Mammary Manifestations of Some Systemic Disorders

Rasha Mohamed Kamal, Soha Talaat Hamed

1Professor of Radiology, Women Imaging Unit, Radiology Department, Kasr el Aini, Cairo University Hospitals., Consultant and Assisstant Project manager of the Egyptian National Screening Program, “Women healthcare Outreach Program,” Member of the Global Initiative for Cancer Awareness, Member in the Initiative to Improve Cancer Care in the Arab World. 2Assisstant Professor of Radiology, Woman Imaging Unit, Radiology, Department, Kasr el Aini, Cairo University Hospitals, Consultant in the Egyptian National Screening Program “Women healthcare Outreach Program.”

Abstract Introduction

A variety of benign and malignant pathologies affect A variety of benign and malignant pathologies may the breast. These lesions may arise from the breast or affect the female breast. These lesions may originate from anatomically related tissues. Systemic disorders may from the breast tissue itself or from anatomically related infrequently affect the breast. Some of these lesions have tissues. Cancer is without question the most important characteristic findings while others may of these pathologic processes.1 mimic benign or malignant breast pathologies. These lesions pose a diagnostic challenge to clinicians and radiologists alike. When faced with suspicious clinical or mammography In this review article, we will discuss the imaging features findings, patients may be anxious about the possibility of some mammary lesions that are relevant to the clinical of breast cancer. Because most of these lesions are and pathologic presentations of some systemic disorders benign, clinicians attempt to make a rapid and accurate Awareness of the full clinical history and the characteristic diagnosis to definitely rule out carcinoma. Imaging plays imaging findings are the first step towards making a correct a key role in diagnostic strategies.2 diagnosis. Diagnosis is usually confirmed after revision of pathology specimens. Unusual lesions of the breast can pose a diagnostic challenge. These unusual lesions include systemic diseases that infrequently involve the breast, but mammography findings may be striking.3

In this review article, we will discuss the imaging features of some mammary lesions that are relevant to the clinical and pathologic presentations of some systemic disorders. We believe it is important for clinicians and radiologists to be familiar with the

Corresponding Author: Rasha M K Fouad, 52, Tayaran Street (Mahmoud Shaltoot), Nasr City, Cairo, Egypt. Telephone: 00202- 24013478, Cell phone: 002- 0127457992, 002-0113433792, Fax: 00202- 24013478, e-mail: [email protected] 237 Rasha Mohamed Kamal features of these lesions particularly that some have across all age groups, but except for acute leukemia it characteristic appearances, while others may mimic mostly occurs in the middle and older age groups.4 cancer or benign breast pathology. Illustrative cases, for the different pathologic entities were performed in the Breast lymphoma, either as a manifestation of primary ‘Women Imaging Unit’ of Kasr Al Aini, Cairo University extra-nodal disease or as secondary involvement is a rare Hospital and will be demonstrated in this article. malignancy and its diagnosis, prognosis, and treatment have not been clearly defined.7,8,9,10 Relatively small groups of patients are reported in the literature. The Neoplastic diseases of the reported incidence of primary breast lymphoma ranges haematopoeitic and lymphoid from 0.04% to 0.5% of all breast malignancies7,6 and tissue that of secondary breast lymphoma is 0.07%.9,10

Hematologic malignancies (HM) include a broad Primary breast lymphomas in general have a non spectrum of malignancies affecting any component of specific appearance and lack primary and secondary the hempoetic system; blood, bone marrow, and lymph mammography changes associated with malignancy.11 nodes. As the three are intimately connected through Mammography described pictures of primary lymphomas the immune system, a disease affecting one of the are relatively circumscribed masses, a solitary indistinctly three will often affect the others as well. Hematologic marginated non calcified mass, or diffusely increased malignancies may derive from either one of the two breast density.12,13 On U/S, lymphomatous lesions major blood cell lineages: myeloid and lymphoid cell appear as rounded slightly lobulated and homogenous lines. Lymphomas, lymphocytic leukemias, and myeloma masses with relatively smooth margins that do not are from the lymphoid line, while acute and chronic contain tumor calcifications and are not surrounded by myelogenous leukemia, myelodysplastic syndromes parenchymal distortion seen in carcinomas of the breast. and myeloproliferative diseases are myeloid in origin.4,5 On examination of pathology specimens, sheets of cells Within these categories, lymphomas are the most with large convoluted and prominent nucleoli with common. Even though rare in the general population scanty cytoplasm are characteristic.11 (Fig. 1). (1-4 per 100,000 per year),6 HM strike individuals

A b

Figure (1): 70 year-old female presenting by hard A- Right cranio-caudal mammogram shows a dense well circumscribed mass with lobulated outline B- US examination revealed rounded lobulated masses with relatively smooth margins that do not contain tumor calcifications and are not surrounded by parenchymal distortion. Biopsy revealed primary Hodgkin lymphoma

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Secondary lymphoma of the breast, a manifestation ductal cancer at mammography. The borders of the mass of lymphoma elsewhere in the body, is more common may be indistinct due to lymphomatous infiltration into than primary lymphoma, although both are rare. surrounding glandular tissue (Fig. 2 &3). Pathological lymphadenopathy is the most common manifestation of secondary lymphoma involving the Breast metastases in cases of leukemia are very rare breast, seen on mammograms as large, round lymph and occur primarily in patients with acute myeloid nodes in the axilla with loss of the fatty hila. Secondary leukemia The mammography findings in cases of breast lymphoma is usually caused by non-Hodgkin leukemia in the can be normal, diffusely coarse lymphomatous infiltration of breast tissue.14 It is a rare breast parenchyma, irregular shaped masses with ill cause of an ill-defined mass that resembles invasive defined margins, or well-defined bordered lesions with a

A B C

Figure (2): 48 year-old female presenting by palpable breast mass A- MLO view of right breast showing a rounded partially obscured mass with indistinct posterior border (arrow) B- On US the mass is an enlarged intramammary node as evidenced by the eccentric fatty echogenic hilum C- US of the right axilla shows two similar pathological lymphnodes with infiltrated hila.. Biopsy revealed non Hodgkin lymphoma

A B C Figure (3): A well known case of lymphoma that developed bilateral axillary masses and extensive inflammatory breast changes. A. Bilateral MLO views showing diffuse edema pattern of both breasts with increased breast densities, coarse trabeculae and overlying skin thickening. Bilateral pathological axillary lymphadenopathy. B. Ultrasound examination of both breasts showed diffuse inflammatory changes with interstitial edema lines seen delineating the echogenic edematous fat lobules. C. The pathological axillary lymphnodes appeared as large hypo echoic mass lesions with indistinct fatty hila.

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A B

Figure(4): 50 year-old female, known case of hairy cell leukemia, under chemo and radiotherapy presenting by UOQ breast mass A- Panoramic US view of the right axilla & UOQ shows multiple variable sized hypo-echoic mass lesions and pathological enlarged axillary lymphnodes B- Power Doppler shows high vascularity in lymph nodes.

A B

Figure 5: 35-year-old female presenting by markedly swollen breasts. On mammography examination (A) both breasts showed a diffuse edema pattern. Complementary ultrasound examination (B) showed underlying well defined mass lesions. Examination of biopsy specimens revealed extra medullary plasma cytoma. benign appearance. On ultrasound, variable pictures are during the evolution of a multiple myeloma. They also described including hypo echoic nodules, lobulated usually present by well defined intra mammary mass hypoechoiec mass lesions simulating fibroadenomas or ill lesions (Fig 5). defined lesions. Pathological axillary lymphadenopathy are also reported15,16 (fig. 4). Metastatic tumors to the Extramedullary myeloma (plasmacytoma) is a breast malignant tumor composed entirely of plasma cells in the absence of bone involvement. These tumors can The dilemma of differentiating primary breast cancer occur anywhere in the soft tissue, especially in the from metastatic disease in the breast may prove difficult upper respiratory tract and oral cavity. Isolated cases are especially in patients with undiagnosed primaries. reported in the breast, mostly as a secondary involvement Therapy is quite different, and must be based upon an

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Figure (6): 63 year- old female with history of malignant melanoma of the left middle terminal phalanx, presenting by palpable breast masses, edema and enlarged axillary nodes. A- Craniocaudal view of the left breast shows diffuse edema pattern of the breast with two adjacent breast masses seen in the UOQ (arrows). B- Gray-scale US shows irregular hypoechoic subcutaneous masses C- US of left axilla shows enlarged axillary nodes with central liquefaction (patient under chemotherapy) D- Power Doppler shows intense peripheral vascularity.

accurate diagnosis. The therapy in these cases should on mammography and ultrasound. However, when be aimed at the underlying malignancy and surgical the metastatic lesion is diffuse, the appearance is management of breast lesions should be as conservative indistinguishable from that of inflammatory breast as possible.17 In general, breast metastases are uncommon carcinoma. The typical mammography appearance is presenting about 0.5-2.0% of all breast malignancies.17 round to lobulated nodules and ultrasound appearances In the female patient, the most common primary site vary from hypoechoic, heterogeneous to hyperechoic is the contra lateral breast; but it is usually difficult to masses. This appearance can mimic both benign and differentiate from a secondary primary lesion. Other malignant lesions21 (Fig. 6 & 7). sites include melanoma, lung and ovary.18 Occasionally, breast metastasis is the first manifestation of a previously occult primary lesion located elsewhere in the body.19 Autoimmune disorders Biopsy is the useful way to confirm the diagnosis and is often confirmatory.20 Metastatic tumors to the Autoimmune disorders are diseases caused by the body breast appear as relatively small, superficially located, producing an inappropriate immune response against its poorly defined, irregular nodules without calcification own tissues. Sometimes the immune system will cease to

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Figure (7): 50 year-old female with squamous cell carcinoma of the left forearm presenting with bilateral breast mass lesions. Bilateral mammography (A) shows obscured right axillary tail mass lesion. Complementary Ultraosund examination revealed intra mammary superficially located vascular lesions (B) associated with enlarged pathological axillary lymphnodes (C).

Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 242 Mammary Manifestations of Some Systemic Disorders recognize one or more of the body’s normal constituents muscle. Diagnosis is usually based on clinical symptoms as “self” and will create autoantibodies – antibodies that (weakness of proximal muscle groups) and elevated attack its own cells, tissues, and/or organs. This causes levels of serum muscle enzymes (creatine phosphokinase inflammation and damage and it leads to autoimmune and aldolase), and findings at muscle biopsy.3 In the disorders. Autoimmune disorders fall into two general types: clinical entity ‘Amyopathic dermatomyositis’ there those that damage many organs (systemic autoimmune is absence of clinically evident muscle disease.24 diseases) and those where only a single organ or tissue is However skin lesions are the same in both classic and directly damaged by the autoimmune process (localized). amyopathic varieties. The classical picture of mammary However, the distinctions become blurred as the effect dermatomyositis is extensive bizarre subcutaneous of localized autoimmune disorders frequently extends dystrophic calcification. Typically skin biopsies reveal beyond the targeted tissues, indirectly affecting other body an interface dermatitis that is difficult to differentiate organs and systems. Some of the most common types of from lupus erythematosis. Vacuolar changes of columnar autoimmune disorders include: Systemic autoimmune epithelium and lymphocytic inflammatory infiltrates diseases including diseases as rheumatoid arthritis, at the dermal epidermal junction can occur. Muscle systemic lupus, scleroderma, polymyalgia rheumatica while biopsies also reveal inflammatory cellular infiltrates localized forms include type 1 diabetes mellitus, multiple intermingled in areas of muscle fiber degeneration and sclerosis and others.22,23 regeneration (Fig. 8).

Dermatomyositis is a rare multisystem autoimmune Systemic lupus erythematosus, or SLE, is a multisystem collagen vascular disorder of adults and children of autoimmune disorder without a known cure. Lupus unknown etiology that primarily affects skin and skeletal is a rare chronic inflammatory reaction of the

Figure (8): MLO mammogram of both breasts showing bizarre shaped subcutaneous calcification rising to the axillae in a case of amyopathic dermatomyositis.

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A

C

Figure (9): Well known case of Systemic lupus erythematosis who developed lupus mastitis. Ultrasound examination of the breast showed a diffuse inflammatory reaction of both breasts with edematous echogenic fat and moderate interstitial edema lines (A). Multiple 2-3 cm well defined, completely avascular mass lesions are identified (B). A huge abscess cavity was also seen in the UOQ of the left breast (C). The patient was immune compromised.

B subcutaneous fat that may occur in 2-3% of patients fibrosis. Awareness of the radiologic and clinical features with systemic lupus erythematosis, usually between the will be helpful in future diagnoses of lupus mastitis25 ages of 20 and 50 years, and its occurrence is two times (Fig. 9). greater in women than in men. Lesions are often firm, 3-cm masses that may or may not be tender. Epidermal Scleroderma is an autoimmune disease characterized changes of atrophy and ulceration may be present. The by fibrosis (or hardening), vascular alterations, and clinical course of lupus mastitis is often chronic with flares auto antibodies. There are two types of scleroderma: and remissions. Surgical excision alone may not cure the generalized form known as systemic sclerosis and patient if there is inadequate immunosuppression. The localized form known as morphea. case documents the progressive mammographic changes of lupus mastitis, including the increasing density Systemic sclerosis usually involves the skin, but reflecting the development of fibrosis; the progression sometimes it also affects the gastrointestinal tract, the in dystrophic calcifications from thin curvilinear lungs and the kidneys. At the same time a narrowing calcifications to large dense coarse ones; and the overall of the small blood vessels takes place. Because the decrease in size of the breast, again associated with the hardening of the skin is the most typical symptom of the

Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 244 Mammary Manifestations of Some Systemic Disorders disease, it is called ‘scleroderma’. Since the disease also unexplained breast erythema benefit from early tissue affects internal organs, the name ‘systemic sclerosis’ is biopsy to confirm a clinical diagnosis and thus guide more precise. Lymphadenopathy has also been reported subsequent therapeutic interventions27,28 (Fig. 10). to be the first presentation of some patients with systemic sclerosis.26 Diabetic mastopathy is an uncommon condition affecting women with a history of long-standing Morphea are localized asymmetrical skin thickening insulin dependant diabetes mellitus (IDDM).29 The that is similar to those seen in systemic sclerosis but pathogenesis of diabetic mastopathy is not known. It without the disease features in the multiple internal was postulated that an autoimmune reaction resulting organs and blood vessels. Breast-associated morphea from abnormal cross linking of collagen might be (BAM) can mimic benign and malignant inflammatory responsible.30 The women typically present with firm, breast disorders. Breast Morphea has been reported mobile breast masses that may be irregular in contour. to be the first presentation of some patients who have Mammograms in women classically show dense breast given no history of autoimmune disorders. Patients with tissue, and, because of the density, a focal breast mass

A B Figure (10): 45 year-old female patient presenting with bilateral swollen edematous breasts with marked skin thickening and discoloration. On ultrasound examination there was a diffuse inflammatory reaction of both breasts (A) together with axillary and cervical lymphadenopathy (B). The patient was as inflammatory breast carcinoma on basis of imaging findings and biopsy was recommended. The diagnosis of localized scleroderma was confirmed by skin and lymphnode biopsy

A B Figure (11): 44 year- old female with un-controlled IDDM complaining of painful swelling in the right lower inner breast quadrant (arrow) A- Cranio-caudal mammogram shows an ill defined opacity seen in the lower inner quadrant. B- US and color Doppler studies revealed an irregular hypo-echoic mass with feeding vessels in its periphery. US guided FNAB revealed diabetic mastopathy with invasive carcinoma grade II on-top.

Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 245 Rasha Mohamed Kamal may not be easily seen.29 The US findings described 2. lymphatic spread from intra-thoracic and intra- in literature is irregular hypo echoic areas with dense abdominal foci, or 3. direct spread. Popli, 199936 classified posterior acoustic shadowing.31 On histopathology the disease presentation into three forms: nodular, examination lesions typically show massive fibrosis and diffuse and sclerosing. The nodular form is characterized lymphocytic infiltration around the lobules and ducts32 by a slowly growing caseating lesion and may present (Fig. 11). as a dense round or oval well defined shadow which may show indistinct margins on mammography. The diffuse form consists of multiple, intercommunicating Breast Granulomas foci of tuberculosis within the breast, which may caseate leading to ulceration and numerous discharging sinuses. Granulomas are special types of inflammation The skin may be thickened with a tense and tender characterized by accumulations of macrophages, some breast. In the diffuse form there is a dense breast and of which coalesce into “giant cells”. Granulomatous thickened skin. In addition to the breast lesions, the inflammation is especially characteristic of tuberculosis, axillary lymphnodes were affected. In the sclerosing some deep fungal infections (like histoplasmosis and form, excessive fibrosis rather than caseation is the coccidioidomycosis), sarcoidosis (a disease of unknown dominant feature. Progress is slow and suppuration is cause), and reaction to foreign bodies. rare. The breast is hard and the may be retracted. Increased density of the gland is seen on mammography. Tuberculosis of the breast is a rare disease with a, No definite sonographic details of breast tuberculosis reported incidence between 0.025 and 0.1%33 It is are reported in literature. In his limited study on 7 uncommon even in countries were the incidence of patients, Popli, 1999,36 found that nodular lesions pulmonary and extra pulmonary tuberculosis is high. are hypoechoeic or complex cystic. In diffuse lesions, Two forms of tuberculosis were described; the primary they are ill defined and hypoechoiec. While in the form in which breast infection is the only manifestation sclerosing form, there is increased echogenicity of the of the disease and secondary infection in which the background parenchyma with no definite lesions. The patient has tuberculosis elsewhere.34 Primary infection diagnostic criteria are the presence of granulomatous occured probably through skin abrasions or through the infiltrates and/or tubercles with central caseation, seen duct openings in the nipple.35 Routes of infection in on histology or bacteriological culture of the aspirate36 secondary tuberculosis include: 1. hematogenous spread, (Fig. 12, 13 &14).

A B

Figure (12): Female patient presenting by a palpable axillary tail and axillary mass lesions A- MLO mammogram shows right axillary lymphadenopathy. B- US revealed oval hypo-echoic lymph nodes showing cystic changes (caseation). Biopsy revealed primary TB adenitis of the breast.

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A B

C D

Figure (13): 28-year-old female presenting by palpable breast mass A- Craniocaudal mammogram shows subtle deeply seated opacity displacing pectoral muscle anteriorly (arrow) B- On US examination a complex cystic mass with fine internal echoes is seen causing underlying rib erosion C- Axial post contrast CT shows the soft tissue mass and underlying rib erosion. Associated para-vertebral soft tissue mass (cold abscess) is noted. D- Sagittal T1 MRI of the breast shows the same lesion. Biopsy revealed tuberculous mastitis.

Sarcoidosis is a systemic disorder of unknown cause process or from lymph node enlargement.37 that is characterized by the presence of noncaseating granulomas with proliferation of epithelioid cells. At mammography, breast sarcoidosis has been described Sarcoidosis commonly affects young and middle as irregular or ill-defined, often spiculated masses that are aged patients, with a slightly higher prevalence in suspicious for carcinoma, although small, well-defined women. Involvement of the breast parenchyma is a round masses have also been described. The latter finding rare manifestation of sarcoidosis, representing only may in some cases represent intramammary lymph node approximately 1% of all cases.Primary breast involvement involvement. Calcifications are absent. US findings are can be considered exceptional. It manifests clinically as also indicative of malignancy, with irregular hypo echoic firm to hard masses, often mimicking carcinoma. Breast masses being the most common38 (Fig. 15). involvement may result from intrinsic parenchyma involvement by the granulomatous inflammatory

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A B

C D

Figure (14): 45-year-old female presenting with multiple discharging skin sinuses. Cranio caudal (A) and mediolateral oblique (B) views showed markedly distorted right breast parenchyma associated with right pathological axillary lymphadenopathy. C, D. On ultrasound examination there were multiple skin sinus tracts leading to intercommunicating abscess cavities. After revision of the pathology specimens, the diagnosis of diffuse tuberculous mastitis was established

A B Fig (15): A well known case of sarcoidosis showing inflammatory reaction of the right breast with well defined intra mammary mass lesions representing enlarged intra mammary lymphnodes (A). Bilateral axillary lymphadenopathy with eccentric hila were also identified (B).

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Breast Lesions Secondary to secondary hyperparathyroidism, may develop extensive Major Organ Failure diffuse vascular calcifications in the breast.3 Diffuse coarse, subcutaneous breast calcifications can also Major Organ failure has major consequences on all be seen, particularly in patients undergoing dialysis. body systems with incremental degrees of physiological Patients with chronic renal failure may also present derangements. Major organ failure resulting in with breast symptoms similar to those described with redistribution of intravascular fluid volume and congestive heart failure (Fig.16). enhanced microvascular permeability may present with edema. is a benign enlargement of the male breast resulting from a proliferation of the glandular Unilateral breast edema as part of the generalized component of the breast. Gynecomastia results from edema in patients with congestive heart failure was an altered estrogen-androgen balance, in favor of reported frequently in literature.39,40 There are rare estrogen, or from increased breast sensitivity to a reports of difficulties in differentiating inflammatory normal circulating estrogen level. Gynaecomastia is a breast carcinoma from congestive heart failure.41,42 common presentation in men with liver cell failure.44 However inflammatory breast carcinoma has distinctive Clinically, the breast is enlarged, soft and tender and histologic and microscopic characteristics allowing a mass may be palpable in the retro-areolar region. On the establishment of proper diagnosis. In addition, mammography, gynecomastia can be nodular, dendritic Pluchinotta, et al, 1994,43 said that the clue to the or diffuse. Dendritic gynecomastia appears as ‘flame- correct diagnosis clinically is the presence of pitting shaped’ fibroglandular tissue in the retro-areolar area, edema which is not found in malignant breast edema. which radiates from the nipple into the deeper adipose On mammography patients with congestive heart tissue. In diffuse gynecomastia, the mammographic failure may show marked diffuse unilateral breast appearance is that of a dense breast. There is edema and skin thickening. No underlying masses or enlargement of the breast with diffuse density and both lymphadenopathy are detected on U/S examination. dendritic and nodular features may be seen. Absence of a well-defined identifiable mass and the secondary signs Patients with chronic renal failure, who develop differentiate gynecomastia from malignancy45 (Fig. 17).

A B Figure (16): A female patient with chronic renal failure under regular haemodialysis. Her mammogram (A) shows difuse edema pattern of her left breast. Complementary ultrasound (B) showed diffuse interstitial edema lines. No associated mass le- sions or pathological lymphnodes are identified.

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Figure (17): Male patient with liver cell failure presenting with dendritic gynaecomastia showing flame shaped fibro glandular tissue in the retroareolar region A

Breast Lesions Secondary to (Coagulation Defects)

Breast are common after traumas, surgeries, or contusions.46 They are very rarely spontaneously. Hematomas can occur spontaneously in patients with hematologic disease or with coagulation disorder. Complete resolution should be achieved after 60 days. Good history, ultrasound and mammography are the basic techniques in diagnosing spontaneous breast hematomas. Hematomas in the breast are usually well or partially defined, and may B show calcification.46 They may reach large sizes and thus have to be differentiated from other giant breast masses as lipomas hamartomas, giant fibroadenomas and cysts.47,48 On ultrasound, hematomas are difficult to be differentiated from breast abscesses. Both are sonolucent, although some may demonstrate some low-amplitude internal echoes. Good history usually differentiates the two (Fig. 18).

Atherosclerotic Vascular Calcification

Mammary arterial atherosclerotic calcifications are commonly encountered at mammography. They C are usually easily identified as benign findings.49 Two forms of arterial calcifications that are recognized at Figure (18): 22 year-old female, known to have idiopathic radiographic and histopathologic analysis are described: thrombocytopenic purpura, presenting by a breast mass. A- Craniocaudal mammogram showing a well circumscribed those of the arterial intima (atherosclerosis or nodular dense mass with lobulated outline (arrow). Another adjacent arteriosclerosis) and those of the media (Mönckeberg mass with ill defined borders is seen. B- US revealed a large medial calcific sclerosis). Calcifications of the intima oval heterogeneous hypo-echoic mass resembling breast appear as relatively large, discontinuous calcific deposits abscess but no inflammatory changes and no flow was and generally occur in large and medium-sized arteries. detected on color Doppler examination C-Follow up after one month shows partial resolution and liquefaction of the Calcifications of the media are more fine grained and previously detected mass.

Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 250 Mammary Manifestations of Some Systemic Disorders diffuse and tend to involve the entire circumference of track.51 At histopathology analysis, Mönckeberg medial peripheral arterioles. It is the latter form (Mönckeberg calcific sclerosis appears as ring-like calcification within medial calcific sclerosis) that occurs in thebreast and that the media of small to medium-sized vessels. There is identified at mammography as vascular calcification. is no associated thickening of the intima. The exact At the time of presentation most patients give past pathogenesis of Mönckeberg medial calcific sclerosis is history of diabetes, hypertension or atherosclerotic unknown, and there is no known inciting injury of the calcifications elsewhere.50 On mammography they media49 (Fig 19). manifest as linear, parallel calcifications in a railroad

Figure (19): A case of multifocal breast carcinoma associated with linear railroad track vascular calcifications.

Figure 20: Screening mammograms of a well known case with neurofibromatosis Type 1 who presented with café au lait patches and multiple neurofibromas at the region of both breasts. Her mammogram showed multiple, bilateral, partially obscured nodu- lar lesions.

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