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Primary Amyloidosis of the Breast Presenting Solely as Microcalcifications

¹ Department of Radiology, Hospital Universitario, Carretera de Andalucía km 5, 4, 28041 Madrid, Spain.
² Department of Pathology, Hospital Universitario, Carretera de Andalucía km 5, 4, 28041 Madrid, Spain.

Received December 19, 2000; accepted after revision March 7, 2001.

 
Address correspondence to F. López-Ríos.

Introduction

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Primary amyloidosis of the breast is unusual. Patients usually seek medical attention because of the presence of a palpable mass. Hence, the name "amyloid tumor" was given to this entity [1]. We describe a case of primary amyloidosis of the breast presenting on a baseline mammogram as clustered microcalcifications not associated with a lump.

Case Report

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A 71-year-old asymptomatic menopausal woman presented to the radiology department for a screening mammogram. The patient's medical history was noncontributory, and her physical examination was within normal limits. The mammogram showed that both breasts were almost entirely composed of fat. A cluster of fine linear and branching microcalcifications was detected in the upper inner quadrant of the right breast at the 2-o'clock position in the posterior third of the breast (Figs. 1A and 1B). Other scattered benign calcifications were observed bilaterally. No masses, architectural distortions, or asymmetric densities were observed. The left breast was unremarkable. Previous mammograms were not available for comparison. The microcalcifications were considered as suspicious for malignancy (Breast Imaging Reporting and Data System [BI-RADS] category 4: suspicious abnormality [2]); thus, a biopsy was recommended.

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A. —71-year-old woman was diagnosed with microcalcifications after presenting for screening mammogram. Right craniocaudal mammogram shows cluster of microcalcifications.

View larger version (158K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B. —71-year-old woman was diagnosed with microcalcifications after presenting for screening mammogram. Spot magnification mammogram shows cluster of fine linear and branching microcalcifications.

Before the patient underwent surgical biopsy, a fenestrated compression paddle and hookwire system was administered for localization. An irregular piece of breast tissue measuring 4 x 3 x 2 cm was excised. The specimen radiograph revealed microcalcifications. Gross examination of the specimen was unremarkable and did not disclose any masses. Histologically, deposits of amorphous, eosinophilic material were seen around the ducts and lobules. Numerous calcifications were found within the deposits. The homogeneous material was positive for Congo red stain and was dichroic under polarized light (Fig. 1C). Paraffin immunohistochemistry showed the absence of amyloid A. The histologic findings showed amyloid deposition in the breast. The systemic workup, including serum and urine electrophoresis, was negative for amyloidosis. Twenty-four months after surgery the patient remains well and without recurrence of the breast lesion. No clinical or laboratory evidence of systemic amyloidosis has been found subsequent to the operation.

View larger version (172K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C. —71-year-old woman was diagnosed with microcalcifications after presenting for screening mammogram. Photomicrograph of histopathologic specimen shows deposits of amorphous material seen around ducts and lobules positive for Congo red stain. Note calcification in lower right corner. (x100)

Discussion

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Amyloid deposition in the breast encompasses two forms: breast involvement in systemic disease as reported in primary amyloidosis and in association with multiple myeloma, plasmacytosis, and rheumatoid arthritis; and in the localized primary form, even rarer, as in the case presented here. [1].

Primary amyloidosis of the breast occurs predominantly in elderly women. The few reported cases have presented as palpable masses. Unfortunately, none of the cases have been included in the radiology literature, and the mammographic results are not discussed in detail [1, 3,4,5,6]. Moreover, a recent radiologic review of unusual breast lesions does not illustrate amyloidosis [7]. The mammographic findings of amyloid tumor of the breast are reported as irregular masses or as an irregular high-density area with spiculations radiating from the area. In addition, irregularly shaped calcifications may be associated with the mass [1, 3,4,5,6]. To our knowledge, this case is unique because of the absence of a mass. In a recent article, Gluck et al. [8] describe a patient with multiple myeloma in whom amyloidosis of the breast presented as a nonpalpable abnormality on mammography. Therefore, this report further challenges the dogma of breast amyloidosis as an entity associated with a lump. Regardless of the presence of a lump or microcalcificactions, breast amyloidosis may be confused clinically and mammographically with carcinoma. In this setting, surgical excision is mandatory to exclude malignancy [1]. Pathologically, amyloidosis has typical features and may be confirmed by a Congo red stain and immunohistochemistry [1].

In summary, we have reported a unique presentation for primary amyloidosis of the breast. The potential occurrence of amyloidosis should be recognized in the differential diagnosis of clustered microcalcifications, both from the radiologic and pathologic point of view.

Acknowledgments
 
We thank the Departments of Gynecology and Internal Medicine for clinical and follow-up information. We are also grateful to Gustavo Pérez Espejo for his pathologic diagnosis.

References

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1. Luo JH, Rotterdam H. Primary amyloid tumor of the breast: a case report and review of the literature. Mod Pathol 1997;10:735 -738[Medline]
2. American College of Radiology. Breast Imaging reporting and data system (BI-RADS), 3rd ed. Reston, VA: American College of Radiology, 1998
3. Yokoo H, Nakazato Y. Primary localized amyloid tumor of the breast with osseus metaplasia. Pathol Int 1998;48:545 -548[Medline]
4. Lynch LA, Moriarty AT. Localized primary amyloid tumor associated with osseus metaplasia presenting as bilateral breast masses: cytologic and radiologic features. Diagn Cytopathol 1993;9:570 -575[Medline]
5. Gupta RK, Dowle CS. Cytodiagnosis of amyloid deposition within the breast. Cytopathology 1996;7:136 -140[Medline]
6. Hecht AH, Tan A, Shen JF. Case report: primary systemic amyloidosis presenting as breast masses, mammographically simulating carcinoma. Clin Radiol 1991;44:123 -124[Medline]
7. Feder JM, de Paredes ES, Hogge JP, Wilken JJ. Unusual breast lesions: radiologic-pathologic correlation. RadioGraphics 1999;19:S11 -S26
8. Gluck BS, Cabrera J, Strauss B, Ricca R, Brancaccio W, Tamsen A. Amyloid deposition of the breast. AJR 2000;175:1590[Free Full Text]

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