodgkin’s lymphoma with plasmacytic features, epitheliod malignant melanoma, and pseudolymphoma.17

A plasmacytoma appears on a mammography as a well-defined mass with some irregular borders, athough smooth contours are also seen. Ultrasound can show a heteroge-neous, hypoechoic, or nonechoic density, appearing benign. Fine needle aspiration can yield plasmacytoid cells in differ-ent stages of maturation. Immunohistochemical staining of a fine needle aspirate has been reported to be a rapid and accurate diagnostic technique, including staining with epithelial membrane antigen, cytoembryonic antigen, leukocyte common antigen (CD45), melanoma marker, and vimentin.19

Treatment options for extramedullary plasmacytoma include radiation therapy, chemotherapy, or resection. Studies have shown radiation therapy to be as effective as surgical resection in resectable nonupper airway lesions.46 In approximately 30%-40% of soft tissue plasmacytomas, the first draining nodal basin might be involved at the time of presentation or at relapse.47 Thus, a complete resec-tion including lymph node dissection may be considered.48

Recommendations, however, are based on evidence from well-conducted case studies, and no randomized trials have been conducted to date.49

ConclusionWe report on a case of plasmacytoma in the breast with

axillary lymph node involvement in a 78-year-old man with a history of MM and other plasmacytic tumors. Unlike the patient’s previous soft tissue plasmacytomas, the lesion in the breast was refractory to systemic treatment and increased in size. A left total mastectomy and axillary dissection was performed in accordance with the general consensus in the literature, and there were 2 plasmacytic tumors in his breast and 4 positive lymph nodes. Our findings suggest that plas-

Literature Review of the Clinical Presentation of Plasmacytomas in the Breast4-45

Table 1

Patients are women unless otherwise indicated.Abbreviation: Ig = immunoglobulin

Plasmacytomas in the Breast Without Evidence of Multiple Myeloma

• Cutler, 19344

• Innes and Newall, 19615

• Proctor et al, 19756

• Bloomberg et al, 19807

• Merino, 19848

• Kirshenbaum and Rhone, 19859

• Desablens et al, 198610

• Kujat and Scherer, 1988 (metastatic solitary bone plasmacytoma)11

• Gorczyca et al, 199212

• Alhan et al, 199513

• Kumar et al, 1997 (male aged 30 years)14

• Picardi et al, 1998 (IgG-λ; metastatic osseous plasmacytoma)15

• Cangiarella et al, 200016

• De Chiara et al, 200117

• van Nieuwkoop et al, 200118

• Vera-Alvarez, 2003 (male aged 73 years)19

• Kaviani et al, 2004 (metastatic extramedullary plasmactyoma)20

Plasmacytomas in the Breast as the Clinical Presentation of Multiple Myeloma

• Rosenberg et al, 196321

• Maeda et al, 1973 (IgA)22

• Bassett and Weiss, 1979 (IgA-κ)23

• Ben-Yehuda et al, 1989 (2 cases, IgG-λ, IgG-κ)24

• Carere et al, 1993 (IgG-κ)25

• Vetto et al, 2004 (κ light chains; male aged 79 years)26

Plasmacytomas in the Breast with a Known Diagnosis of Multiple Myeloma (Including Recurrent or Late-Stage Myeloma)

• Vasiliu and Popa, 192827

• Craft, 196728

• Mangalik and Gupta, 197429

• Wiltshaw, 1976 (IgA-κ)3

• Phelip et al, 1977 (IgA-κ; male aged 66 years)30

• Mottot et al, 1978 (IgA-λ; male aged 69 years)31

• Ross et al et al, 1987 (κ light chains)32

• Flageat et al, 198933

• Grande, 199034

• Lombardi et al, 1992 (λ light chains; male aged 71 years)35

• Moulopoulos et al, 199336

• Collins et al, 1994 (IgG-κ)37

• Domanski, 1995 (IgM; male aged 82 years)38

• Sanal et al, 1996 (IgA-λ; male aged 82 years)39

• Quilichini et al, 1997 (2 cases: IgG-λ and λ light chains)40

• Ampil, 1998 (IgA-κ)41

• Ataga et al, 1999 (IgA-κ)42

• Lamy et al, 200043

• Cangiarella et al, 2000 (λ light chains; male aged 70 years)16

• Kanno et al, 200144

• Brem et al, 2002 (IgA)45

Clinical Breast Cancer April 2006 • 83

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Plasmacytoma of the Breast

macytic tumors in the breast can mimic mammary adenocar-cinoma, especially when axillary lymph nodes are involved.

AcknowledgementsWe thank Mathew Harris, MD, and Jerry Waisman, MD, from

the New York University Clinical Cancer Center in Manhattan, NY, for reviewing the article and for their helpful discussions. We are also grateful to Stephanie Stoss and Kathleen Curran, RN, from St. Vincent’s Comprehensive Cancer Center in Manhattan, NY, for their contributions to this work.

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