Mammary Manifestations of Some Systemic Disorders
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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 Breast 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 mammography 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 breast mass 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 Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 238 Mammary Manifestations of Some Systemic Disorders 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 breasts 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. Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 239 Rasha Mohamed Kamal 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 Austral - Asian Journal of Cancer ISSN-0972-2556, Vol. 10, No. 4, October 2011 pp 237-253 240 Mammary Manifestations of Some Systemic Disorders Figure (6): 63 year- old female with history of malignant melanoma of the left