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European Journal of Radiology 61 (2007) 184–186
Quiz Alexandra Athanasiou a,b,∗ , Daniel Vanel a , Gorana Tomasic c , Francois Bidault a , Corinne Balleyguier a a
Department of Radiology, Institut de Canc´erologie, Gustave-Roussy, 94805 Villejuif, France b Department of Radiology, Institut Curie, 26 rue d’Ulm, 75005 Paris, France c Department of Pathology, Institut de Canc´ erologie, Gustave-Roussy, 94805 Villejuif, France
Received 18 August 2006; received in revised form 21 August 2006; accepted 24 August 2006
Abstract Primary breast amyloidosis is rare, usually manifestating as suspicious masses in mammography, with or without associated microcalcifications. The final diagnosis is based on fine needle aspiration cytology or biopsy yielding clumps of amorphous material surrounded by lymphocytes and giant cells that characterize this entity. In our case the only mammographic finding was that of clustered, suspicious microcalcifications without any associated mass. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Clustered suspicious breast microcalcifications; Breast amyloid
A 56-year-old woman was referred to our hospital for further investigation due to detection of suspicious, clustered microcalcifications during a routine screening mammography visit. Standard cranio-caudal and medio-lateral oblique projections of the left breast depicted two groups of irregular, pleiomorphic microcalcifications presenting a triangular orientation towards the nipple. These microcalcifications were not visible on the previous mammograms. Their recent development as well as their mammographic characteristics justified their being classified as a BIRADS 5 lesion with a high suspicion of malignancy. No associated abnormal density, architectural distortion or mass was seen on the mammogram. Ultrasonography was normal. The physical breast examination was unremarkable. The patient was referred to our Interventional Mammography Unit for a stereotactic core biopsy. Clinical history revealed type II non-insulin-dependent diabetes and arterial hypertension that were both controlled by medical treatment. No family history of breast malignancy was mentioned. The patient had been on hormonal replacement therapy for about 10 years, after a hysterectomy for uterine fibromas. Additional mammographic spot compression–magnification views were taken in our unit before the core biopsy. They clearly ∗
Corresponding author at: Department of Radiology, Institut Curie, 26 rue d’Ulm, 75005 Paris, France. Tel.: +33 1 44 32 42 00. E-mail address: [email protected] (A. Athanasiou). 0720-048X/$ – see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrad.2006.08.031
depicted two distinct groups of microcalcifications located in the outer and lower quadrants, respectively (Fig. 1). They consisted of irregular, pleiomorphic, amorphous, segmentally distributed calcifications (Fig. 2). A stereotactically guided core biopsy was performed with a vacuum-assisted biopsy system. Both target lesions were sampled. A clip was used to mark one of the biopsy sites as there were no residual microcalcifications. Immediate post-procedure analysis confirmed the presence of microcalcifications in specimens (Fig. 3). No complications were recorded during or after the biopsy. 1. Primary breast amyloidosis presenting solely as suspicious clustered microcalcifications At histological analysis, no sign of carcinoma was found. On the contrary, the histological diagnosis was breast amyloidosis. Amyloid deposits were clearly visible in the basal membrane and in the perilobular and periductal spaces. Numerous microcalcifications and giant cells were found between the ducts and the lobules in the stroma. No dysplastic or carcinomatous cells were found. The red Congo stain was positive (Fig. 4). Despite the benign histology, surgical excision of the clustered microcalcifications was performed because they were classified as BIRADS 5. Pre-operative wire-hook localisation was performed and specimen radiographs (Fig. 5) confirmed the
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Fig. 1. Spot compression magnification profile view of the left breast. Two groups of suspicious segmentally distributed, irregular and pleiomorphic microcalcifications are visualized.
Fig. 3. Radiographic view of the biopsy specimen confirming the presence of microcalcifications.
complete excision of microcalcifications. Histological analysis of the surgically excised material confirmed the initial diagnosis based on the biopsy sample. No associated carcinoma was found. Considering the nature of amyloid, further investigation was carried out. The patient’s haematological profile was normal and serum electrophoresis was unremarkable. Bence-Jones proteins were negative in the urine analysis. No skeletal abnormality was
Fig. 2. Spot compression magnification cranio-caudal view of the left breast confirms the suspicious appearance of these clustered microcalcifications. Furthermore, another group of microcalcifications is detected but they exhibit a monomorphous appearance and are classified as BIRADS 3.
Fig. 4. Nodular deposits of amyloid with multinucleated giant cells are present in fibrocollagenous stroma and around ducts.
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an associated anomaly is found at clinical examination. Other mammographic appearances already described are diffuse breast oedema and skin thickening mimicking inflammatory breast carcinoma [8], or isolated suspicious microcalcifications [7]. The coexistence of amyloidosis and breast carcinoma has also been described [8]. The histological explanation for the development of microcalcifications in cases of localized breast amyloidosis is that amyloid fibrils have an affinity for calcium [2]. As amyloid deposits are found around ducts and vessels, they exhibit an irregular, branching pattern on mammography, prompting the clinician to perform a stereotactically guided biopsy because this appearance is usually classified as BIRADS 4–5. Our findings are similar to those of a few cases that have already been published [2,7]. The presence of microcalcifications without any other associated mammographic or clinical findings is the rarest form of breast amyloidosis. One case of breast amyloidosis presenting as a breast mass in a patient with insulin-dependent diabetes mellitus has already been reported [9], however, in our case, the patient was suffering from non-insulin-dependent diabetes mellitus. No aetiology was found for the primary localized breast amyloid deposition in our case. 3. Conclusion Fig. 5. Radiographic view of the surgical specimen confirming the wide excision of suspicious microcalcifications. A better appraisal of the branched pattern and the irregular morphology is possible with this view.
depicted on the radiographs. The diagnosis was primary amyloid deposition of the breast. 2. Discussion Breast amyloidosis was first described by Fernandez and Hernandez [1]. Amyloidosis is typically divided into a systemic form and a localized form. Fifteen different types of amyloid protein structures have been defined to date; AL and AA types are the most common. Breast amyloidosis occurs mainly in elderly patients who already present a systemic form of the disease or other systemic disorders such as multiple myeloma or rhumatoid arthritis which may promote the onset of amyloidosis [2,3]. The histological definition of amyloidosis refers to the extracellular deposition of amorphous amyloid congophilic proteins associated with lymphocytes and giant cells (foreign-body-like reaction) [4]. This deposition occurs mainly in periductal and perivascular tissue as well as in lobules. Characteristically, the Congo red stain is positive under a polarizing microscope. Localized breast amyloidosis is rare [5]) and can give rise to various mammographic and clinical findings. Most cases reported in the literature describe irregular or spiculated masses with or without microcalcifications that can be solitary or even multiple and bilateral [2,6,7]. In the majority of cases,
Breast amyloidosis is rare and the primary and localized form is even rarer. Clinical and mammographic findings can mimic a malignancy, as in our case, with suspicious microcalcifications. Only histology can provide the definitive diagnosis. References [1] Fernandez BB, Hernandez FJ. Amyloid tumor of the breast. Arch Pathol 1973;95:102–5. [2] Gluck BS, Cabrera J, Strauss B, Ricca R, Brancaccio W, Tamsen A. Amyloid deposition of the breast. Am J Roentgenol 2000;175:1590. [3] Sabate JM, Clotet M, Gomez A, de la Heras P, Torrubia S, Salinas T. Radiologic evaluation of uncommon inflammatory and reactive breast disorders. Radiographics 2005;25:411–24. [4] Deolekar MV, Larsen J, Morris JA. Primary amyloid tumour of the breast: a case report. J Clin Pathol 2002;55:634–5. [5] Silverman JF, Dabbs DJ, Norris HT, Porries WJ, Legier J, Key S. Localized primary (AL) amyloid tumor fo the breast: cytologic, histologic, immunocytochemical and ultrastructural observations. Am J Surg Pathol 1986;10:539–45. [6] Hecht AH, Tan A, Shen JF. Case report: primary systemic amyloidosis presenting as breast masses, mammographically simulating carcinoma. Clin Radiol 1991;44:123–4. [7] Diaz-Bustamante T, Iribar M, Vilarrasa A, Benito A, Lopez-Rios F. Primary amyloidosis of the breast presenting solely as microcalcifications. Am J Roentgenol 2001;177:903–4. [8] Fu K, Basset LW. Mammographic findings of diffuse amyloidosis and carcinoma of the breast. Am J Roentgenol 2001;177:901–2. [9] Sahoo S, Reeves W, DeMay RM. Amyloid tumor: a clinical and cytomorphologic study. Diagn Cytopathol 2003;28(6):325–8.
Quiz Alexandra Athanasiou a,b,∗ , Daniel Vanel a , Gorana Tomasic c , Francois Bidault a , Corinne Balleyguier a a
Department of Radiology, Institut de Canc´erologie, Gustave-Roussy, 94805 Villejuif, France b Department of Radiology, Institut Curie, 26 rue d’Ulm, 75005 Paris, France c Department of Pathology, Institut de Canc´ erologie, Gustave-Roussy, 94805 Villejuif, France
Received 18 August 2006; received in revised form 21 August 2006; accepted 24 August 2006
Abstract Primary breast amyloidosis is rare, usually manifestating as suspicious masses in mammography, with or without associated microcalcifications. The final diagnosis is based on fine needle aspiration cytology or biopsy yielding clumps of amorphous material surrounded by lymphocytes and giant cells that characterize this entity. In our case the only mammographic finding was that of clustered, suspicious microcalcifications without any associated mass. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Clustered suspicious breast microcalcifications; Breast amyloid
A 56-year-old woman was referred to our hospital for further investigation due to detection of suspicious, clustered microcalcifications during a routine screening mammography visit. Standard cranio-caudal and medio-lateral oblique projections of the left breast depicted two groups of irregular, pleiomorphic microcalcifications presenting a triangular orientation towards the nipple. These microcalcifications were not visible on the previous mammograms. Their recent development as well as their mammographic characteristics justified their being classified as a BIRADS 5 lesion with a high suspicion of malignancy. No associated abnormal density, architectural distortion or mass was seen on the mammogram. Ultrasonography was normal. The physical breast examination was unremarkable. The patient was referred to our Interventional Mammography Unit for a stereotactic core biopsy. Clinical history revealed type II non-insulin-dependent diabetes and arterial hypertension that were both controlled by medical treatment. No family history of breast malignancy was mentioned. The patient had been on hormonal replacement therapy for about 10 years, after a hysterectomy for uterine fibromas. Additional mammographic spot compression–magnification views were taken in our unit before the core biopsy. They clearly ∗
Corresponding author at: Department of Radiology, Institut Curie, 26 rue d’Ulm, 75005 Paris, France. Tel.: +33 1 44 32 42 00. E-mail address: [email protected] (A. Athanasiou). 0720-048X/$ – see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrad.2006.08.031
depicted two distinct groups of microcalcifications located in the outer and lower quadrants, respectively (Fig. 1). They consisted of irregular, pleiomorphic, amorphous, segmentally distributed calcifications (Fig. 2). A stereotactically guided core biopsy was performed with a vacuum-assisted biopsy system. Both target lesions were sampled. A clip was used to mark one of the biopsy sites as there were no residual microcalcifications. Immediate post-procedure analysis confirmed the presence of microcalcifications in specimens (Fig. 3). No complications were recorded during or after the biopsy. 1. Primary breast amyloidosis presenting solely as suspicious clustered microcalcifications At histological analysis, no sign of carcinoma was found. On the contrary, the histological diagnosis was breast amyloidosis. Amyloid deposits were clearly visible in the basal membrane and in the perilobular and periductal spaces. Numerous microcalcifications and giant cells were found between the ducts and the lobules in the stroma. No dysplastic or carcinomatous cells were found. The red Congo stain was positive (Fig. 4). Despite the benign histology, surgical excision of the clustered microcalcifications was performed because they were classified as BIRADS 5. Pre-operative wire-hook localisation was performed and specimen radiographs (Fig. 5) confirmed the
A. Athanasiou et al. / European Journal of Radiology 61 (2007) 184–186
185
Fig. 1. Spot compression magnification profile view of the left breast. Two groups of suspicious segmentally distributed, irregular and pleiomorphic microcalcifications are visualized.
Fig. 3. Radiographic view of the biopsy specimen confirming the presence of microcalcifications.
complete excision of microcalcifications. Histological analysis of the surgically excised material confirmed the initial diagnosis based on the biopsy sample. No associated carcinoma was found. Considering the nature of amyloid, further investigation was carried out. The patient’s haematological profile was normal and serum electrophoresis was unremarkable. Bence-Jones proteins were negative in the urine analysis. No skeletal abnormality was
Fig. 2. Spot compression magnification cranio-caudal view of the left breast confirms the suspicious appearance of these clustered microcalcifications. Furthermore, another group of microcalcifications is detected but they exhibit a monomorphous appearance and are classified as BIRADS 3.
Fig. 4. Nodular deposits of amyloid with multinucleated giant cells are present in fibrocollagenous stroma and around ducts.
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an associated anomaly is found at clinical examination. Other mammographic appearances already described are diffuse breast oedema and skin thickening mimicking inflammatory breast carcinoma [8], or isolated suspicious microcalcifications [7]. The coexistence of amyloidosis and breast carcinoma has also been described [8]. The histological explanation for the development of microcalcifications in cases of localized breast amyloidosis is that amyloid fibrils have an affinity for calcium [2]. As amyloid deposits are found around ducts and vessels, they exhibit an irregular, branching pattern on mammography, prompting the clinician to perform a stereotactically guided biopsy because this appearance is usually classified as BIRADS 4–5. Our findings are similar to those of a few cases that have already been published [2,7]. The presence of microcalcifications without any other associated mammographic or clinical findings is the rarest form of breast amyloidosis. One case of breast amyloidosis presenting as a breast mass in a patient with insulin-dependent diabetes mellitus has already been reported [9], however, in our case, the patient was suffering from non-insulin-dependent diabetes mellitus. No aetiology was found for the primary localized breast amyloid deposition in our case. 3. Conclusion Fig. 5. Radiographic view of the surgical specimen confirming the wide excision of suspicious microcalcifications. A better appraisal of the branched pattern and the irregular morphology is possible with this view.
depicted on the radiographs. The diagnosis was primary amyloid deposition of the breast. 2. Discussion Breast amyloidosis was first described by Fernandez and Hernandez [1]. Amyloidosis is typically divided into a systemic form and a localized form. Fifteen different types of amyloid protein structures have been defined to date; AL and AA types are the most common. Breast amyloidosis occurs mainly in elderly patients who already present a systemic form of the disease or other systemic disorders such as multiple myeloma or rhumatoid arthritis which may promote the onset of amyloidosis [2,3]. The histological definition of amyloidosis refers to the extracellular deposition of amorphous amyloid congophilic proteins associated with lymphocytes and giant cells (foreign-body-like reaction) [4]. This deposition occurs mainly in periductal and perivascular tissue as well as in lobules. Characteristically, the Congo red stain is positive under a polarizing microscope. Localized breast amyloidosis is rare [5]) and can give rise to various mammographic and clinical findings. Most cases reported in the literature describe irregular or spiculated masses with or without microcalcifications that can be solitary or even multiple and bilateral [2,6,7]. In the majority of cases,
Breast amyloidosis is rare and the primary and localized form is even rarer. Clinical and mammographic findings can mimic a malignancy, as in our case, with suspicious microcalcifications. Only histology can provide the definitive diagnosis. References [1] Fernandez BB, Hernandez FJ. Amyloid tumor of the breast. Arch Pathol 1973;95:102–5. [2] Gluck BS, Cabrera J, Strauss B, Ricca R, Brancaccio W, Tamsen A. Amyloid deposition of the breast. Am J Roentgenol 2000;175:1590. [3] Sabate JM, Clotet M, Gomez A, de la Heras P, Torrubia S, Salinas T. Radiologic evaluation of uncommon inflammatory and reactive breast disorders. Radiographics 2005;25:411–24. [4] Deolekar MV, Larsen J, Morris JA. Primary amyloid tumour of the breast: a case report. J Clin Pathol 2002;55:634–5. [5] Silverman JF, Dabbs DJ, Norris HT, Porries WJ, Legier J, Key S. Localized primary (AL) amyloid tumor fo the breast: cytologic, histologic, immunocytochemical and ultrastructural observations. Am J Surg Pathol 1986;10:539–45. [6] Hecht AH, Tan A, Shen JF. Case report: primary systemic amyloidosis presenting as breast masses, mammographically simulating carcinoma. Clin Radiol 1991;44:123–4. [7] Diaz-Bustamante T, Iribar M, Vilarrasa A, Benito A, Lopez-Rios F. Primary amyloidosis of the breast presenting solely as microcalcifications. Am J Roentgenol 2001;177:903–4. [8] Fu K, Basset LW. Mammographic findings of diffuse amyloidosis and carcinoma of the breast. Am J Roentgenol 2001;177:901–2. [9] Sahoo S, Reeves W, DeMay RM. Amyloid tumor: a clinical and cytomorphologic study. Diagn Cytopathol 2003;28(6):325–8.